Cardiology — Hypertension
106. Hypertensive Emergencies
emergency (acute end-organ damage → controlled IV lowering) vs severe uncontrolled HTN (no end-organ damage → oral, gradual) · the distinction is target-organ injury, not the number · separate dosed plans · Super Compact
Approach — the question is not "how high is the BP" but "is there acute end-organ damage?" A hypertensive emergency is severe hypertension with acute target-organ injury (brain, heart, kidneys, aorta, eyes) — it needs controlled IV therapy in a monitored setting. Severe asymptomatic (uncontrolled) hypertension is a high number without acute end-organ damage — managed with oral agents and gradual lowering. The cardinal rule of the true emergency: lower BP gradually (generally ≤25% in the first hour) — except where rapid lowering is indicated (aortic dissection) — because over-rapid lowering causes cerebral/coronary/renal hypoperfusion.
Key discriminators: severe HTN + headache/confusion/seizure/visual changes (after excluding stroke) → encephalopathy · severe HTN + acute dyspnea/diffuse B-lines → flash pulmonary edema (afterload-driven) · severe HTN + rising creatinine/hematuria/proteinuria → HTN-mediated AKI · severe HTN + tearing chest/back pain + pulse/BP differential → aortic dissection (lower fast + HR control) · severe HTN, no acute symptoms/injury → severe uncontrolled (asymptomatic) HTN
Initial Sx/Data (all comers): measure BP correctly (both arms, appropriate cuff), assess end-organ injury — neuro exam, fundoscopy (papilledema/hemorrhages), cardiopulmonary exam · ECG, troponin, BMP (creatinine), UA (hematuria/proteinuria), CBC (hemolysis/MAHA), CXR; head CT if neuro symptoms; CTA if dissection suspected; plasma metanephrines if pheo suspected · (don't anchor — don't aggressively lower BP in acute ischemic stroke, where permissive hypertension is the rule)
Neg (don't-miss):denies acute ischemic stroke (lowering BP can extend the infarct — different thresholds) · denies aortic dissection (needs rapid lowering + beta-blockade first — opposite of the gradual rule) · denies that "severe HTN" is actually pain/anxiety/withdrawal/missed meds · denies a secondary cause (pheochromocytoma, renovascular, cocaine/sympathomimetic, eclampsia) · denies treating an asymptomatic high number as an emergency
DDx: hypertensive emergency — encephalopathy / pulmonary edema / AKI (and ACS, aortic dissection, eclampsia, MAHA) · severe uncontrolled (asymptomatic) HTN · (also: ischemic/hemorrhagic stroke, pheochromocytoma, sympathomimetic toxicity, pain/anxiety-driven HTN, white-coat/pseudo-resistant)
Plan — by Diagnosis
CONSULT (as relevant): ICU (emergency requiring IV titratable agents + arterial line) · Cardiology (cardiac end-organ, dissection) · Nephrology (HTN-mediated AKI, renovascular/secondary HTN) · Neurology (encephalopathy vs stroke) · Cardiac/Vascular surgery (aortic dissection)
Hypertensive emergency — encephalopathy
– Confirm: severe HTN with headache, confusion, seizures, or visual changes, often papilledema; EXCLUDE stroke/ICH with head CT first (encephalopathy is a diagnosis of exclusion, reversible with BP control)
– Rx: ICU + IV titratable agent with arterial line — nicardipine infusion 5 mg/h, titrate by 2.5 mg/h q5–15 min (max 15 mg/h), or clevidipine, or labetalol 10–20 mg IV bolus then infusion; lower MAP ~10–20% in the first hour, no more than ~25%, then gradually toward normal over 24–48 h; treat seizures; identify precipitant
– Monitor / daily labs: arterial line, continuous neuro checks, q1h vitals, daily BMP; improvement supports the diagnosis
– Dispo: transition to oral agents once stable; address secondary cause; neurology if uncertain; cardiology/PCP follow-up; med rec
– Hypertensive encephalopathy is reversible with controlled BP lowering — but exclude stroke first, because in an ischemic stroke aggressively dropping the pressure extends the infarct. The rule is gradual: ~25% max in the first hour.
Hypertensive emergency — pulmonary edema (flash)
– Confirm: severe HTN + acute dyspnea/hypoxemia + diffuse B-lines — afterload-driven flash pulmonary edema
– Rx: IV nitroglycerin infusion start 10–20 mcg/min, titrate up (afterload/preload reduction is the dominant lever), or nicardipine/clevidipine; early NIPPV (CPAP/BiPAP); IV loop diuretic (furosemide 40–80 mg IV); sit upright, O2; treat ischemic precipitant
– Monitor / daily labs: continuous SpO2/telemetry, q15 min BP on nitro drip, serial ABG, daily BMP
– Dispo: step-down/ICU until stable; transition to oral antihypertensives + HF pathway; cardiology follow-up; med rec
– Hypertensive flash pulmonary edema responds dramatically to afterload reduction (IV nitroglycerin) plus NIPPV — diuretics are secondary because these patients are usually euvolemic with fluid shifted into the lungs by the high afterload.
Hypertensive emergency — AKI
– Confirm: severe HTN with AKI (rising creatinine, hematuria, proteinuria), possibly microangiopathic hemolysis (malignant HTN with TMA)
– Rx: controlled IV lowering — nicardipine or clevidipine infusion (titratable, no significant renal toxicity); avoid over-rapid lowering (worsens renal perfusion); gradual reduction over 24–48 h; evaluate secondary causes — scleroderma renal crisis → ACE inhibitor (captopril 6.25–12.5 mg PO, titrate) is the specific targeted therapy; renovascular, glomerulonephritis; dialysis if needed
– Monitor / daily labs: daily BMP (creatinine, K), urine output, CBC (hemolysis/schistocytes), arterial line
– Dispo: nephrology; transition to oral agents; treat secondary cause; close renal follow-up; med rec
– In HTN-mediated AKI, control the pressure gradually with a titratable IV agent and watch the kidneys closely — and consider scleroderma renal crisis, where an ACE inhibitor is the targeted, life-and-kidney-saving therapy.
Severe uncontrolled (asymptomatic) hypertension
– Confirm: markedly elevated BP WITHOUT acute end-organ damage and without acute symptoms (asymptomatic severe HTN / "hypertensive urgency")
– Rx: NOT an emergency — manage with oral agents + gradual lowering over days, NOT aggressive IV therapy or rapid reduction; restart/adjust the home oral regimen or address nonadherence (most common cause); ensure calm environment, rule out pain/anxiety; AVOID PRN IV/oral boluses (IV hydralazine/labetalol, oral clonidine) chasing the number — overshoot hypotension harms
– Monitor: serial BP in a calm setting; no arterial line/ICU needed
– Dispo: close outpatient follow-up; investigate secondary causes if resistant; adherence support; med rec
– A high number without end-organ damage is not an emergency — the harm comes from treating it like one. Restart the home meds, address adherence, follow up. Acutely overshooting the pressure causes strokes and MIs.
106. Hypertensive Emergencies
complete reference · emergency (acute end-organ damage) versus severe asymptomatic hypertension · controlled IV lowering versus gradual oral therapy · a dosed, bulleted plan per scenario · Full Card
Approach — Is There Acute End-Organ Damage?
The question is not how high the blood pressure is but whether there is acute target-organ injury (brain, heart, kidneys, aorta, eyes). A hypertensive emergency is severe hypertension with acute end-organ damage and needs controlled IV therapy in a monitored setting.
Severe asymptomatic (uncontrolled) hypertension is a high number without acute end-organ damage and is managed with oral agents and gradual lowering, not aggressive IV treatment.
The cardinal rule of the true emergency is to lower the blood pressure in a controlled, gradual way (generally no more than 25% in the first hour), except where rapid lowering is specifically indicated (aortic dissection), because over-rapid lowering causes cerebral, coronary, and renal hypoperfusion.
Key discriminators: severe HTN with headache, confusion, seizure, or visual changes (after excluding stroke) is encephalopathy; with acute dyspnea and diffuse B-lines is flash pulmonary edema; with a rising creatinine, hematuria, and proteinuria is hypertension-mediated AKI; with tearing chest or back pain and a pulse differential is aortic dissection (lower fast with HR control); and a high number with no acute symptoms or injury is severe uncontrolled hypertension.
Initial Symptoms / Data (all comers)
Measure the blood pressure correctly (both arms, an appropriate cuff) and assess for end-organ injury with a neurologic exam, fundoscopy (papilledema, hemorrhages), and a cardiopulmonary exam.
ECG, troponin, BMP (creatinine), urinalysis (hematuria, proteinuria), CBC (hemolysis or MAHA), and CXR; a head CT if there are neurologic symptoms; CT angiography if dissection is suspected; and plasma metanephrines if pheochromocytoma is suspected.
Neg (don't-miss)
Pt denies an acute ischemic stroke (lowering the blood pressure can extend the infarct — permissive hypertension applies with different thresholds) and denies an aortic dissection (which needs rapid lowering and beta-blockade first, the opposite of the gradual rule).
Pt denies that the "severe HTN" is actually driven by pain, anxiety, withdrawal, or missed medications, and denies a secondary cause (pheochromocytoma, renovascular disease, cocaine or sympathomimetic use, eclampsia).
Pt denies treating an asymptomatic high number as an emergency with aggressive IV agents.
DDx
Hypertensive emergency — encephalopathy, pulmonary edema, or AKI (and ACS, aortic dissection, eclampsia, microangiopathic hemolysis) · severe uncontrolled (asymptomatic) hypertension · (also ischemic or hemorrhagic stroke, pheochromocytoma, sympathomimetic toxicity, pain or anxiety-driven hypertension, and white-coat or pseudo-resistant hypertension)
Plan — by Diagnosis
CONSULT (as relevant): ICU (a hypertensive emergency requiring IV titratable agents and an arterial line) · Cardiology (cardiac end-organ involvement, dissection) · Nephrology (hypertension-mediated AKI, renovascular or secondary hypertension) · Neurology (encephalopathy versus stroke) · Cardiac or Vascular surgery (aortic dissection)
Hypertensive emergency — encephalopathy
Confirm: severe HTN with headache, confusion, seizures, or visual changes, often with papilledema, after excluding stroke and intracranial hemorrhage with a head CT (encephalopathy is a diagnosis of exclusion, reversible with BP control).
Therapy: ICU care with an IV titratable agent and an arterial line — a nicardipine infusion at 5 mg/h titrated by 2.5 mg/h every 5–15 minutes (maximum 15 mg/h), or clevidipine, or labetalol 10–20 mg IV bolus then an infusion — lowering the mean arterial pressure by about 10–20% in the first hour and no more than about 25%, then gradually toward normal over 24–48 hours; treat seizures; and identify the precipitant.
Monitoring / daily labs: an arterial line, continuous neurologic checks, hourly vitals, and a daily BMP; neurologic improvement supports the diagnosis.
Mobility / consults / disposition: transition to oral agents once stable; address any secondary cause; neurology if uncertain; cardiology or PCP follow-up; medication reconciliation.
Hypertensive emergency — pulmonary edema (flash)
Confirm: severe HTN with acute dyspnea and hypoxemia and diffuse B-lines — afterload-driven flash pulmonary edema.
Therapy: an IV nitroglycerin infusion starting at 10–20 mcg/min and titrated up (afterload and preload reduction is the dominant lever), or nicardipine or clevidipine; early NIPPV (CPAP or BiPAP); an IV loop diuretic (furosemide 40–80 mg IV); sitting the patient upright with O2; and treatment of any ischemic precipitant.
Monitoring / daily labs: continuous SpO2 and telemetry, blood pressure every 15 minutes on the nitroglycerin drip, serial ABG, and a daily BMP.
Mobility / consults / disposition: step-down or ICU care until stable; transition to oral antihypertensives and the HF pathway; cardiology follow-up; medication reconciliation.
Hypertensive emergency — AKI
Confirm: severe HTN with acute kidney injury (a rising creatinine, hematuria, proteinuria), possibly with microangiopathic hemolysis (malignant or accelerated hypertension with thrombotic microangiopathy).
Therapy: controlled IV BP lowering with a nicardipine or clevidipine infusion (titratable, without significant renal toxicity); avoid over-rapid lowering, which can worsen renal perfusion, reducing gradually over 24–48 hours; evaluate for secondary causes, with scleroderma renal crisis treated specifically with an ACE inhibitor (captopril 6.25–12.5 mg PO titrated up) as the targeted therapy, plus renovascular disease and glomerulonephritis; and dialyze if needed.
Monitoring / daily labs: a daily BMP (creatinine, potassium), urine output, a CBC (for hemolysis and schistocytes), and an arterial line.
Mobility / consults / disposition: nephrology; transition to oral agents; treat the secondary cause; close renal follow-up; medication reconciliation.
Severe uncontrolled (asymptomatic) hypertension
Confirm: a markedly elevated blood pressure without acute end-organ damage and without acute symptoms (asymptomatic severe hypertension, sometimes called hypertensive urgency).
Therapy: recognize this is not an emergency and manage it with oral agents and gradual lowering over days rather than aggressive IV therapy or rapid reduction; restart or adjust the home oral regimen (or address nonadherence, the most common cause), ensure a calm environment, and rule out pain or anxiety; and avoid acute as-needed IV or oral boluses (PRN IV hydralazine or labetalol, oral clonidine) chasing the number, which cause overshoot hypotension and harm.
Monitoring: serial blood pressure in a calm setting, with no arterial line or ICU needed.
Mobility / consults / disposition: close outpatient follow-up; investigate secondary causes if resistant; adherence support; medication reconciliation.
Red Flags
Tearing chest or back pain with a pulse or blood-pressure differential → aortic dissection; rapid lowering with beta-blockade first, the opposite of the gradual rule
Focal neurologic deficits → an acute stroke, where aggressive BP lowering can extend the injury
Microangiopathic hemolysis with AKI and severe HTN → malignant hypertension with thrombotic microangiopathy or scleroderma renal crisis
Episodic severe HTN with palpitations, headache, and diaphoresis → pheochromocytoma (avoid unopposed beta-blockade)
Chasing an asymptomatic high number with PRN IV pushes → overshoot hypotension causing stroke or MI
Senior IM Resident Pearls
End-organ damage, not the number, defines the emergency. A BP of 220/120 with no acute injury is urgency; 170/110 with encephalopathy or flash edema is an emergency.
Lower gradually — about 25% in the first hour. Over-rapid lowering hypoperfuses the brain, heart, and kidneys; the exception is aortic dissection, which needs fast control.
Exclude stroke before treating "encephalopathy." In an ischemic stroke, dropping the pressure extends the infarct.
Flash pulmonary edema is afterload. IV nitroglycerin and NIPPV beat diuretics in the usually-euvolemic hypertensive patient.
Scleroderma renal crisis wants an ACE inhibitor. It's the one hypertensive-AKI scenario with a specific targeted drug.
Don't treat a high number like a fire. Asymptomatic severe HTN gets oral meds and follow-up — the PRN IV reflex causes the harm.
Common mistake: aggressively lowering an asymptomatic high BP with IV boluses, or dropping the pressure fast in an ischemic stroke and extending the infarct.
Cardiology — Valvular Heart Disease
107. Valvular Heart Disease
severe aortic stenosis · severe mitral regurgitation (acute vs chronic) · prosthetic valve complications · TAVR/SAVR complications · separate dosed plans per lesion · Super Compact
Approach — identify the lesion, grade its severity, and decide whether this is a stable lesion to manage medically or an acute valvular emergency that needs the surgeon now. The echocardiogram is the central tool. Two physiologic principles drive bedside decisions: a fixed obstruction (severe AS, or a stenotic/obstructed prosthesis) is preload- and afterload-dependent — vasodilators and aggressive diuresis can cause sudden collapse; and acute severe regurgitation (acute MR/AR) is a surgical emergency the ventricle/atrium have had no time to adapt to, presenting as flash pulmonary edema or shock. Always ask native vs prosthetic — prosthetic problems (thrombosis, endocarditis, dehiscence) have their own urgent pathways.
