Pulmonary Hypertension Decompensation

Acute right ventricular failure from elevated pulmonary vascular resistance — cor pulmonale, syncope, volume overload; high mortality without prompt targeted therapy

Symptoms / Associated Sx

• Progressive dyspnea on exertion (hallmark)

• Syncope or presyncope (exertional — reduced cardiac output from RV failure)

• Chest pain (RV angina from ischemia)

• Fatigue, weakness

• Peripheral edema, ascites, JVD (right heart failure)

• Loud P2 (pulmonic component of S2), RV heave, TR murmur on exam

• Hemoptysis (pulmonary arterial hypertension — rare but serious)

Denies

• Orthopnea, PND (left heart failure — right heart failure causes edema without orthopnea typically)

• Pleuritic chest pain + hemoptysis + leg swelling without prior PH (rules out PE as sole trigger if truly absent — PE causes PH)

• Productive cough + fever (rules out infectious trigger if absent)

Social History (SHx)

Known PAH diagnosis and WHO group, current pulmonary vasodilator therapy and compliance (sildenafil, riociguat, bosentan, ambrisentan, macitentan, epoprostenol, treprostinil), prior hospitalizations for decompensation, RHC (right heart catheterization) baseline values, underlying cause (connective tissue disease, CTEPH, idiopathic), prior vasodilator escalation, oxygen use.

Main Etiology

• WHO Group 1 (PAH): Idiopathic, heritable, drug/toxin-induced (methamphetamine, anorexigens), CTD-associated