Key discriminators: exertional dyspnea/angina/syncope + harsh crescendo-decrescendo systolic murmur + slow-rising pulse → severe AS · acute flash edema/shock + new holosystolic murmur post-MI or with endocarditis → acute severe MR · chronic exertional dyspnea + holosystolic murmur radiating to axilla → chronic severe MR · prosthetic valve + new murmur/HF/embolism/muffled clicks → prosthetic thrombosis/obstruction/dysfunction · recent TAVR/SAVR + conduction block/hypotension/HF → procedural complication
Initial Sx/Data (all comers): dyspnea, exertional symptoms (angina, syncope, dyspnea), palpitations, HF/shock; auscultation (murmur character, radiation, intensity), pulse character, perfusion · echo (TTE → TEE for prosthetic/endocarditis/MR mechanism — gradients, valve area, regurgitant severity, LV/RV, vegetation); ECG; CXR; BNP, troponin; blood cultures if endocarditis suspected · (don't anchor — a new murmur with decompensation is an acute valvular emergency until the echo says otherwise)
Neg (don't-miss):denies acute severe regurgitation (papillary muscle rupture post-MI, acute prosthetic dehiscence, endocarditis with leaflet destruction → surgical emergency) · denies prosthetic valve thrombosis/obstruction (a stuck mechanical valve is life-threatening) · denies endocarditis on the valve (blood cultures, TEE) · denies giving a vasodilator/aggressive diuresis across a fixed obstruction (severe AS) · denies missing the conduction block needing a pacemaker after TAVR
DDx: severe aortic stenosis · severe mitral regurgitation (acute vs chronic) · prosthetic valve complications (thrombosis, dysfunction, hemolysis, dehiscence, endocarditis) · TAVR/SAVR complications · (also: aortic regurgitation, mitral stenosis, tricuspid disease, HCM with outflow obstruction, mixed valve disease)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology + Structural/Interventional cardiology (valve eval, TAVR, valvuloplasty, prosthetic dysfunction) · Cardiac surgery (acute regurgitation, valve replacement/repair, prosthetic complications) · CICU (acute valvular emergency, shock) · ID (prosthetic valve endocarditis)
Severe aortic stenosis
– Confirm: exertional dyspnea/angina/syncope; harsh crescendo-decrescendo murmur, slow-rising carotid pulse; echo — AVA <1.0 cm², mean gradient ≥40 mmHg, peak velocity ≥4 m/s
– Definitive: aortic valve replacement — TAVR or SAVR (heart-team) for symptomatic severe AS; balloon aortic valvuloplasty as a bridge in unstable/decompensated patients
– Medical caveats: AVOID aggressive preload/afterload reduction (vasodilators, high-dose nitrates, over-diuresis) — fixed obstruction can precipitate hypotension/syncope/collapse; maintain preload + sinus rhythm; cautious diuresis (furosemide low-dose IV) for congestion; treat decompensation trigger
– Monitor / daily labs: telemetry, daily BMP, BP trend; echo-guided
– Dispo: structural heart referral; PT/OT; cardiology follow-up; activity counseling; med rec
– Severe symptomatic AS is fixed-obstruction physiology — the ventricle needs preload and afterload to cross the tight valve, so vasodilators and aggressive diuresis can cause sudden collapse. The real fix is replacing the valve; valvuloplasty only bridges.
Severe mitral regurgitation — acute
– Confirm: sudden flash pulmonary edema or cardiogenic shock + new murmur (may be soft/absent); papillary muscle rupture (post-MI), chordal rupture, or endocarditis; echo/TEE confirms
– Rx (surgical emergency): URGENT cardiac surgery consult for repair/replacement; afterload reduction to improve forward flow — IV nitroprusside 0.3–0.5 mcg/kg/min titrated (or nitroglycerin) if BP tolerates; IV diuretics; intra-aortic balloon pump as a bridge in shock; NIPPV/mechanical ventilation for pulmonary edema; revascularize the MI
– Monitor / daily labs: CICU, arterial line, continuous SpO2/telemetry, serial ABG, daily BMP
– Dispo: operative management; avoid delay; cardiac surgery/CICU coordination; med rec
– Acute severe MR is a surgical emergency — the unprepared LA/LV can't accommodate the sudden volume, so it flash-floods the lungs. Afterload reduction and a balloon pump buy time, but the answer is the OR. Post-MI, think papillary muscle rupture.
Severe mitral regurgitation — chronic
– Confirm: chronic exertional dyspnea/fatigue; holosystolic murmur radiating to axilla; echo — severe regurgitant volume/fraction, LA/LV dilation, LV function and symptoms
– Rx: GDMT for HF symptoms (diuretics for congestion, GDMT if reduced EF); surgical mitral repair (preferred) or replacement for severe symptomatic primary MR, or asymptomatic with LV dysfunction/dilation; transcatheter edge-to-edge repair (MitraClip) for selected high-risk patients (esp secondary/functional MR on optimized GDMT — COAPT); rate/rhythm control of AF
– Monitor / daily labs: telemetry, daily BMP, serial echo surveillance
– Dispo: structural/surgical referral; timing is key (operate before irreversible LV dysfunction); cardiology follow-up; med rec
– Chronic severe MR is about timing the intervention before the LV is irreversibly damaged — repair beats replacement when feasible, and MitraClip extends a transcatheter option to high-risk and functional-MR patients (COAPT). Don't wait until the EF has already fallen.
Prosthetic valve complications
– Confirm: prosthetic valve + new symptoms — thrombosis/obstruction (muffled clicks, new gradient, HF, embolism — esp subtherapeutic anticoagulation), structural deterioration (bioprosthetic, late), paravalvular leak/dehiscence, hemolysis (schistocytes, ↑LDH, ↓haptoglobin), or endocarditis; TEE is key (+ fluoroscopy/CT for leaflet motion)
– Rx: thrombosis → anticoagulation, fibrinolysis, or urgent surgery by valve side/severity/stability; obstruction/structural failure → surgical or transcatheter (valve-in-valve); paravalvular leak/dehiscence → percutaneous closure or surgery; hemolysis → treat the leak, supportive transfusion; endocarditis → cultures, antibiotics, ID + surgery; restore/optimize anticoagulation for mechanical valves (heparin bridge to warfarin, target INR per valve)
– Monitor / daily labs: telemetry, daily BMP/CBC, LDH/haptoglobin if hemolysis, INR/anti-Xa
– Dispo: cardiac surgery/structural + cardiology; anticoagulation management; follow-up; med rec
– A prosthetic valve with new heart failure, embolism, or muffled clicks is a stuck/obstructed valve until proven otherwise — get a TEE urgently. Subtherapeutic anticoagulation is the classic setup for mechanical valve thrombosis, and treatment depends on side and severity.
TAVR / SAVR complications
– Confirm: recent TAVR or SAVR + new problem — new conduction block (AV block/LBBB) needing a pacemaker, paravalvular leak, vascular access complications (TAVR), stroke, valve thrombosis, pericardial effusion/tamponade
– Rx: conduction block → continuous telemetry, permanent pacemaker for high-grade AV block (common, expected TAVR complication); significant paravalvular leak → closure/intervention; vascular access complication → vascular surgery/IR; stroke → stroke pathway; valve thrombosis → anticoagulation per structural cardiology; effusion/tamponade → echo, pericardiocentesis
– Monitor / daily labs: continuous telemetry (watch for AV block), daily BMP/CBC, access-site checks
– Dispo: coordinate with the structural heart/surgical team that performed the procedure; cardiology follow-up; med rec
– After TAVR, watch the conduction system — high-grade AV block is a common, expected complication, and a substantial fraction of TAVR patients need a permanent pacemaker. Keep them on telemetry and loop in the structural team for any decompensation.
107. Valvular Heart Disease
complete reference · severe aortic stenosis, acute and chronic mitral regurgitation, prosthetic valve complications, and TAVR/SAVR complications · fixed-obstruction and acute-regurgitation physiology · a dosed, bulleted plan per lesion · Full Card
Approach — Identify the Lesion, Grade It, and Decide Medical Versus Surgical
Identify the lesion, grade its severity, and decide whether this is a stable lesion to manage medically or an acute valvular emergency that needs the surgeon now. The echocardiogram is the central tool.
Two physiologic principles drive the bedside decisions: a fixed obstruction (severe aortic stenosis, or a stenotic or obstructed prosthesis) is preload- and afterload-dependent, so vasodilators and aggressive diuresis can cause sudden collapse; and acute severe regurgitation (acute MR or AR) is a surgical emergency that the ventricle and atrium have had no time to adapt to, presenting as flash pulmonary edema or shock.
Always ask whether the valve is native or prosthetic, because prosthetic problems (thrombosis, endocarditis, dehiscence) have their own urgent pathways.
Key discriminators: exertional dyspnea, angina, or syncope with a harsh systolic murmur and a slow-rising pulse suggests severe aortic stenosis; acute flash edema or shock with a new holosystolic murmur post-MI suggests acute severe MR; chronic exertional dyspnea with a holosystolic murmur radiating to the axilla suggests chronic severe MR; a prosthetic valve with a new murmur, heart failure, embolism, or muffled clicks suggests prosthetic dysfunction; and a recent TAVR or SAVR with conduction block, hypotension, or heart failure suggests a procedural complication.
Initial Symptoms / Data (all comers)
Dyspnea, exertional symptoms (angina, syncope, dyspnea), palpitations, and signs of heart failure or shock, with careful cardiac auscultation (murmur character, radiation, intensity), pulse character, and peripheral perfusion.
An echocardiogram (TTE first, with TEE for prosthetic valves, endocarditis, and the mechanism of MR — gradients, valve area, regurgitant severity, LV and RV function, vegetations); ECG; CXR; BNP and troponin; and blood cultures if endocarditis is suspected.
Neg (don't-miss)
Pt denies acute severe regurgitation (papillary muscle rupture post-MI, acute prosthetic dehiscence, endocarditis with leaflet destruction — a surgical emergency) and denies prosthetic valve thrombosis or obstruction (a stuck mechanical valve is life-threatening).
Pt denies endocarditis on the valve (blood cultures, TEE) and denies the danger of giving a vasodilator or aggressive diuresis across a fixed obstruction (severe AS, which can cause collapse).
Pt denies missing the conduction block that needs a pacemaker after TAVR.
DDx
Severe aortic stenosis · severe mitral regurgitation (acute versus chronic) · prosthetic valve complications (thrombosis, dysfunction, hemolysis, dehiscence, endocarditis) · TAVR/SAVR complications · (also aortic regurgitation, mitral stenosis, tricuspid disease, HCM with outflow obstruction, and mixed valve disease)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology and Structural or Interventional cardiology (valve evaluation, TAVR, valvuloplasty, prosthetic dysfunction) · Cardiac surgery (acute regurgitation, surgical valve replacement or repair, prosthetic complications) · CICU (acute valvular emergency, cardiogenic shock) · Infectious disease (prosthetic valve endocarditis)
Severe aortic stenosis
Confirm: exertional dyspnea, angina, or syncope with a harsh crescendo-decrescendo systolic murmur and a slow-rising carotid pulse, and an echo showing an aortic valve area under 1.0 cm², a mean gradient of 40 mmHg or more, and a peak velocity of 4 m/s or more.
Definitive therapy: aortic valve replacement — TAVR or SAVR by a heart-team decision based on risk and anatomy — for symptomatic severe AS, with balloon aortic valvuloplasty as a bridge in unstable or decompensated patients.
Medical caveats: avoid aggressive preload or afterload reduction (vasodilators, high-dose nitrates, over-diuresis) because the obstruction is fixed and these can precipitate hypotension, syncope, or collapse; maintain preload and sinus rhythm; diurese cautiously (low-dose IV furosemide) for congestion; and treat the trigger of decompensation.
Monitoring / daily labs: telemetry, a daily BMP, and a blood-pressure trend; echo-guided management.
Mobility / consults / disposition: structural heart referral; PT and OT; cardiology follow-up; activity counseling; medication reconciliation.
Severe mitral regurgitation — acute
Confirm: sudden flash pulmonary edema or cardiogenic shock with a new murmur (which may be soft or absent), from papillary muscle rupture (post-MI), chordal rupture, or endocarditis with leaflet destruction, confirmed on echo or TEE.
Therapy (surgical emergency): an urgent cardiac surgery consult for valve repair or replacement; afterload reduction to improve forward flow with IV nitroprusside 0.3–0.5 mcg/kg/min titrated (or nitroglycerin) if the blood pressure tolerates, plus IV diuretics; an intra-aortic balloon pump as a bridge in shock; NIPPV or mechanical ventilation for pulmonary edema; and revascularization of the MI.
Monitoring / daily labs: CICU care with an arterial line, continuous SpO2 and telemetry, serial ABG, and a daily BMP.
Mobility / consults / disposition: operative management without delay, with cardiac surgery and CICU coordination; medication reconciliation.
Severe mitral regurgitation — chronic
Confirm: chronic exertional dyspnea and fatigue with a holosystolic murmur radiating to the axilla, and an echo showing a severe regurgitant volume or fraction with LA and LV dilation, assessing LV function and symptoms.
Therapy: guideline-directed medical therapy for HF symptoms (diuretics for congestion, GDMT if the EF is reduced); surgical mitral valve repair (preferred) or replacement for severe symptomatic primary MR or for asymptomatic disease with LV dysfunction or dilation; transcatheter edge-to-edge repair (MitraClip) for selected high-surgical-risk patients, especially secondary or functional MR on optimized GDMT (COAPT); and rate and rhythm control of AF.
Monitoring / daily labs: telemetry, a daily BMP, and serial echo surveillance.
Mobility / consults / disposition: structural and surgical referral with careful timing of intervention before irreversible LV dysfunction; cardiology follow-up; medication reconciliation.
Prosthetic valve complications
Confirm: a prosthetic valve with new symptoms, considering thrombosis or obstruction (muffled or absent clicks, a new gradient, heart failure, embolism, especially with subtherapeutic anticoagulation), structural deterioration (late, bioprosthetic), paravalvular leak or dehiscence, hemolysis (schistocytes, an elevated LDH, a low haptoglobin), or endocarditis, with TEE as the key test (plus fluoroscopy or CT for mechanical leaflet motion).
Therapy: for thrombosis, anticoagulation, fibrinolysis, or urgent surgery depending on the valve side, severity, and stability; for obstruction or structural failure, surgical or transcatheter (valve-in-valve) replacement; for a significant paravalvular leak or dehiscence, percutaneous closure or surgery; for hemolysis, treatment of the leak with supportive transfusion; for endocarditis, blood cultures, antibiotics, and ID and surgery (see the endocarditis card); and restoration or optimization of anticoagulation for mechanical valves (a heparin bridge to warfarin with a valve-specific INR target).
Monitoring / daily labs: telemetry, a daily BMP and CBC, LDH and haptoglobin if hemolysis is suspected, and INR or anti-Xa monitoring.
Mobility / consults / disposition: cardiac surgery or structural cardiology and cardiology; anticoagulation management; follow-up; medication reconciliation.
TAVR / SAVR complications
Confirm: a recent transcatheter (TAVR) or surgical (SAVR) aortic valve replacement with a new problem, commonly a new conduction block (AV block or LBBB) needing a pacemaker, a paravalvular leak, vascular access complications (TAVR), stroke, valve thrombosis, or a pericardial effusion or tamponade.
Therapy: for a conduction disturbance, continuous telemetry with a permanent pacemaker for high-grade AV block (a common, expected TAVR complication); for a significant paravalvular leak, evaluation for closure or intervention; for a vascular access complication, vascular surgery or IR; for stroke, the stroke pathway; for valve thrombosis, anticoagulation per structural cardiology; and for an effusion or tamponade, echo and pericardiocentesis.
Monitoring / daily labs: continuous telemetry (watching for AV block), a daily BMP and CBC, and access-site checks.
Mobility / consults / disposition: coordinate with the structural heart and surgical team that performed the procedure; cardiology follow-up; medication reconciliation.
Red Flags
A new murmur with flash pulmonary edema or shock → acute severe regurgitation; a surgical emergency
A prosthetic valve with new HF, embolism, or muffled clicks → valve thrombosis or obstruction; urgent TEE
Syncope, angina, or HF with severe AS → a replacement indication; avoid vasodilators and aggressive diuresis
Subtherapeutic anticoagulation with a mechanical valve → the classic setup for valve thrombosis
High-grade AV block after TAVR → a permanent pacemaker indication
Senior IM Resident Pearls
Severe AS is fixed-obstruction physiology. The ventricle needs its preload and afterload to cross the tight valve — vasodilators and over-diuresis can cause sudden collapse, and the real fix is replacing the valve.
Acute severe MR is a surgical emergency. The unprepared atrium and ventricle flash-flood the lungs; afterload reduction and a balloon pump buy time to the operating room. Post-MI, think papillary muscle rupture.
Chronic MR is about timing. Operate (repair over replace) before the LV is irreversibly damaged; MitraClip extends a transcatheter option to high-risk and functional MR (COAPT).
A prosthetic valve with new symptoms is guilty until cleared. Thrombosis, dehiscence, hemolysis, or endocarditis — get a TEE, and remember subtherapeutic anticoagulation sets up mechanical valve thrombosis.
Watch the conduction system after TAVR. High-grade AV block is expected, and many patients need a permanent pacemaker.
A soft or absent murmur doesn't exclude severe regurgitation. In acute MR the pressures equalize fast, so the murmur can be quiet despite catastrophic physiology.
Common mistake: giving a vasodilator or aggressively diuresing a patient with critical AS and precipitating collapse, or under-appreciating a quiet new murmur in acute decompensation.
Cardiology — Pericardial Disease
108. Pericardial Disease
acute pericarditis (anti-inflammatory, exclude MI) · pericardial effusion · cardiac tamponade (fill + drain, never diurese) · separate dosed plans per entity · Super Compact
Approach — name the syndrome along a spectrum: inflamed pericardium (pericarditis), fluid in the sac (effusion), or fluid compressing the heart (tamponade). The same disease process can sit anywhere on this line, so the bedside question is "is the heart being compressed?"Acute pericarditis is an anti-inflammatory problem — but you must exclude myocardial infarction and myocarditis first. A pericardial effusion may be incidental or large — the issue is whether it's hemodynamically significant. Cardiac tamponade is the emergency: a compressing effusion that obstructs filling, treated by volume + urgent pericardiocentesis — NEVER diuresis or vasodilators, which drop the preload the obstructed heart depends on.
Key discriminators: pleuritic chest pain better sitting forward + friction rub + diffuse ST-elevation/PR-depression → acute pericarditis · effusion on echo without compression, often incidental/chronic → pericardial effusion · hypotension + JVD + muffled heart sounds (Beck's triad) + pulsus paradoxus + RA/RV diastolic collapse on echo → cardiac tamponade · tearing pain/hemopericardium/recent procedure → think dissection or iatrogenic cause
Initial Sx/Data (all comers): chest pain (pleuritic, positional), dyspnea; exam — friction rub, JVD, distant heart sounds, pulsus paradoxus, hypotension · ECG (diffuse ST-elevation + PR-depression in pericarditis; electrical alternans/low voltage with large effusion), echo (effusion size, tamponade physiology — RA/RV diastolic collapse, IVC plethora, respirophasic flow), troponin (myopericarditis), CXR, CRP/ESR, CBC, BMP · (don't anchor — the diffuse ST-elevation of pericarditis can be mistaken for STEMI and vice versa)
Neg (don't-miss):denies STEMI mistaken for pericarditis (regional ST-elevation with reciprocal changes → cath, not NSAIDs) · denies tamponade (the effusion that's compressing → drain, don't diurese) · denies myocarditis/myopericarditis (troponin rise, LV dysfunction) · denies aortic dissection or free-wall rupture causing hemopericardium (pericardiocentesis alone can be harmful → surgery) · denies purulent/bacterial or malignant effusion needing specific therapy
DDx: acute pericarditis · pericardial effusion · cardiac tamponade · (also: myopericarditis, constrictive pericarditis, STEMI, aortic dissection with hemopericardium, malignant/uremic/purulent effusion, post-cardiac-injury syndrome)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology (effusion assessment, tamponade, pericardiocentesis, constriction) · CICU (tamponade, hemodynamic instability) · Cardiac surgery (hemopericardium, surgical drainage/window, dissection) · relevant specialty for cause (ID, rheumatology, oncology, nephrology)
Acute pericarditis
– Confirm: ≥2 of — pleuritic positional chest pain (better leaning forward), friction rub, diffuse ST-elevation/PR-depression, new/worsening effusion; EXCLUDE MI and check troponin for myopericarditis
– Rx: NSAID — ibuprofen 600–800 mg PO TID (or aspirin 750–1000 mg PO TID, preferred post-MI) for ~1–2 weeks then taper, WITH a PPI (omeprazole 20–40 mg PO daily) for gastroprotection; PLUS colchicine 0.5 mg PO BID (0.5 mg daily if <70 kg) for ~3 months (reduces recurrence); activity restriction until symptom + biomarker resolution
– Caveats: AVOID anticoagulation if possible (hemorrhagic effusion risk); reserve corticosteroids for refractory/specific causes (autoimmune, uremic, contraindication to NSAID); treat the underlying cause
– Monitor / daily labs: symptom response, CRP trend, repeat ECG/echo if effusion; daily BMP if hospitalized
– Dispo: most outpatient with low-risk features; admit if high-risk (fever, large effusion, tamponade, troponin rise, immunosuppression, anticoagulation, failure of NSAID); cardiology follow-up; med rec
– NSAID + colchicine is the backbone, and colchicine is the part that prevents recurrence — don't skip it. Always check a troponin to catch myopericarditis, and avoid anticoagulation. After an MI, use aspirin rather than other NSAIDs.
Pericardial effusion
– Confirm: effusion on echo — characterize size, chronicity, and (critically) hemodynamic significance; assess for early tamponade physiology
– Rx: no compression → treat the underlying cause (viral, autoimmune, uremic → dialysis, malignant, hypothyroid, infectious), serial echo surveillance, often no drainage; large/symptomatic or diagnostic need (suspected purulent/malignant/TB) → pericardiocentesis with fluid analysis (cell count, cytology, cultures, AFB, adenosine deaminase); avoid volume depletion in a large effusion
– Monitor / daily labs: serial echo, vitals for early tamponade, daily BMP; pericardial fluid studies if drained
– Dispo: manage the cause; cardiology follow-up; specialty referral by etiology; med rec
– Effusion size matters less than whether it's compressing the heart — a small, fast-accumulating effusion can tamponade while a huge chronic one doesn't. Drain for tamponade or for diagnosis (purulent, malignant, TB), and otherwise treat the cause and watch.
Cardiac tamponade
– Confirm: hypotension + elevated JVP + muffled heart sounds (Beck's triad), pulsus paradoxus >10 mmHg, echo — RA/RV diastolic collapse, IVC plethora, respirophasic transvalvular flow variation; obstructive shock physiology
– Rx (emergency): URGENT pericardiocentesis (echo-guided) — definitive; while preparing, give IV fluids (volume) to augment preload and maintain filling; vasopressors (norepinephrine) as a temporizing bridge if hypotensive; do NOT give diuretics or vasodilators and avoid intubation/positive-pressure if possible (all reduce the preload the obstructed heart depends on and can precipitate arrest); surgical drainage/window if recurrent or not amenable
– Monitor / daily labs: CICU, continuous hemodynamics, post-drainage echo, pericardial fluid analysis, daily BMP/CBC
– Dispo: treat the cause of the effusion; cardiology/surgery; recurrence prevention; follow-up echo; med rec
– Tamponade is preload-dependent obstructive shock — fill the tank and drain the sac. The lethal reflex is to treat the hypotension and JVD as heart failure and reach for a diuretic or vasodilator, which drops preload and can cause arrest. If it's hemopericardium from dissection or rupture, the patient needs the OR, not just a needle.
108. Pericardial Disease
complete reference · the spectrum from acute pericarditis to effusion to tamponade · the preload-dependent physiology of compression · a dosed, bulleted plan per entity · Full Card
Approach — Inflamed Pericardium, Fluid in the Sac, or a Compressed Heart
Name the syndrome along a spectrum: an inflamed pericardium (pericarditis), fluid in the sac (effusion), or fluid compressing the heart (tamponade). The same process can sit anywhere on this line, so the bedside question is whether the heart is being compressed.
Acute pericarditis is an anti-inflammatory problem, but myocardial infarction and myocarditis must be excluded first. A pericardial effusion may be incidental or large, and the issue is whether it is hemodynamically significant.
Cardiac tamponade is the emergency — a compressing effusion that obstructs filling, treated by volume and urgent pericardiocentesis and never by diuresis or vasodilators, which drop the preload the obstructed heart depends on.
Key discriminators: pleuritic chest pain relieved by sitting forward with a friction rub and diffuse ST-elevation and PR-depression is acute pericarditis; an effusion on echo without compression is a pericardial effusion; and hypotension with JVD and muffled heart sounds (Beck's triad), pulsus paradoxus, and RA or RV diastolic collapse on echo is tamponade.
Initial Symptoms / Data (all comers)
Chest pain (pleuritic and positional) and dyspnea, with an exam for a friction rub, JVD, distant heart sounds, pulsus paradoxus, and hypotension.
An ECG (diffuse ST-elevation with PR-depression in pericarditis; electrical alternans or low voltage with a large effusion), an echocardiogram (effusion size and tamponade physiology — RA and RV diastolic collapse, IVC plethora, respirophasic flow variation), troponin (for myopericarditis), CXR, CRP and ESR, CBC, and BMP.
Neg (don't-miss)
Pt denies a STEMI mistaken for pericarditis (regional ST-elevation with reciprocal changes means the cath lab, not NSAIDs) and denies tamponade (the compressing effusion needs drainage, not diuresis).
Pt denies myocarditis or myopericarditis (a troponin rise with LV dysfunction) and denies aortic dissection or free-wall rupture causing hemopericardium (where pericardiocentesis alone can be harmful and surgery is needed).
Pt denies a purulent, bacterial, or malignant effusion needing specific therapy.
DDx
Acute pericarditis · pericardial effusion · cardiac tamponade · (also myopericarditis, constrictive pericarditis, STEMI, aortic dissection with hemopericardium, malignant, uremic, or purulent effusion, and post-cardiac-injury syndrome)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology (effusion assessment, tamponade, pericardiocentesis, constriction) · CICU (tamponade, hemodynamic instability) · Cardiac surgery (hemopericardium, surgical drainage or window, dissection) · the relevant specialty for the cause (ID, rheumatology, oncology, nephrology)
Acute pericarditis
Confirm: at least two of pleuritic positional chest pain (better leaning forward), a friction rub, diffuse ST-elevation with PR-depression, and a new or worsening effusion; exclude MI and check a troponin to detect myopericarditis.
Therapy: an NSAID — ibuprofen 600–800 mg PO three times daily (or aspirin 750–1000 mg PO three times daily, preferred after an MI) for about 1–2 weeks then a taper — together with a PPI (omeprazole 20–40 mg PO daily) for gastroprotection; plus colchicine 0.5 mg PO twice daily (0.5 mg daily if under 70 kg) for about 3 months to reduce recurrence; and activity restriction until symptoms and biomarkers resolve.
Caveats: avoid anticoagulation where possible (risk of a hemorrhagic effusion); reserve corticosteroids for refractory cases or specific causes (autoimmune, uremic, or an NSAID contraindication), as they increase recurrence; and treat the underlying cause.
Monitoring / daily labs: the symptom response, the CRP trend, a repeat ECG and echo if an effusion is present, and a daily BMP if hospitalized.
Mobility / consults / disposition: most low-risk patients are managed outpatient; admit high-risk features (fever, a large effusion, tamponade, a troponin rise, immunosuppression, anticoagulation, or NSAID failure); cardiology follow-up; medication reconciliation.
Pericardial effusion
Confirm: an effusion on echo, characterized by size, chronicity, and (critically) hemodynamic significance, with an assessment for early tamponade physiology.
Therapy: if there is no compression, treat the underlying cause (viral, autoimmune, uremic with dialysis, malignant, hypothyroid, or infectious) with serial echo surveillance and often no drainage; if it is large and symptomatic or there is a diagnostic need (suspected purulent, malignant, or tuberculous effusion), perform pericardiocentesis with fluid analysis (cell count, cytology, cultures, AFB, adenosine deaminase); and avoid volume depletion in a large effusion.
Monitoring / daily labs: serial echo, vital signs for early tamponade, a daily BMP, and pericardial fluid studies if drained.
Mobility / consults / disposition: manage the cause; cardiology follow-up; specialty referral by etiology; medication reconciliation.
Cardiac tamponade
Confirm: hypotension with an elevated JVP and muffled heart sounds (Beck's triad), pulsus paradoxus greater than 10 mmHg, and echo findings of RA and RV diastolic collapse, IVC plethora, and respirophasic transvalvular flow variation — obstructive shock physiology.
Therapy (emergency): urgent echo-guided pericardiocentesis as the definitive treatment; while preparing, give IV fluids to augment preload and maintain filling, and use a vasopressor (norepinephrine) as a temporizing bridge if hypotensive; do not give diuretics or vasodilators and avoid intubation and positive-pressure ventilation if possible, since all reduce the preload the obstructed heart depends on and can precipitate arrest; and arrange surgical drainage or a pericardial window if the effusion is recurrent or not amenable to needle drainage.
Monitoring / daily labs: CICU care with continuous hemodynamic monitoring, a post-drainage echo, pericardial fluid analysis, and a daily BMP and CBC.
Mobility / consults / disposition: treat the cause of the effusion; cardiology and surgery involvement; recurrence prevention; a follow-up echo; medication reconciliation.
Red Flags
Hypotension, JVD, and muffled heart sounds with pulsus paradoxus → tamponade; fill and drain, never diurese
Regional ST-elevation with reciprocal changes → a STEMI, not pericarditis; activate the cath lab
A troponin rise with LV dysfunction → myopericarditis; admit and restrict activity
Hemopericardium with tearing pain or after a procedure → dissection or free-wall rupture; surgery, not a needle alone
A purulent effusion or sepsis → bacterial pericarditis; drainage and antibiotics
Senior IM Resident Pearls
Tamponade is preload-dependent obstructive shock. Fill the tank and drain the sac — the lethal reflex is to read the hypotension and JVD as heart failure and reach for a diuretic or vasodilator.
Colchicine is the part that prevents recurrence. NSAID plus colchicine is the backbone of acute pericarditis; don't skip the colchicine.
Always check a troponin in pericarditis. It catches the myopericarditis that changes the disposition and the activity restriction.
Effusion size matters less than the rate of accumulation. A small, fast effusion can tamponade while a huge chronic one is tolerated.
Pericarditis versus STEMI is a classic ECG trap. Diffuse ST-elevation with PR-depression is pericarditis; regional elevation with reciprocal change is an occlusion.
Avoid anticoagulation in acute pericarditis. It raises the risk of a hemorrhagic effusion.
Common mistake: diuresing a tamponade because the JVD and hypotension look like heart failure, or starting NSAIDs on a STEMI mislabeled as pericarditis.
Cardiology — Venous Thromboembolism
109. Venous Thromboembolic Disease
PE risk-stratified by RV strain + hemodynamics: high-risk/massive (lyse) · intermediate/submassive (anticoagulate + watch) · low-risk (DOAC, often outpatient) · DVT with RV strain · separate dosed plans · Super Compact
Approach — once PE is confirmed, the entire management decision is driven by risk stratification: hemodynamics first, then RV strain. Everyone gets anticoagulated unless contraindicated; the question is who needs more than anticoagulation. Hemodynamic instability (sustained hypotension/shock) = high-risk (massive) PE → reperfusion (systemic thrombolysis). Normotensive but with RV dysfunction (echo/CT) and/or troponin/BNP elevation = intermediate-risk (submassive) → anticoagulate, monitor closely, have a rescue-reperfusion plan. Normotensive, no RV strain, low score = low-risk → anticoagulate (DOAC), consider outpatient. The RV is the organ that fails and kills in PE — protect it: avoid over-aggressive fluids, support perfusion.
Key discriminators: sustained hypotension (SBP <90 / vasopressors) or arrest → high-risk/massive · normotensive + RV dysfunction on echo/CT and/or ↑troponin/BNP → intermediate/submassive · normotensive + no RV strain + normal biomarkers + low PESI/sPESI → low-risk · proximal DVT + signs of RV strain or concurrent PE → DVT with RV strain (treat as the corresponding PE)
Initial Sx/Data (all comers): dyspnea, pleuritic chest pain, tachycardia, hypoxia, syncope (ominous), leg swelling/pain; assess hemodynamics + RV (JVD, loud P2) · CT pulmonary angiography (or V/Q); ECG (sinus tach, S1Q3T3, RV strain), troponin + BNP (RV strain markers), echo (RV size/function, McConnell's), lower-extremity Doppler; ABG; risk score (PESI/sPESI), bleeding risk · (don't anchor — syncope or unexplained hypoxia/tachycardia can be the only clue; protect the RV)
Neg (don't-miss):denies missed high-risk/massive PE (the hypotensive PE that needs lysis now, not just heparin) · denies a contraindication to anticoagulation/thrombolysis (active bleed, recent surgery/stroke) · denies over-resuscitating the failing RV with fluids (worsens RV distension/output) · denies missing the deteriorating submassive PE (RV can fail hours later → rescue lysis) · denies anchoring on PE when it's ACS/dissection/tamponade/sepsis
DDx: high-risk (massive) PE · intermediate-risk (submassive) PE · low-risk PE · DVT with RV strain · (also: ACS, aortic dissection, tamponade, pneumonia, pneumothorax, decompensated HF, chronic thromboembolic PH)
Plan — by Diagnosis
CONSULT (as relevant): Pulmonary Embolism Response Team (PERT) / Pulmonology / Cardiology (risk stratification, reperfusion decisions) · CICU (massive PE, shock, RV failure) · Interventional radiology / cardiology (catheter-directed therapy, embolectomy) · Hematology (provoking factors, anticoagulation in complex cases)
High-risk (massive) PE
– Confirm: PE + sustained hypotension (SBP <90 ≥15 min or needing vasopressors) or cardiac arrest — obstructive shock from RV failure
– Reperfuse (emergency): systemic thrombolysis — alteplase 100 mg IV over 2 h (unless contraindicated); if thrombolysis contraindicated/fails → catheter-directed therapy or surgical embolectomy; full-dose anticoagulation — IV unfractionated heparin (80 U/kg bolus then infusion, titrated; easily held/reversed periprocedurally)
– Support the RV: norepinephrine 0.05–0.5 mcg/kg/min for hypotension; cautious/limited fluids (~250–500 mL, avoid over-filling the distended RV); dobutamine for low output; correct hypoxia/avoid acidosis (lower PVR); activate PERT
– Monitor / daily labs: CICU, arterial line, continuous hemodynamics, serial troponin/lactate, daily CBC/BMP/coags
– Dispo: transition to oral anticoagulation when stable; workup for provoking factors; cardiology/pulmonary + hematology follow-up; med rec
– Massive PE is obstructive shock — the hypotensive patient needs reperfusion (lysis) now, not just heparin. Use heparin (not a DOAC/LMWH) so it can be held for procedures, support the RV with norepinephrine, and be sparing with fluids — flooding the distended RV makes it worse.
Intermediate-risk (submassive) PE
– Confirm: PE, normotensive, WITH RV dysfunction (echo/CT — RV/LV ratio >0.9) and/or elevated troponin/BNP; intermediate-high = both RV dysfunction + positive biomarkers
– Rx: full-dose anticoagulation promptly — LMWH (enoxaparin 1 mg/kg SC q12h) or IV heparin, or a DOAC in stable selected patients; monitored setting (step-down/ICU) with a clear rescue plan — escalate to reperfusion (systemic lysis, or catheter-directed/reduced-dose lysis) if hemodynamic deterioration; PERT involvement; routine upfront full-dose systemic lysis NOT recommended (bleeding risk)
– Support: O2; avoid over-aggressive fluids; treat hypoxia
– Monitor / daily labs: continuous telemetry/hemodynamics, serial vitals + troponin, daily CBC/BMP; watch for the RV failing over hours
– Dispo: transition to oral anticoagulation; provoking-factor workup; pulmonary/cardiology + hematology follow-up; med rec
– The submassive PE is the one that fools you — normotensive now, but the RV can fail hours later. Anticoagulate, watch in a monitored bed, and have the rescue-lysis trigger defined in advance. Don't reflexively give full-dose lytics upfront (bleeding), but don't be caught flat-footed when they crash.
Low-risk PE
– Confirm: PE, normotensive, NO RV dysfunction, normal troponin/BNP, low PESI/sPESI; no high-risk features
– Rx: anticoagulation alone — a DOAC preferred: apixaban 10 mg PO BID ×7 days then 5 mg PO BID, or rivaroxaban 15 mg PO BID ×21 days then 20 mg PO daily (no parenteral lead-in needed); LMWH/warfarin if DOAC unsuitable (e.g. antiphospholipid, severe renal impairment, pregnancy); assess for outpatient management (Hestia criteria/sPESI=0) if reliable, no high bleeding risk, good support
– Monitor: confirm no RV strain before low-risk pathway; ensure follow-up
– Dispo: outpatient if criteria met with anticoagulation education + early follow-up; provoking-factor + duration-of-therapy plan (provoked ~3 months vs unprovoked/cancer extended); med rec
– A normotensive PE with a normal RV and biomarkers and a low PESI is the one many patients can be treated for at home on a DOAC — but only after you've confirmed there's no RV strain. The risk stratification is what earns the outpatient pathway, not just "the PE looks small."
DVT with RV strain (or concurrent PE)
– Confirm: proximal DVT on Doppler with signs of RV strain or documented concurrent PE; treat as the corresponding PE risk category
– Rx: full-dose anticoagulation (DOAC, LMWH, or heparin per acuity/stability); risk-stratify the PE component (RV function, biomarkers, hemodynamics) and escalate to reperfusion if high-risk; IVC filter ONLY if anticoagulation is contraindicated (e.g. active bleeding) — not as an add-on to anticoagulation; consider catheter-directed therapy for extensive iliofemoral DVT (limb-threatening phlegmasia)
– Monitor / daily labs: hemodynamics, RV markers, daily CBC/BMP, limb perfusion
– Dispo: anticoagulation + duration plan; compression for post-thrombotic syndrome; hematology if unprovoked/recurrent; follow-up; med rec
– A proximal DVT with any RV strain is functionally a PE — stratify and treat it as one. The IVC filter is for the patient who truly can't be anticoagulated, not a belt-and-suspenders addition; anticoagulation is the real therapy.
109. Venous Thromboembolic Disease
complete reference · PE stratified by hemodynamics and RV strain · massive, submassive, and low-risk PE plus DVT with RV strain · protecting the right ventricle · a dosed, bulleted plan per category · Full Card
Approach — Risk-Stratify by Hemodynamics, Then RV Strain
Once PE is confirmed, the entire management decision is driven by risk stratification: hemodynamics first, then RV strain. Everyone gets anticoagulated unless contraindicated; the question is who needs more than anticoagulation.
Hemodynamic instability (sustained hypotension or shock) is high-risk (massive) PE and warrants reperfusion with systemic thrombolysis. A normotensive patient with RV dysfunction on imaging and/or elevated troponin or BNP is intermediate-risk (submassive) and warrants anticoagulation, close monitoring, and a rescue-reperfusion plan. A normotensive patient with no RV strain, normal biomarkers, and a low score is low-risk and warrants anticoagulation with consideration of outpatient management.
The right ventricle is the organ that fails and kills in PE — protect it by avoiding over-aggressive fluids and supporting perfusion.
Key discriminators: sustained hypotension or arrest is high-risk; normotension with RV dysfunction and/or positive biomarkers is intermediate or submassive; normotension with no RV strain and a low PESI is low-risk; and a proximal DVT with RV strain or concurrent PE is treated as the corresponding PE category.
Initial Symptoms / Data (all comers)
Dyspnea, pleuritic chest pain, tachycardia, hypoxia, syncope (an ominous sign), and leg swelling or pain, with an assessment of hemodynamics and the RV (JVD, a loud P2).
CT pulmonary angiography (or a V/Q scan); an ECG (sinus tachycardia, an S1Q3T3 pattern, RV strain); troponin and BNP as RV-strain markers; an echocardiogram (RV size and function, McConnell's sign); lower-extremity Doppler; an ABG; and a risk score (PESI or sPESI) with an assessment of bleeding risk.
Neg (don't-miss)
Pt denies a missed high-risk or massive PE (the hypotensive PE that needs lysis now, not just heparin) and denies a contraindication to anticoagulation or thrombolysis (active bleeding, recent surgery or stroke).
Pt denies over-resuscitating the failing RV with fluids (which worsens RV distension and output) and denies missing the deteriorating submassive PE (the RV can fail hours later, requiring rescue lysis).
Pt denies anchoring on PE when the picture is actually ACS, aortic dissection, tamponade, or sepsis.
DDx
High-risk (massive) PE · intermediate-risk (submassive) PE · low-risk PE · DVT with RV strain · (also ACS, aortic dissection, cardiac tamponade, pneumonia, pneumothorax, decompensated heart failure, and chronic thromboembolic pulmonary hypertension)
Plan — by Diagnosis
CONSULT (as relevant): a Pulmonary Embolism Response Team (PERT), Pulmonology, or Cardiology (risk stratification and reperfusion decisions) · CICU (massive PE, shock, RV failure) · Interventional radiology or cardiology (catheter-directed therapy, embolectomy) · Hematology (provoking factors, anticoagulation in complex cases)
High-risk (massive) PE
Confirm: PE with sustained hypotension (SBP under 90 for at least 15 minutes or requiring vasopressors) or cardiac arrest — obstructive shock from RV failure.
Reperfusion (emergency): systemic thrombolysis — alteplase 100 mg IV over 2 hours unless contraindicated; if thrombolysis is contraindicated or fails, catheter-directed therapy or surgical embolectomy; and full-dose anticoagulation with IV unfractionated heparin (an 80 U/kg bolus then a titrated infusion, chosen because it can be held and reversed periprocedurally).
RV support: norepinephrine 0.05–0.5 mcg/kg/min for hypotension; cautious, limited fluids (about 250–500 mL, avoiding over-filling the distended RV); dobutamine for low output; correction of hypoxia and avoidance of acidosis to lower pulmonary vascular resistance; and activation of the PERT.
Monitoring / daily labs: CICU care with an arterial line, continuous hemodynamics, serial troponin and lactate, and a daily CBC, BMP, and coagulation studies.
Mobility / consults / disposition: transition to oral anticoagulation when stable; a workup for provoking factors; cardiology, pulmonary, and hematology follow-up; medication reconciliation.
Intermediate-risk (submassive) PE
Confirm: PE in a normotensive patient with RV dysfunction (an RV-to-LV ratio over 0.9 on echo or CT) and/or an elevated troponin or BNP, with intermediate-high risk defined by both RV dysfunction and positive biomarkers.
Therapy: prompt full-dose anticoagulation — LMWH (enoxaparin 1 mg/kg SC every 12 hours) or IV heparin, or a DOAC in stable, selected patients — in a monitored setting (a step-down unit or ICU) with a clear rescue plan to escalate to reperfusion (systemic thrombolysis, or catheter-directed or reduced-dose lysis) if there is hemodynamic deterioration, with PERT involvement; routine upfront full-dose systemic lysis is not recommended because of bleeding risk.
Support: O2, avoidance of over-aggressive fluids, and treatment of hypoxia.
Monitoring / daily labs: continuous telemetry and hemodynamic monitoring, serial vitals and troponin, and a daily CBC and BMP, watching for the RV failing over hours.
Mobility / consults / disposition: transition to oral anticoagulation; a provoking-factor workup; pulmonary, cardiology, and hematology follow-up; medication reconciliation.
Low-risk PE
Confirm: PE in a normotensive patient with no RV dysfunction, normal troponin and BNP, and a low PESI or sPESI, without high-risk features.
Therapy: anticoagulation alone, with a DOAC preferred — apixaban 10 mg PO twice daily for 7 days then 5 mg PO twice daily, or rivaroxaban 15 mg PO twice daily for 21 days then 20 mg PO daily (no parenteral lead-in needed) — or LMWH or warfarin if a DOAC is unsuitable (antiphospholipid syndrome, severe renal impairment, pregnancy); and assessment for outpatient management (Hestia criteria or sPESI of 0) if the patient is reliable, has no high bleeding risk, and has good support.
Monitoring: confirm there is no RV strain before committing to the low-risk pathway, and ensure follow-up.
Mobility / consults / disposition: outpatient management if criteria are met, with anticoagulation education and early follow-up; a provoking-factor and duration-of-therapy plan (about 3 months for a provoked event, extended for unprovoked or cancer-associated disease); medication reconciliation.
DVT with RV strain (or concurrent PE)
Confirm: a proximal DVT on Doppler with signs of RV strain or a documented concurrent PE, treated as the corresponding PE risk category.
Therapy: full-dose anticoagulation (a DOAC, LMWH, or heparin per acuity and stability); risk-stratification of the PE component (RV function, biomarkers, hemodynamics) with escalation to reperfusion if high-risk; an IVC filter only if anticoagulation is contraindicated (such as active bleeding) rather than as an addition to anticoagulation; and consideration of catheter-directed therapy for extensive iliofemoral DVT (limb-threatening phlegmasia).
Monitoring / daily labs: hemodynamics, RV markers, a daily CBC and BMP, and limb perfusion.
Mobility / consults / disposition: anticoagulation with a duration plan; compression for post-thrombotic syndrome; hematology if unprovoked or recurrent; follow-up; medication reconciliation.
Red Flags
Sustained hypotension or arrest with PE → massive PE; systemic thrombolysis now, not heparin alone
Syncope or unexplained hypoxia and tachycardia → may be the only clue to a significant PE
A normotensive PE with RV dysfunction and a positive troponin → intermediate-high risk that can deteriorate; a monitored bed and a rescue plan
Over-resuscitating with fluids → worsens the distended, failing RV
An IVC filter used instead of anticoagulation when anticoagulation is actually feasible → undertreatment
Senior IM Resident Pearls
Hemodynamics first, then RV strain. The hypotensive PE needs reperfusion now; the normotensive PE is sorted by RV function and biomarkers.
The submassive PE is the one that fools you. Normotensive now, but the RV can fail hours later — monitor it and define the rescue-lysis trigger in advance.
Protect the RV. Be sparing with fluids and support the systemic pressure with norepinephrine; flooding the distended RV worsens its output.
Use heparin when reperfusion is on the table. It can be held and reversed for thrombolysis or a procedure, unlike a DOAC or LMWH.
Low-risk status earns the outpatient pathway. A normal RV, normal biomarkers, and a low PESI — confirmed — let many patients go home on a DOAC.
An IVC filter is for the patient who can't be anticoagulated. It's not an add-on to anticoagulation.
Common mistake: giving only heparin to a hypotensive massive PE, or sending home a "small" PE without confirming the RV is normal, or flooding the failing RV with fluids.
Cardiology — Cardiac Devices
110. Device-Related Admissions
pacemaker malfunction · appropriate vs inappropriate ICD shock · electrical storm · lead issues · CIED infection · separate dosed plans per problem · Super Compact
Approach — figure out what the device did or failed to do, and whether the problem is the device, the leads, or the heart. Cardiac implantable electronic device (CIED) admissions split into a few buckets: a pacemaker not pacing/sensing correctly, an ICD that shocked (appropriately for a real arrhythmia, or inappropriately), repeated shocks (electrical storm — an emergency), a lead problem, or a device infection. The two universal early moves: get device interrogation (the device tells you what happened) and place a magnet correctly when needed — over a pacemaker a magnet forces asynchronous pacing; over an ICD a magnet suspends shock therapy (without affecting pacing). And always ask: is the patient pacemaker-dependent?
Key discriminators: bradycardia/pauses/symptoms with failure to pace/capture/sense on interrogation → pacemaker malfunction · ICD shock that terminated a real VT/VF on interrogation → appropriate shock (find the trigger) · shock during AF/SVT/sinus tach or from oversensing/lead noise → inappropriate shock · ≥3 sustained VT/VF episodes (or shocks) in 24 h → electrical storm (emergency) · abnormal lead impedance/sensing → lead failure (high impedance = fracture; low = insulation breach) · pocket erythema/drainage/systemic infection + bacteremia → CIED infection
Initial Sx/Data (all comers): syncope/presyncope, palpitations, shocks (how many), pocket pain/redness, fever; pacemaker dependence, device type/indication · DEVICE INTERROGATION (essential), 12-lead ECG + telemetry, CXR (lead position/fracture), BMP/Mg, troponin if ischemia, blood cultures + pocket exam if infection; echo if vegetation/dysfunction · magnet + external pacing/defibrillation pads available · (don't anchor — interrogate before assuming; an inappropriate-shock storm and a true VT storm are managed very differently)
Neg (don't-miss): denies electrical storm (recurrent shocks needing urgent suppression + EP) · denies a CIED infection seeding the bloodstream (needs complete system extraction, not just antibiotics) · denies pacemaker dependence with failure to capture (needs immediate external/transvenous pacing) · denies a reversible trigger of the arrhythmia (ischemia, electrolytes, HF, QT drugs) · denies treating an inappropriate shock as if the device worked correctly
DDx: pacemaker malfunction · appropriate ICD shock · inappropriate ICD shock · electrical storm · lead failure · CIED infection · (also: device-related endocarditis, Twiddler's syndrome, pacemaker-mediated tachycardia, battery depletion/EOL, pocket hematoma)
Plan — by Diagnosis
CONSULT (as relevant): Electrophysiology / Cardiology (device interrogation, reprogramming, lead/generator revision, storm) · CICU (electrical storm, hemodynamic instability) · ID + Cardiac surgery / EP (CIED infection — extraction)
Pacemaker malfunction
– Confirm: symptoms (syncope, bradycardia, pauses) + interrogation showing failure to pace, capture, or sense (or oversensing); establish pacemaker dependence
– Rx: EP/device interrogation to define + reprogram; if pacemaker-dependent with failure to capture/pace → immediate transcutaneous pacing then transvenous pacing as a bridge; a magnet over the pacemaker forces asynchronous pacing (useful for oversensing-induced inhibition); treat reversible causes (electrolytes, ischemia, raised capture thresholds, drugs); generator/lead revision for hardware failure or battery depletion
– Monitor / daily labs: continuous telemetry, daily BMP/Mg, repeat interrogation post-reprogram
– Dispo: EP follow-up, device clinic; med rec; education on device
– The first move is interrogation, and the critical question is pacemaker-dependence — a dependent patient with failure to capture needs external pacing immediately. A magnet forces asynchronous pacing, which rescues a device being inappropriately inhibited by oversensing.
Appropriate ICD shock
– Confirm: interrogation shows the ICD detected + terminated a genuine VT/VF — the device did its job
– Rx: find + treat the arrhythmia trigger — ischemia (troponin, ECG), electrolyte derangement (K/Mg), HF decompensation, QT-prolonging/proarrhythmic drugs, progression of cardiomyopathy; antiarrhythmics (amiodarone, beta-blockers) to reduce recurrence; optimize GDMT; EP for VT ablation if recurrent
– Monitor / daily labs: telemetry, serial troponin, daily BMP/Mg, echo for new dysfunction
– Dispo: EP follow-up; reassess substrate + GDMT; driving counseling per guidance; med rec
– A single appropriate shock means the device saved the patient — now your job is to find why the heart fired: ischemia, electrolytes, HF, or proarrhythmic drugs. Fixing the trigger and optimizing therapy prevents the next one.
Inappropriate ICD shock
– Confirm: interrogation shows shock for a non-life-threatening rhythm — rapid AF/SVT/sinus tach conducted fast, or oversensing (T-wave, lead noise, electromagnetic interference, lead fracture)
– Rx: EP reprogramming (rate cutoffs, discriminators, sensing); rate control of AF/SVT (beta-blocker; AV-nodal agents per rhythm); if a lead fracture/noise is the cause → lead revision; a magnet over the ICD suspends shock therapy as a temporizing measure for ongoing inappropriate shocks (continuous magnet or tape it on, with monitoring — it does NOT stop pacing)
– Monitor / daily labs: telemetry, interrogation, daily BMP; lead parameters
– Dispo: EP follow-up; treat underlying arrhythmia; med rec
– For inappropriate shocks, the magnet is your friend — placed over the ICD it suspends shocks (but not pacing) while EP sorts out reprogramming or a lead problem. Most inappropriate shocks are rapid AF or a fractured lead.
Electrical storm
– Confirm: ≥3 separate sustained VT/VF episodes (or appropriate shocks) within 24 h — a medical emergency
– Rx: ICU; IV antiarrhythmics — amiodarone 150 mg IV over 10 min then 1 mg/min ×6 h, 0.5 mg/min ×18 h; IV beta-blockade (sympathetic drive is central) — esmolol infusion or metoprolol, propranolol; sedation (reduces sympathetic surge; intubation/deep sedation in refractory storm); correct ischemia + electrolytes (K, Mg 2 g IV); reprogram device (anti-tachycardia pacing, raise cutoffs to reduce shocks); urgent EP for VT ablation; mechanical support if hemodynamically unstable; stellate ganglion block in refractory cases
– Monitor / daily labs: CICU continuous monitoring, serial troponin/BMP/Mg, QT, sedation depth
– Dispo: EP (ablation); optimize GDMT/antiarrhythmics; med rec
– Electrical storm is driven by a sympathetic surge — so beta-blockade and sedation are as important as amiodarone, and deep sedation can break a refractory storm. Treat the ischemic/electrolyte trigger and get EP for ablation; this is an ICU emergency.
Lead failure
– Confirm: abnormal lead parameters on interrogation — high impedance (conductor fracture), low impedance (insulation breach), sensing/pacing abnormalities, noise causing oversensing; CXR may show fracture/dislodgement
– Rx: EP evaluation; reprogram to mitigate (e.g. suspend shocks if oversensing causing inappropriate shocks — magnet as temporizing bridge); lead revision/replacement for confirmed failure; if pacemaker-dependent with lead failure → ensure backup pacing (external/transvenous)
– Monitor / daily labs: telemetry, serial interrogation, daily BMP
– Dispo: EP for lead revision; device clinic follow-up; med rec
– Lead impedance points to the failure mode: high impedance means a conductor fracture, low impedance means an insulation breach. Lead noise from a fracture is a classic cause of inappropriate shocks — and a dependent patient with a failing pacing lead needs backup pacing.
CIED infection
– Confirm: pocket signs (erythema, swelling, drainage, erosion) and/or systemic infection/bacteremia (esp Staphylococcus); TEE for lead vegetations/device-related endocarditis
– Rx: blood cultures + pocket cultures; empiric IV vancomycin (covers MRSA) → organism-directed; complete device + lead extraction is required (antibiotics alone do not clear hardware infection) — EP/cardiac surgery for percutaneous or surgical lead extraction; prolonged IV antibiotics (duration per pocket vs bloodstream vs endocarditis); reimplant on the contralateral side after clearance once indicated
– Monitor / daily labs: serial blood cultures (document clearance), daily CBC/BMP, TEE, inflammatory markers
– Dispo: ID-guided antibiotic course; staged reimplantation; ID + EP follow-up; med rec
– The non-negotiable rule in CIED infection: the entire system — generator and all leads — must come out. Antibiotics alone never clear infected hardware. Staph bacteremia in a device patient is a device infection until a TEE and extraction prove otherwise.
110. Device-Related Admissions
complete reference · pacemaker malfunction, appropriate and inappropriate ICD shocks, electrical storm, lead failure, and CIED infection · interrogate first and use the magnet correctly · a dosed, bulleted plan per problem · Full Card
Approach — What Did the Device Do, and Is the Problem the Device, the Leads, or the Heart?
Cardiac implantable electronic device admissions split into a few buckets: a pacemaker not pacing or sensing correctly, an ICD that shocked (appropriately for a real arrhythmia or inappropriately), repeated shocks (electrical storm, an emergency), a lead problem, or a device infection.
The two universal early moves are to get device interrogation (the device records exactly what happened) and to use a magnet correctly when needed — over a pacemaker a magnet forces asynchronous pacing, while over an ICD a magnet suspends shock therapy without affecting pacing.
Always ask whether the patient is pacemaker-dependent, because that changes the urgency of every pacing problem.
Key discriminators: symptomatic bradycardia or pauses with failure to pace, capture, or sense on interrogation is pacemaker malfunction; a shock that terminated a real VT or VF is an appropriate shock; a shock during AF, SVT, sinus tachycardia, or from oversensing is inappropriate; three or more sustained VT or VF episodes in 24 hours is electrical storm; abnormal lead impedance or sensing is lead failure (high impedance suggests fracture, low suggests insulation breach); and pocket or systemic infection with bacteremia is a CIED infection.
Initial Symptoms / Data (all comers)
Syncope or presyncope, palpitations, the number of shocks delivered, pocket pain or redness, and fever, with the pacemaker-dependence status and the device type and indication.
Device interrogation (essential), a 12-lead ECG and telemetry, a CXR (lead position and fracture), BMP and magnesium, troponin if ischemia is suspected, and blood cultures with a pocket exam if infection is suspected; an echocardiogram if a vegetation or device dysfunction is suspected.
A magnet and external pacing and defibrillation pads available at the bedside.
Neg (don't-miss)
Pt denies electrical storm (recurrent shocks needing urgent suppression and EP) and denies a CIED infection seeding the bloodstream (which needs complete system extraction, not just antibiotics).
Pt denies pacemaker dependence with failure to capture (which needs immediate external or transvenous pacing) and denies a reversible trigger of the arrhythmia (ischemia, electrolytes, HF, QT-prolonging drugs).
Pt denies treating an inappropriate shock as though the device functioned correctly.
DDx
Pacemaker malfunction · appropriate ICD shock · inappropriate ICD shock · electrical storm · lead failure · CIED infection · (also device-related endocarditis, Twiddler's syndrome, pacemaker-mediated tachycardia, battery depletion or end-of-life, and pocket hematoma)
Plan — by Diagnosis
CONSULT (as relevant): Electrophysiology and Cardiology (device interrogation, reprogramming, lead or generator revision, storm management) · CICU (electrical storm, hemodynamic instability) · Infectious disease with Cardiac surgery or EP (CIED infection requiring extraction)
Pacemaker malfunction
Confirm: symptoms (syncope, bradycardia, pauses) with interrogation showing failure to pace, capture, or sense (or oversensing), and establish whether the patient is pacemaker-dependent.
Therapy: EP and device interrogation to define and reprogram the problem; for a pacemaker-dependent patient with failure to capture or pace, immediate transcutaneous pacing then transvenous pacing as a bridge; a magnet over the pacemaker to force asynchronous pacing (useful when oversensing is inappropriately inhibiting output); treatment of reversible causes (electrolytes, ischemia, raised capture thresholds, drugs); and generator or lead revision for hardware failure or battery depletion.
Monitoring / daily labs: continuous telemetry, a daily BMP with magnesium, and repeat interrogation after reprogramming.
Mobility / consults / disposition: EP follow-up and a device clinic; medication reconciliation; device education.
Appropriate ICD shock
Confirm: interrogation showing the ICD detected and terminated a genuine VT or VF — the device did its job.
Therapy: find and treat the arrhythmia trigger — ischemia (troponin, ECG), electrolyte derangement (potassium, magnesium), HF decompensation, QT-prolonging or proarrhythmic drugs, or progression of the cardiomyopathy; use antiarrhythmics (amiodarone, beta-blockers) to reduce recurrence; optimize GDMT; and involve EP for VT ablation if shocks are recurrent.
Monitoring / daily labs: telemetry, serial troponin, a daily BMP with magnesium, and an echo for new dysfunction.
Mobility / consults / disposition: EP follow-up; reassess the substrate and GDMT; driving counseling per guidance; medication reconciliation.
Inappropriate ICD shock
Confirm: interrogation showing a shock for a non-life-threatening rhythm — rapidly conducted AF, SVT, or sinus tachycardia, or oversensing (T-wave oversensing, lead noise, electromagnetic interference, or a lead fracture).
Therapy: EP reprogramming (rate cutoffs, discriminators, sensing); rate control of AF or SVT (a beta-blocker, with AV-nodal agents per the rhythm); lead revision if a fracture or noise is the cause; and a magnet over the ICD to suspend shock therapy as a temporizing measure for ongoing inappropriate shocks (held continuously or taped on, with monitoring — it does not stop pacing).
Monitoring / daily labs: telemetry, interrogation, a daily BMP, and lead parameters.
Mobility / consults / disposition: EP follow-up; treat the underlying arrhythmia; medication reconciliation.
Electrical storm
Confirm: three or more separate sustained VT or VF episodes (or appropriate shocks) within 24 hours — a medical emergency.
Therapy: ICU care with IV antiarrhythmics — amiodarone 150 mg IV over 10 minutes then 1 mg/min for 6 hours and 0.5 mg/min for 18 hours; IV beta-blockade, since sympathetic drive is central (an esmolol infusion, or metoprolol or propranolol); sedation to reduce the sympathetic surge (with intubation and deep sedation in a refractory storm); correction of ischemia and electrolytes (potassium, and magnesium 2 g IV); device reprogramming (anti-tachycardia pacing, raised cutoffs to reduce shocks); urgent EP for VT ablation; mechanical circulatory support if hemodynamically unstable; and a stellate ganglion block in refractory cases.
Monitoring / daily labs: CICU continuous monitoring, serial troponin, BMP, and magnesium, the QT interval, and sedation depth.
Mobility / consults / disposition: EP for ablation; optimize GDMT and antiarrhythmics; medication reconciliation.
Lead failure
Confirm: abnormal lead parameters on interrogation — high impedance (a conductor fracture), low impedance (an insulation breach), or sensing and pacing abnormalities with noise causing oversensing; a CXR may show a fracture or dislodgement.
Therapy: EP evaluation; reprogramming to mitigate (for example suspending shocks if oversensing is causing inappropriate shocks, with a magnet as a temporizing bridge); lead revision or replacement for a confirmed failure; and for a pacemaker-dependent patient with lead failure, ensuring backup pacing (external or transvenous).
Monitoring / daily labs: telemetry, serial interrogation, and a daily BMP.
Mobility / consults / disposition: EP for lead revision; device clinic follow-up; medication reconciliation.
CIED infection
Confirm: pocket signs (erythema, swelling, drainage, erosion) and/or systemic infection or bacteremia (especially Staphylococcus), with a TEE for lead vegetations or device-related endocarditis.
Therapy: blood cultures and pocket cultures; empiric IV vancomycin (covering MRSA) transitioned to organism-directed therapy; complete device and lead extraction, which is required because antibiotics alone do not clear hardware infection (EP or cardiac surgery for percutaneous or surgical lead extraction); a prolonged IV antibiotic course (duration depending on whether the infection is pocket-confined, bloodstream, or endocarditis); and reimplantation on the contralateral side after clearance once still indicated.
Monitoring / daily labs: serial blood cultures to document clearance, a daily CBC and BMP, a TEE, and inflammatory markers.
Mobility / consults / disposition: an ID-guided antibiotic course; staged reimplantation; ID and EP follow-up; medication reconciliation.
Red Flags
Three or more shocks or VT/VF episodes in 24 hours → electrical storm; an ICU emergency needing beta-blockade, sedation, and EP
A pacemaker-dependent patient with failure to capture → immediate external then transvenous pacing
Staphylococcal bacteremia in a device patient → a CIED infection until a TEE and extraction prove otherwise
Pocket erythema, drainage, or erosion → device infection requiring complete system extraction
Repeated inappropriate shocks → apply a magnet to suspend therapy while EP reprograms or revises the lead
Senior IM Resident Pearls
Interrogate first. The device records exactly what happened — don't guess at an appropriate versus inappropriate shock or a pacing failure before reading it out.
Know what the magnet does. Over a pacemaker it forces asynchronous pacing; over an ICD it suspends shocks without stopping pacing — invaluable for ongoing inappropriate shocks.
Pacemaker dependence changes everything. A dependent patient with failure to capture needs external pacing immediately.
Electrical storm is a sympathetic problem. Beta-blockade and sedation are as important as amiodarone, and deep sedation can break a refractory storm.
Lead impedance localizes the failure. High impedance is a conductor fracture; low impedance is an insulation breach; lead noise causes inappropriate shocks.
Infected hardware must come out. The whole system — generator and all leads — is extracted; antibiotics alone never cure it.
Common mistake: treating a CIED infection with antibiotics alone, or managing an inappropriate-shock storm as if it were true VT.
Cardiology — Infective Endocarditis
111. Infective Endocarditis
native valve · prosthetic valve · CIED (device-lead) endocarditis · cultures before antibiotics, echo, and know the surgical indications · separate dosed plans · Super Compact
Approach — secure the microbiology before antibiotics, image the valve, and know who needs surgery. The diagnosis rests on the modified Duke criteria, built from two pillars: persistently positive blood cultures with a typical organism and echocardiographic evidence (vegetation, abscess, new regurgitation, prosthetic dehiscence). So the two foundational moves are drawing ≥3 sets of blood cultures from separate sites before starting antibiotics (unless septic shock) and getting an echo (TTE then TEE — TEE essential for prosthetic valves and CIED). Then ask native vs prosthetic vs device — each has a distinct antibiotic course and surgical threshold. The questions that change management: heart failure, perivalvular abscess (new AV block), persistent bacteremia, large mobile vegetation, embolization, fungal/resistant organism → surgery.
Key discriminators: native valve + vegetation + typical organism (Staph aureus, viridans strep, enterococcus) → native valve IE · prosthetic valve (early <1 yr = nosocomial staph/coagulase-negative; late = community) → prosthetic valve IE (higher surgical threshold) · pacemaker/ICD + lead vegetation + Staph bacteremia → CIED endocarditis (mandatory complete extraction) · new AV block on ECG → perivalvular abscess · new/worsening regurgitation + HF → surgical urgency
Initial Sx/Data (all comers): fever, malaise, night sweats, new murmur, embolic phenomena (stroke, splinter hemorrhages, Janeway lesions, Osler nodes, Roth spots), HF; IVDU/prosthetic valve/device/recent procedure risk · ≥3 blood culture sets from separate sites BEFORE antibiotics, echo (TTE→TEE), ECG (serial — watch for new AV block = abscess), CBC, BMP, inflammatory markers, UA; modified Duke criteria · (don't anchor — Staph aureus bacteremia always prompts the question "is this endocarditis?")
Neg (don't-miss): denies a perivalvular abscess (new AV block/conduction disease → urgent TEE + surgery) · denies heart failure from valve destruction (the #1 surgical indication) · denies septic emboli (stroke, splenic/renal infarct, mycotic aneurysm, septic pulmonary emboli in right-sided) · denies a prosthetic-valve or CIED infection treated as native (these need extraction/different thresholds) · denies starting antibiotics before drawing cultures in a stable patient
DDx: native valve endocarditis · prosthetic valve endocarditis · CIED (device/lead) endocarditis · (also: culture-negative endocarditis, marantic/non-bacterial thrombotic endocarditis, right-sided IE in IVDU, Libman-Sacks, other bacteremia without IE)
Plan — by Diagnosis
CONSULT (as relevant): Infectious Disease (regimen + duration) · Cardiology + Cardiac surgery (Endocarditis/Heart-Valve Team — surgical indications, timing) · EP/Cardiac surgery (CIED extraction) · Neurology/Neurosurgery (septic emboli, mycotic aneurysm)
Native valve endocarditis
– Confirm: modified Duke criteria — ≥3 blood culture sets (separate sites) positive for a typical organism + echo vegetation/new regurgitation; identify organism (Staph aureus, viridans strep, enterococcus)
– Rx: empiric IV vancomycin (15–20 mg/kg q8–12h, target trough/AUC) ± additional gram-negative coverage by risk while cultures pending → narrow to organism-directed therapy; total IV course usually 4–6 weeks (organism/valve-dependent) timed from first negative culture; serial cultures to document clearance
– Surgery for: heart failure from valve dysfunction (#1 indication), perivalvular abscess/new AV block, persistent bacteremia despite appropriate antibiotics, large (>10 mm) mobile vegetation ± embolization, recurrent emboli, fungal or highly resistant organisms — engage the valve team early
– Monitor / daily labs: serial blood cultures (clearance), serial ECG (new AV block = abscess), daily CBC/BMP, inflammatory markers, repeat echo for complications
– Dispo: OPAT (outpatient parenteral antibiotics) when stable + cleared; ID + cardiology follow-up; dental/source evaluation; PT/OT; med rec
– Two rules anchor native IE: cultures before antibiotics (≥3 sets), and surgery is driven by complications — heart failure first, then abscess (new AV block), persistent bacteremia, and big mobile vegetations. Watch the PR interval on serial ECGs; it's your abscess detector.
Prosthetic valve endocarditis
– Confirm: prosthetic valve + Duke criteria; TEE essential (TTE insensitive for prosthetic); early (<1 yr post-op) = nosocomial staph (incl coagulase-negative/MRSA), late = community organisms
– Rx: empiric IV vancomycin + gentamicin + rifampin for staph (rifampin penetrates prosthetic-material biofilm) → organism-directed; longer course, usually ≥6 weeks; serial cultures for clearance
– Surgery (lower threshold than native): for HF, dehiscence/new regurgitation, abscess, persistent bacteremia, prosthetic obstruction, relapsing infection, large vegetation/embolism — many prosthetic IE (esp early/staphylococcal/complicated) need surgery; valve team early
– Monitor / daily labs: serial cultures, serial ECG (abscess), daily CBC/BMP, LFTs (rifampin), repeat TEE
– Dispo: ID-guided course (OPAT when appropriate); cardiology/surgery follow-up; anticoagulation management for mechanical valve per team; med rec
– Prosthetic valve IE has a lower surgical threshold and needs rifampin for staph (to penetrate the biofilm on the prosthetic material) plus a longer course. Early prosthetic IE is nosocomial staph and frequently surgical — the TEE, not the TTE, makes the diagnosis.
CIED (device/lead) endocarditis
– Confirm: pacemaker/ICD + bacteremia (esp Staph) + lead/valve vegetation on TEE; pocket infection may or may not be present
– Rx: blood cultures → empiric IV vancomycin → organism-directed; complete device + lead extraction is mandatory (the system cannot be sterilized in place) via percutaneous or surgical lead extraction (EP/cardiac surgery); prolonged IV antibiotics (duration per bacteremia/vegetation/valve involvement, often ≥4–6 weeks); reimplant on the contralateral side after blood-culture clearance, only if still indicated
– Monitor / daily labs: serial blood cultures (document clearance before reimplant), daily CBC/BMP, TEE, inflammatory markers
– Dispo: ID-guided antibiotics; staged reimplantation; ID + EP/cardiology follow-up; med rec
– Like any infected hardware, CIED endocarditis demands complete extraction of the generator and all leads — antibiotics alone will not cure it. Staph bacteremia in any device patient must trigger a TEE to look for lead vegetations.
111. Infective Endocarditis
complete reference · native, prosthetic, and CIED endocarditis · cultures before antibiotics, echo (TEE for prosthetic and device), and the surgical indications · a dosed, bulleted plan per setting · Full Card
Approach — Secure the Microbiology, Image the Valve, Know Who Needs Surgery
The diagnosis rests on the modified Duke criteria, built from two pillars: persistently positive blood cultures with a typical organism, and echocardiographic evidence (a vegetation, an abscess, new regurgitation, or prosthetic dehiscence).
The two foundational moves are drawing at least three sets of blood cultures from separate sites before starting antibiotics (unless the patient is in septic shock) and obtaining an echo (TTE then TEE, with TEE essential for prosthetic valves and devices).
Then ask whether the valve is native, prosthetic, or a device, because each has a distinct antibiotic course and surgical threshold. The questions that change management are heart failure, perivalvular abscess (heralded by new AV block), persistent bacteremia, a large mobile vegetation, embolization, and a fungal or resistant organism — each pushing toward surgery.
Key discriminators: a native valve with a vegetation and a typical organism (Staph aureus, viridans strep, enterococcus) is native valve IE; a prosthetic valve (early under 1 year is nosocomial staph, late is community) is prosthetic valve IE with a higher surgical threshold; a pacemaker or ICD with a lead vegetation and Staph bacteremia is CIED endocarditis requiring complete extraction; and new AV block signals a perivalvular abscess.
Initial Symptoms / Data (all comers)
Fever, malaise, night sweats, a new murmur, embolic phenomena (stroke, splinter hemorrhages, Janeway lesions, Osler nodes, Roth spots), and heart failure, with attention to risk factors (IV drug use, a prosthetic valve, a device, a recent procedure).
At least three blood culture sets from separate sites before antibiotics, an echocardiogram (TTE then TEE), a serial ECG (watching for new AV block, which signals an abscess), CBC, BMP, inflammatory markers, and urinalysis, applied to the modified Duke criteria.
Neg (don't-miss)
Pt denies a perivalvular abscess (new AV block or conduction disease prompting an urgent TEE and surgery) and denies heart failure from valve destruction (the leading surgical indication).
Pt denies septic emboli (stroke, splenic or renal infarct, mycotic aneurysm, or septic pulmonary emboli in right-sided disease) and denies a prosthetic valve or CIED infection being treated as native disease (these need extraction or different thresholds).
Pt denies starting antibiotics before drawing cultures in a stable patient.
DDx
Native valve endocarditis · prosthetic valve endocarditis · CIED (device or lead) endocarditis · (also culture-negative endocarditis, marantic or non-bacterial thrombotic endocarditis, right-sided IE in IV drug use, Libman-Sacks endocarditis, and other bacteremia without endocarditis)
Plan — by Diagnosis
CONSULT (as relevant): Infectious disease (regimen and duration) · Cardiology and Cardiac surgery (an Endocarditis or Heart-Valve Team for surgical indications and timing) · EP and Cardiac surgery (CIED extraction) · Neurology and Neurosurgery (septic emboli, mycotic aneurysm)
Native valve endocarditis
Confirm: the modified Duke criteria — at least three blood culture sets from separate sites positive for a typical organism, plus an echo vegetation or new regurgitation — and identify the organism (Staph aureus, viridans strep, enterococcus).
Therapy: empiric IV vancomycin (15–20 mg/kg every 8–12 hours, dosed to a target trough or AUC) with additional gram-negative coverage by risk while cultures are pending, narrowed promptly to organism-directed therapy; a total IV course of usually 4–6 weeks (organism- and valve-dependent) timed from the first negative culture; and serial cultures to document clearance.
Surgery for: heart failure from valve dysfunction (the leading indication), a perivalvular abscess or new AV block, persistent bacteremia despite appropriate antibiotics, a large (over 10 mm) mobile vegetation with or without embolization, recurrent emboli, and fungal or highly resistant organisms — engaging the valve team early.
Monitoring / daily labs: serial blood cultures for clearance, serial ECGs (new AV block signals an abscess), a daily CBC and BMP, inflammatory markers, and a repeat echo for complications.
Mobility / consults / disposition: outpatient parenteral antibiotic therapy (OPAT) once stable and cleared; ID and cardiology follow-up; a dental and source evaluation; PT and OT; medication reconciliation.
Prosthetic valve endocarditis
Confirm: a prosthetic valve meeting the Duke criteria, with TEE essential (TTE is insensitive for prosthetic valves); early disease (under 1 year post-op) is nosocomial staphylococcal (including coagulase-negative staph and MRSA), and late disease involves community organisms.
Therapy: empiric IV vancomycin plus gentamicin plus rifampin for staphylococcal disease (rifampin penetrates the biofilm on prosthetic material), narrowed to organism-directed therapy; a longer course, usually at least 6 weeks; and serial cultures for clearance.
Surgery (a lower threshold than native disease): for heart failure, dehiscence or new regurgitation, an abscess, persistent bacteremia, prosthetic obstruction, relapsing infection, or a large vegetation or embolism — many prosthetic IE cases (especially early, staphylococcal, or complicated) require surgery, so engage the valve team early.
Monitoring / daily labs: serial cultures, serial ECGs (for abscess), a daily CBC and BMP, LFTs (on rifampin), and a repeat TEE.
Mobility / consults / disposition: an ID-guided course (OPAT when appropriate); cardiology and surgery follow-up; anticoagulation management for a mechanical valve per the team; medication reconciliation.
CIED (device or lead) endocarditis
Confirm: a pacemaker or ICD with bacteremia (especially Staphylococcus) and a lead or valve vegetation on TEE, with or without a pocket infection.
Therapy: blood cultures then empiric IV vancomycin narrowed to organism-directed therapy; mandatory complete device and lead extraction (the system cannot be sterilized in place) via percutaneous or surgical lead extraction (EP and cardiac surgery); a prolonged IV antibiotic course (duration per the bacteremia, vegetation, and valve involvement, often at least 4–6 weeks); and reimplantation on the contralateral side after blood-culture clearance, only if still indicated.
Monitoring / daily labs: serial blood cultures (documenting clearance before reimplantation), a daily CBC and BMP, a TEE, and inflammatory markers.
Mobility / consults / disposition: ID-guided antibiotics; staged reimplantation; ID and EP or cardiology follow-up; medication reconciliation.
Red Flags
New AV block or conduction disease → a perivalvular abscess; urgent TEE and surgery
Heart failure from valve destruction → the leading surgical indication; engage the valve team urgently
Persistent bacteremia despite appropriate antibiotics → an uncontrolled focus needing source control or surgery
A large mobile vegetation with embolic events → consider early surgery
Staphylococcal bacteremia in a prosthetic-valve or device patient → presumed prosthetic or CIED IE until a TEE clarifies
Senior IM Resident Pearls
Cultures before antibiotics. At least three sets from separate sites in the stable patient — the microbiology drives the entire regimen and duration.
The PR interval is your abscess detector. Serial ECGs catch the new AV block that signals a perivalvular abscess and a surgical valve.
Surgery is driven by complications. Heart failure first, then abscess, persistent bacteremia, large mobile vegetations, and fungal or resistant organisms.
TEE makes the prosthetic and device diagnosis. The TTE is insensitive — go to TEE for any prosthetic valve or CIED concern.
Prosthetic staph needs rifampin and often surgery. Rifampin penetrates the biofilm, and early prosthetic IE is nosocomial staph that is frequently surgical.
Infected hardware comes out. CIED endocarditis is cured by complete extraction plus antibiotics, never antibiotics alone.
Common mistake: starting antibiotics before cultures in a stable patient, or missing the new AV block that means an abscess and a trip to the OR.
Cardiology — Cardiomyopathy
112. Cardiomyopathy
dilated (full GDMT) · hypertrophic (don't offload an obstructed LV) · restrictive (find the infiltration, exclude constriction) · amyloidosis (the can't-miss infiltrative cause) · separate dosed plans · Super Compact
Approach — classify the muscle by structure and physiology, because the treatment for one type can be dangerous in another. Three structural buckets: dilated (a big weak ventricle), hypertrophic (a thick, often obstructive ventricle), and restrictive (a stiff, non-compliant ventricle) — each behaves differently. The pivotal safety point: the standard heart-failure offloading approach (diuresis, vasodilators, inotropes) helps dilated cardiomyopathy but can be harmful in obstructive HCM and in the preload-dependent restrictive/amyloid heart, where dropping preload or afterload worsens obstruction or output. The echo classifies; then hunt the etiology — and always ask whether infiltration (especially amyloid) is the cause, because it has specific therapy and specific drugs to avoid.
Key discriminators: dilated LV + globally reduced EF → dilated CM (full GDMT) · thick (asymmetric septal) LV ± dynamic LVOT obstruction/systolic murmur worse with Valsalva → hypertrophic CM · stiff non-dilated ventricles + biatrial enlargement + diastolic dysfunction → restrictive CM · thick walls + LOW-voltage ECG (the classic mismatch) + restrictive physiology → amyloidosis · restrictive picture but pericardium thick/calcified, respirophasic septal shift → constrictive pericarditis (surgically curable mimic)
Initial Sx/Data (all comers): dyspnea, HF symptoms, exertional symptoms, palpitations/syncope (esp HCM), edema/ascites (right-sided in restrictive); family history of cardiomyopathy/sudden death; murmur character · echo (chamber size, wall thickness, EF, diastology, obstruction, strain), ECG (voltage — low in amyloid; LVH, conduction disease), troponin/BNP, cardiac MRI (tissue characterization), labs by suspicion (SPEP/serum free light chains, technetium-PYP scan, iron studies, genetics) · (don't anchor — thick walls aren't always hypertensive LVH; low-voltage thick walls = amyloid until proven otherwise)
Neg (don't-miss): denies offloading an obstructed LV (diuretics/vasodilators/inotropes worsen LVOT obstruction in HCM) · denies missed amyloidosis (thick walls + low voltage; AL amyloid is a heme emergency) · denies constrictive pericarditis masquerading as restrictive CM (surgically curable) · denies SCD risk in HCM (family Hx, syncope, NSVT, massive hypertrophy → ICD) · denies giving digoxin/CCB/vasodilators in amyloid (poorly tolerated/contraindicated)
DDx: dilated cardiomyopathy · hypertrophic cardiomyopathy · restrictive cardiomyopathy · cardiac amyloidosis · (also: ischemic CM, constrictive pericarditis, hypertensive heart disease, sarcoidosis, hemochromatosis, Fabry, stress (Takotsubo), peripartum, arrhythmogenic RV CM)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology / advanced HF (classification, GDMT, transplant/MCS) · Electrophysiology (SCD risk, ICD, ablation) · Hematology (AL amyloid — urgent) · Cardiac amyloid / infiltrative program (ATTR); Genetics (familial)
Dilated cardiomyopathy
– Confirm: dilated LV with globally reduced EF; identify etiology (ischemic vs nonischemic — alcoholic, peripartum, tachycardia-mediated, myocarditis, familial, chemo, idiopathic)
– Rx: full four-pillar GDMT — ARNI sacubitril-valsartan 24/26 mg PO BID → target 97/103 mg BID (or ACEi lisinopril 2.5–5 mg PO daily); BB carvedilol 3.125 mg PO BID or metoprolol succinate 12.5–25 mg PO daily; MRA spironolactone 25 mg PO daily (K<5.0, eGFR>30); SGLT2i dapagliflozin 10 mg PO daily; diurese congestion (furosemide); treat the reversible cause (abstinence, rate control, immunosuppression for selected myocarditis)
– Device: ICD for primary prevention if EF ≤35% AFTER ~90 days optimized GDMT; CRT if EF ≤35% + LBBB with wide QRS
– Monitor / daily labs: daily BMP (K/Cr), weights, telemetry; serial echo for EF recovery
– Dispo: PT/OT, cardiac rehab; advanced-HF referral if progressive; HF + GDMT education; cardiology follow-up; med rec
– Dilated CM is the cardiomyopathy that takes the full HF playbook — all four pillars, dosed and uptitrated — and the ICD decision waits ~90 days because optimized GDMT can recover the EF above the threshold. Always hunt the reversible cause first.
Hypertrophic cardiomyopathy
– Confirm: LV hypertrophy (often asymmetric septal) not explained by loading conditions; assess dynamic LVOT obstruction (murmur louder with Valsalva/standing), SAM of mitral valve; family history; SCD risk factors
– Rx (do NOT offload an obstructed LV): negative inotropes/chronotropes to reduce obstruction — beta-blocker (metoprolol) or non-dihydropyridine CCB (verapamil/diltiazem); AVOID vasodilators (nitrates, ACEi/ARB as afterload reducers, dihydropyridines), aggressive diuresis, and inotropes (digoxin, dobutamine) — all increase obstruction; maintain preload/afterload; cardiac myosin inhibitor (mavacamten) for obstructive HCM; septal reduction (myectomy or alcohol septal ablation) for refractory obstruction; for acute hypotension in obstructive HCM use phenylephrine (pure vasoconstrictor) + fluids, NOT inotropes
– SCD prevention: ICD for high risk (prior arrest/sustained VT, family Hx SCD, unexplained syncope, massive LVH ≥30 mm, NSVT, apical aneurysm) — EP; family screening + genetic counseling
– Monitor / daily labs: telemetry (NSVT), daily BMP, serial echo (gradient)
– Dispo: activity counseling; EP/HCM-center referral; family screening; cardiology follow-up; med rec
– The cardinal HCM rule: do not offload an obstructed ventricle. Diuretics, vasodilators, and inotropes all worsen the dynamic obstruction — the opposite of dilated-CM management. For hypotension, give a pure vasoconstrictor (phenylephrine) and fluids, and risk-stratify everyone for sudden death.
Restrictive cardiomyopathy
– Confirm: stiff, non-dilated ventricles with marked diastolic dysfunction + biatrial enlargement; preserved/near-normal EF until late; distinguish from constrictive pericarditis (thick/calcified pericardium, respirophasic ventricular interdependence/septal shift, equalized diastolic pressures — surgically curable)
– Rx: find + treat the infiltration/cause (amyloid, sarcoid, hemochromatosis → phlebotomy/chelation, Fabry, endomyocardial fibrosis, radiation); gentle diuresis for congestion (furosemide low-dose — preload-dependent, avoid over-diuresis); rate + rhythm control, maintain atrial contribution; cardiac MRI/biopsy for tissue diagnosis
– Monitor / daily labs: daily BMP, weights, telemetry; cause-specific labs
– Dispo: treat underlying disease; pericardiectomy if constriction confirmed; specialty referral by etiology; cardiology follow-up; med rec
– Two tasks in restrictive CM: find what's infiltrating the muscle, and rule out constrictive pericarditis — because constriction looks identical but is surgically curable with pericardiectomy. Diurese gently; the stiff ventricle is preload-dependent.
Cardiac amyloidosis
– Confirm: thick LV walls with LOW-voltage ECG (the classic mismatch), restrictive physiology, granular myocardium/strain pattern; type it: ATTR (technetium-PYP scan positive, exclude monoclonal protein) vs AL (SPEP/serum immunofixation + serum free light chains, biopsy with typing)
– Rx: AL amyloid → URGENT hematology (plasma-cell-directed chemotherapy — a hematologic emergency); ATTR amyloid → tafamidis (TTR stabilizer; ATTR-specific therapies); supportive HF — cautious diuresis for congestion (furosemide, often with an MRA); AVOID digoxin (binds amyloid fibrils → toxicity), and use beta-blockers/CCB/ACEi cautiously or avoid (poorly tolerated — fixed stroke volume, sensitive to preload/rate); manage arrhythmia + conduction disease (pacing/anticoagulation as needed)
– Monitor / daily labs: daily BMP, weights; light chains/biomarkers per heme; telemetry (conduction disease)
– Dispo: amyloid program + hematology (AL); ATTR-directed therapy; genetic counseling (hereditary ATTR); cardiology follow-up; med rec
– Thick walls plus low voltage is amyloid until proven otherwise — and typing is urgent because AL is a hematologic emergency while ATTR gets tafamidis. The drug traps: digoxin is toxic (binds fibrils), and the usual HF agents are poorly tolerated by this preload-dependent, fixed-output heart.
112. Cardiomyopathy
complete reference · dilated, hypertrophic, restrictive, and amyloid cardiomyopathy · why offloading helps one and harms another · a dosed, bulleted plan per type · Full Card
Approach — Classify by Structure and Physiology, Because Treatments Don't Transfer
Classify the muscle by structure and physiology, because the treatment for one type can be dangerous in another. There are three structural buckets: dilated (a big, weak ventricle), hypertrophic (a thick, often obstructive ventricle), and restrictive (a stiff, non-compliant ventricle).
The pivotal safety point is that the standard heart-failure offloading approach (diuresis, vasodilators, inotropes) helps dilated cardiomyopathy but can be harmful in obstructive HCM and in the preload-dependent restrictive or amyloid heart, where dropping preload or afterload worsens the obstruction or the output.
The echo classifies the structure; then hunt the etiology, and always ask whether infiltration (especially amyloid) is the cause, because it has specific therapy and specific drugs to avoid.
Key discriminators: a dilated LV with a globally reduced EF is dilated CM; an asymmetrically thick LV with dynamic LVOT obstruction is hypertrophic CM; stiff non-dilated ventricles with biatrial enlargement are restrictive CM; thick walls with a low-voltage ECG (the classic mismatch) are amyloid; and a restrictive picture with a thick or calcified pericardium and respirophasic septal shift is constrictive pericarditis, the surgically curable mimic.
Initial Symptoms / Data (all comers)
Dyspnea, heart-failure symptoms, exertional symptoms, palpitations or syncope (especially in HCM), and edema or ascites (right-sided in restrictive disease), with a family history of cardiomyopathy or sudden death and the murmur character.
An echocardiogram (chamber size, wall thickness, EF, diastolic function, obstruction, strain), an ECG (voltage — low in amyloid; LVH and conduction disease), troponin and BNP, and cardiac MRI for tissue characterization, plus labs directed by suspicion (SPEP and serum free light chains, a technetium-PYP scan, iron studies, genetic testing).
Neg (don't-miss)
Pt denies the harm of offloading an obstructed LV (diuretics, vasodilators, and inotropes worsen LVOT obstruction in HCM) and denies missed amyloidosis (thick walls with low voltage; AL amyloid is a hematologic emergency).
Pt denies constrictive pericarditis masquerading as restrictive CM (a surgically curable mimic) and denies unaddressed sudden-death risk in HCM (a family history, syncope, NSVT, or massive hypertrophy prompting an ICD).
Pt denies receiving digoxin, calcium channel blockers, or vasodilators in amyloid, which are poorly tolerated or contraindicated.
DDx
Dilated cardiomyopathy · hypertrophic cardiomyopathy · restrictive cardiomyopathy · cardiac amyloidosis · (also ischemic cardiomyopathy, constrictive pericarditis, hypertensive heart disease, sarcoidosis, hemochromatosis, Fabry disease, stress (Takotsubo) cardiomyopathy, peripartum cardiomyopathy, and arrhythmogenic RV cardiomyopathy)
Plan — by Diagnosis
CONSULT (as relevant): Cardiology and advanced heart failure (classification, GDMT, transplant or mechanical support) · Electrophysiology (sudden-death risk, ICD, ablation) · Hematology (AL amyloid — urgent) · a cardiac amyloid or infiltrative program (ATTR) · Genetics (familial disease)
Dilated cardiomyopathy
Confirm: a dilated LV with a globally reduced EF, identifying the etiology (ischemic versus nonischemic — alcoholic, peripartum, tachycardia-mediated, myocarditis, familial, chemotherapy-related, or idiopathic).
Therapy: full four-pillar GDMT — an ARNI, sacubitril-valsartan 24/26 mg PO twice daily titrated toward 97/103 mg twice daily (or an ACEi such as lisinopril 2.5–5 mg PO daily); a beta-blocker, carvedilol 3.125 mg PO twice daily or metoprolol succinate 12.5–25 mg PO daily; an MRA, spironolactone 25 mg PO daily (if potassium is under 5.0 and eGFR over 30); and an SGLT2 inhibitor, dapagliflozin 10 mg PO daily — plus diuresis of congestion (furosemide) and treatment of the reversible cause (abstinence, rate control, immunosuppression for selected myocarditis).
Device: an ICD for primary prevention if the EF remains 35% or less after about 90 days of optimized GDMT, and CRT if the EF is 35% or less with an LBBB and a wide QRS.
Monitoring / daily labs: a daily BMP (potassium, creatinine), daily weights, and telemetry, with serial echo to track EF recovery.
Mobility / consults / disposition: PT and OT and cardiac rehab; advanced-HF referral if progressive; HF and GDMT education; cardiology follow-up; medication reconciliation.
Hypertrophic cardiomyopathy
Confirm: LV hypertrophy (often asymmetric and septal) not explained by loading conditions, with an assessment of dynamic LVOT obstruction (a murmur louder with Valsalva or standing), systolic anterior motion of the mitral valve, the family history, and sudden-death risk factors.
Therapy (do not offload an obstructed LV): negative inotropes and chronotropes to reduce obstruction — a beta-blocker (metoprolol) or a non-dihydropyridine calcium channel blocker (verapamil or diltiazem) — while avoiding vasodilators (nitrates, ACEi or ARB used for afterload reduction, dihydropyridines), aggressive diuresis, and inotropes (digoxin, dobutamine), all of which increase obstruction; maintain preload and afterload; consider a cardiac myosin inhibitor (mavacamten) for obstructive HCM; pursue septal reduction (surgical myectomy or alcohol septal ablation) for refractory obstruction; and for acute hypotension in obstructive HCM use phenylephrine (a pure vasoconstrictor) and fluids rather than inotropes.
Sudden-death prevention: an ICD for high-risk patients (prior arrest or sustained VT, a family history of sudden death, unexplained syncope, massive LVH of 30 mm or more, NSVT, or an apical aneurysm) via EP, with family screening and genetic counseling.
Monitoring / daily labs: telemetry (for NSVT), a daily BMP, and serial echo for the gradient.
Mobility / consults / disposition: activity counseling; EP or HCM-center referral; family screening; cardiology follow-up; medication reconciliation.
Restrictive cardiomyopathy
Confirm: stiff, non-dilated ventricles with marked diastolic dysfunction and biatrial enlargement and a preserved or near-normal EF until late, distinguished from constrictive pericarditis (a thick or calcified pericardium, respirophasic ventricular interdependence and septal shift, and equalized diastolic pressures — surgically curable).
Therapy: find and treat the infiltration or cause (amyloid, sarcoid, hemochromatosis treated with phlebotomy or chelation, Fabry disease, endomyocardial fibrosis, radiation); gentle diuresis for congestion (low-dose furosemide, since the stiff ventricle is preload-dependent and over-diuresis drops output); rate and rhythm control with maintenance of the atrial contribution; and cardiac MRI or biopsy for a tissue diagnosis.
Monitoring / daily labs: a daily BMP, daily weights, and telemetry, with cause-specific labs.
Mobility / consults / disposition: treat the underlying disease; pericardiectomy if constriction is confirmed; specialty referral by etiology; cardiology follow-up; medication reconciliation.
Cardiac amyloidosis
Confirm: thick LV walls with a low-voltage ECG (the classic mismatch), restrictive physiology, and a granular myocardium or a typical strain pattern; then type it — ATTR (a positive technetium-PYP scan with exclusion of a monoclonal protein) versus AL (SPEP and serum immunofixation with serum free light chains, and biopsy with typing).
Therapy: for AL amyloid, urgent hematology referral for plasma-cell-directed chemotherapy (a hematologic emergency); for ATTR amyloid, tafamidis (a TTR stabilizer) and other ATTR-specific therapies; supportive HF care with cautious diuresis for congestion (furosemide, often with an MRA); avoidance of digoxin (which binds amyloid fibrils and causes toxicity) and cautious use or avoidance of beta-blockers, calcium channel blockers, and ACE inhibitors (poorly tolerated by this fixed-stroke-volume, preload- and rate-sensitive heart); and management of arrhythmia and conduction disease (pacing and anticoagulation as needed).
Monitoring / daily labs: a daily BMP and daily weights; light chains and biomarkers per hematology; and telemetry for conduction disease.
Mobility / consults / disposition: an amyloid program and hematology (for AL); ATTR-directed therapy; genetic counseling for hereditary ATTR; cardiology follow-up; medication reconciliation.
Red Flags
Thick walls with a low-voltage ECG → cardiac amyloidosis until proven otherwise; type it urgently
Offloading an obstructed HCM ventricle (diuretics, vasodilators, inotropes) → worsened obstruction and collapse
Syncope, NSVT, massive hypertrophy, or a family history of sudden death in HCM → an ICD evaluation
A restrictive picture with a thick or calcified pericardium → constrictive pericarditis, which is surgically curable
Digoxin in amyloid → toxicity from fibril binding; avoid it
Senior IM Resident Pearls
Treatments don't transfer between cardiomyopathies. The offloading that rescues a dilated ventricle can collapse an obstructed HCM or a preload-dependent amyloid heart.
Thick walls plus low voltage is amyloid. Type it urgently — AL is a hematologic emergency, ATTR gets tafamidis.
Don't offload an obstructed LV in HCM. Use negative inotropes, maintain preload and afterload, and for hypotension give phenylephrine and fluids, not an inotrope.
Always exclude constriction in a restrictive picture. Constrictive pericarditis is the surgically curable mimic.
The dilated-CM ICD decision waits 90 days. Optimized GDMT can recover the EF above the threshold and remove the indication.
Avoid digoxin in amyloid. It binds the fibrils and is toxic; the usual HF drugs are also poorly tolerated.
Common mistake: diuresing or vasodilating an obstructive HCM into collapse, or missing the amyloid behind "hypertensive" thick walls with a paradoxically low voltage.
Cardiology — Adult Congenital Heart Disease
113. Adult Congenital Heart Disease (ACHD)
repaired congenital disease with late complications · ACHD heart failure (systemic RV, Fontan) · ACHD arrhythmias · get the ACHD specialist early · separate dosed plans · Super Compact
Approach — these are grown-up survivors of childhood heart surgery whose anatomy and physiology are not normal, so the cardinal rule is: understand the specific repair and involve an ACHD specialist early. Most adults with congenital heart disease have been repaired or palliated, not cured — they present years later with predictable late complications (residual lesions, arrhythmias, heart failure of an unusual ventricle). Two physiologic concepts dominate the admissions: a systemic right ventricle (an RV doing the work of an LV, which fails over time) and Fontan circulation (a single functional ventricle, with blood flowing passively to the lungs — exquisitely preload-dependent). Standard adult-cardiology reflexes can be wrong here; the safest move is early specialist involvement and care at an ACHD center.
Key discriminators: prior repair (tetralogy of Fallot, coarctation, atrial/arterial switch, VSD/ASD closure) + new late problem (pulmonary regurgitation, recoarctation, baffle leak/obstruction, residual shunt) → repaired congenital disease — late complication · HF symptoms in a systemic RV (transposition s/p atrial switch, congenitally corrected TGA) or a Fontan (single ventricle) → ACHD heart failure · atrial arrhythmias (intra-atrial reentry/flutter, atrial tachycardia) often poorly tolerated → ACHD arrhythmia
Initial Sx/Data (all comers): dyspnea, reduced exercise tolerance, edema/ascites/hepatic congestion (Fontan), palpitations, syncope, cyanosis; KNOW the specific anatomy + surgical history · echo (often limited — needs ACHD expertise), ECG, BNP, cardiac MRI (the workhorse for ACHD anatomy/function), pulse oximetry, labs; review prior operative + cath reports · (don't anchor — never assume normal anatomy or apply standard physiology; get the old records and the ACHD team)
Neg (don't-miss): denies a poorly tolerated arrhythmia in single-ventricle/Fontan physiology (loss of atrial kick can cause rapid decompensation → early rhythm restoration) · denies a hemodynamically significant residual lesion (baffle obstruction, recoarctation, severe PR) · denies over-diuresing a preload-dependent Fontan (drops the passive pulmonary flow and output) · denies managing a systemic RV/Fontan with standard LV-HF assumptions · denies missing the need for endocarditis prophylaxis in high-risk lesions
DDx: repaired congenital disease with late complications · ACHD heart failure (systemic RV, Fontan/single-ventricle) · ACHD arrhythmias · (also: Eisenmenger/pulmonary vascular disease, cyanotic complications — erythrocytosis, residual/recurrent shunts, prosthetic/conduit dysfunction, ACHD endocarditis)
Plan — by Diagnosis
CONSULT (as relevant): ACHD specialist / ACHD center (central to all ACHD admissions — anatomy-specific management) · ACHD electrophysiology (arrhythmia, ablation, devices) · Cardiac surgery (reintervention) · Advanced HF / transplant (failing systemic RV or Fontan); Heart failure cardiology
Repaired congenital disease — late complication
– Confirm: known repair (tetralogy of Fallot, coarctation, atrial switch/Mustard-Senning, arterial switch, VSD/ASD closure) + a new late complication — pulmonary regurgitation (post-TOF), recoarctation, baffle leak/obstruction (atrial switch), residual/recurrent shunt, conduit/valve dysfunction, aortopathy; define with imaging
– Rx: obtain prior operative/cath records; cardiac MRI/echo + ACHD-guided assessment to quantify the lesion; reintervention timed by the ACHD/surgical team — e.g. pulmonary valve replacement for severe PR with RV dilation/dysfunction, stenting/repair of recoarctation, baffle intervention; treat the consequences (HF, arrhythmia) meanwhile; endocarditis prophylaxis for high-risk lesions
– Monitor / daily labs: telemetry, daily BMP, BNP; imaging-guided
– Dispo: ACHD-center referral; surgical/transcatheter planning; lifelong ACHD follow-up; PT/OT; med rec
– A repaired congenital heart is not a cured one — each repair has its signature late complication (tetralogy → pulmonary regurgitation, atrial switch → baffle problems, coarctation → recoarctation). Get the old operative reports and let the ACHD team time the reintervention; don't manage it from first principles.
ACHD heart failure (systemic RV / Fontan)
– Confirm: HF symptoms in unusual physiology — a systemic RV (D-TGA s/p atrial switch, or congenitally corrected TGA) failing under systemic pressure, or a Fontan/single-ventricle circulation (passive, preload-dependent pulmonary flow); identify a correctable driver (arrhythmia, residual lesion, valve dysfunction, baffle/conduit obstruction, Fontan-associated complications — PLE, hepatic congestion)
– Rx: ACHD-directed, individualized therapy (standard GDMT evidence is limited/extrapolated in this population — apply cautiously with ACHD input); treat correctable drivers first; for Fontan — optimize preload and maintain low pulmonary vascular resistance (avoid over-diuresis and anything raising PVR), treat hepatic congestion/PLE, consider PH-directed therapy per ACHD; cautious diuresis (furosemide) for congestion; rhythm control; anticoagulation per lesion/thrombosis risk
– Monitor / daily labs: daily BMP/weights, BNP, LFTs (Fontan hepatic congestion), telemetry; SpO2
– Dispo: advanced HF/transplant evaluation if progressive; ACHD center; PT/OT; HF education; ACHD follow-up; med rec
– The systemic RV and the Fontan don't follow the standard LV-failure script — the Fontan especially is passively, preload-driven, so over-diuresing it or raising pulmonary resistance crashes the output. Find and fix the correctable driver, support gently, and let the ACHD team lead.
ACHD arrhythmias
– Confirm: arrhythmia in ACHD — predominantly ATRIAL (intra-atrial reentrant tachycardia/atrial flutter, atrial tachycardia from scar/suture lines), less commonly ventricular; often poorly tolerated because the abnormal circulation depends on the atrial contribution and a regular rate
– Rx: early rhythm restoration (low threshold for cardioversion — these patients decompensate fast and rate control alone is often insufficient) with anticoagulation/thromboembolism precautions (TEE if duration/anticoagulation uncertain; high thrombotic risk in Fontan); ACHD-EP for catheter ablation (definitive for many atrial reentrant circuits) and device decisions; antiarrhythmics per ACHD-EP; correct hemodynamic/residual-lesion triggers
– Monitor / daily labs: continuous telemetry, daily BMP/Mg, anticoagulation parameters
– Dispo: ACHD-EP follow-up (ablation/device); anticoagulation plan; ACHD center; med rec
– In ACHD, atrial arrhythmias are both common and dangerous — the abnormal circulation leans on atrial kick and a steady rate, so patients decompensate quickly and you restore rhythm earlier than you would in a structurally normal heart. Thromboembolic risk is high (especially Fontan), and ACHD-EP ablation is often the definitive fix.
113. Adult Congenital Heart Disease (ACHD)
complete reference · repaired congenital disease with late complications, ACHD heart failure (systemic RV and Fontan), and ACHD arrhythmias · know the repair and get the specialist · a dosed, bulleted plan per problem · Full Card
Approach — Understand the Specific Repair and Involve an ACHD Specialist Early
These are grown-up survivors of childhood heart surgery whose anatomy and physiology are not normal, so the cardinal rule is to understand the specific repair and involve an ACHD specialist early.
Most adults with congenital heart disease have been repaired or palliated rather than cured, and they present years later with predictable late complications — residual lesions, arrhythmias, and heart failure of an unusual ventricle.
Two physiologic concepts dominate the admissions: a systemic right ventricle (an RV doing the work of an LV, which fails over time) and a Fontan circulation (a single functional ventricle with blood flowing passively to the lungs, making it exquisitely preload-dependent). Standard adult-cardiology reflexes can be wrong here, so the safest move is early specialist involvement and care at an ACHD center.
Key discriminators: a prior repair (tetralogy of Fallot, coarctation, atrial or arterial switch, VSD or ASD closure) with a new late problem (pulmonary regurgitation, recoarctation, a baffle leak or obstruction, a residual shunt) is a repaired-disease late complication; heart-failure symptoms in a systemic RV or a Fontan is ACHD heart failure; and atrial arrhythmias that are poorly tolerated are ACHD arrhythmias.
Initial Symptoms / Data (all comers)
Dyspnea, reduced exercise tolerance, edema, ascites, or hepatic congestion (in a Fontan), palpitations, syncope, and cyanosis, with knowledge of the specific anatomy and surgical history.
An echocardiogram (often limited and needing ACHD expertise), an ECG, BNP, cardiac MRI (the workhorse for ACHD anatomy and function), pulse oximetry, and labs, with a review of prior operative and catheterization reports.
Neg (don't-miss)
Pt denies a poorly tolerated arrhythmia in single-ventricle or Fontan physiology (loss of the atrial contribution can cause rapid decompensation, prompting early rhythm restoration) and denies a hemodynamically significant residual lesion (baffle obstruction, recoarctation, severe pulmonary regurgitation).
Pt denies over-diuresing a preload-dependent Fontan (which drops the passive pulmonary flow and the output) and denies managing a systemic RV or Fontan with standard LV-failure assumptions.
Pt denies missing the need for endocarditis prophylaxis in high-risk lesions.
DDx
Repaired congenital disease with late complications · ACHD heart failure (systemic RV, Fontan or single-ventricle) · ACHD arrhythmias · (also Eisenmenger syndrome and pulmonary vascular disease, cyanotic complications such as erythrocytosis, residual or recurrent shunts, prosthetic or conduit dysfunction, and ACHD endocarditis)
Plan — by Diagnosis
CONSULT (as relevant): an ACHD specialist or ACHD center (central to every ACHD admission for anatomy-specific management) · ACHD electrophysiology (arrhythmia, ablation, devices) · Cardiac surgery (reintervention) · Advanced heart failure and transplant (a failing systemic RV or Fontan)
Repaired congenital disease — late complication
Confirm: a known repair (tetralogy of Fallot, coarctation, an atrial switch such as Mustard or Senning, an arterial switch, or VSD or ASD closure) with a new late complication — pulmonary regurgitation (after tetralogy repair), recoarctation, a baffle leak or obstruction (after an atrial switch), a residual or recurrent shunt, conduit or valve dysfunction, or aortopathy — defined with imaging.
Therapy: obtain the prior operative and catheterization records; quantify the lesion with cardiac MRI or echo and an ACHD-guided assessment; pursue reintervention timed by the ACHD and surgical team (for example, pulmonary valve replacement for severe pulmonary regurgitation with RV dilation or dysfunction, stenting or repair of a recoarctation, or a baffle intervention); treat the consequences (heart failure, arrhythmia) in the meantime; and provide endocarditis prophylaxis for high-risk lesions.
Monitoring / daily labs: telemetry, a daily BMP, and BNP, with imaging-guided management.
Mobility / consults / disposition: ACHD-center referral; surgical or transcatheter planning; lifelong ACHD follow-up; PT and OT; medication reconciliation.
ACHD heart failure (systemic RV / Fontan)
Confirm: heart-failure symptoms in unusual physiology — a systemic RV (D-transposition after an atrial switch, or congenitally corrected transposition) failing under systemic pressure, or a Fontan or single-ventricle circulation with passive, preload-dependent pulmonary flow — and identify a correctable driver (arrhythmia, a residual lesion, valve dysfunction, baffle or conduit obstruction, or Fontan-associated complications such as protein-losing enteropathy and hepatic congestion).
Therapy: ACHD-directed, individualized therapy (the standard GDMT evidence is limited and extrapolated in this population, so apply it cautiously with ACHD input); treat correctable drivers first; for a Fontan, optimize preload and maintain a low pulmonary vascular resistance (avoid over-diuresis and anything that raises PVR), treat hepatic congestion and protein-losing enteropathy, and consider PH-directed therapy per ACHD; use cautious diuresis (furosemide) for congestion; pursue rhythm control; and anticoagulate per the lesion and thrombosis risk.
Monitoring / daily labs: a daily BMP and daily weights, BNP, LFTs (for Fontan hepatic congestion), telemetry, and SpO2.
Mobility / consults / disposition: advanced HF and transplant evaluation if progressive; an ACHD center; PT and OT; HF education; ACHD follow-up; medication reconciliation.
ACHD arrhythmias
Confirm: an arrhythmia in ACHD — predominantly atrial (intra-atrial reentrant tachycardia, atrial flutter, or atrial tachycardia arising from scar and suture lines), less commonly ventricular — often poorly tolerated because the abnormal circulation depends on the atrial contribution and a regular rate.
Therapy: early rhythm restoration with a low threshold for cardioversion (these patients decompensate quickly and rate control alone is often insufficient), with anticoagulation and thromboembolism precautions (a TEE if the duration or anticoagulation status is uncertain, given the high thrombotic risk in Fontan physiology); ACHD electrophysiology for catheter ablation (definitive for many atrial reentrant circuits) and device decisions; antiarrhythmics per ACHD-EP; and correction of hemodynamic and residual-lesion triggers.
Monitoring / daily labs: continuous telemetry, a daily BMP with magnesium, and anticoagulation parameters.
Mobility / consults / disposition: ACHD-EP follow-up (ablation or device); an anticoagulation plan; an ACHD center; medication reconciliation.
Red Flags
An arrhythmia in Fontan or single-ventricle physiology → rapid decompensation; restore rhythm early and anticoagulate
Over-diuresing a preload-dependent Fontan → a fall in passive pulmonary flow and cardiac output
A new hemodynamically significant residual lesion (baffle obstruction, recoarctation, severe pulmonary regurgitation) → ACHD reintervention
A failing systemic RV or Fontan → advanced-HF and transplant evaluation
Applying standard LV-failure physiology to an unusual ventricle → potential harm; involve ACHD early
Senior IM Resident Pearls
Repaired is not cured. Each repair has a signature late complication — tetralogy to pulmonary regurgitation, atrial switch to baffle problems, coarctation to recoarctation — so get the old operative reports.
The Fontan is preload-dependent and passively driven. Over-diuresing it or raising pulmonary vascular resistance crashes the output.
The systemic RV fails on its own schedule. An RV doing the LV's job decompensates over time and doesn't follow the standard script.
Restore rhythm early in ACHD. The abnormal circulation depends on atrial kick and a steady rate, so these patients decompensate fast and the cardioversion threshold is low.
Thromboembolic risk is high, especially in Fontan. Anticoagulate around arrhythmias and per the lesion.
The ACHD specialist leads. Standard adult-cardiology reflexes can be wrong, and care belongs at an ACHD center.
Common mistake: managing a systemic RV or Fontan with standard LV-failure assumptions, or over-diuresing a Fontan and dropping the cardiac output.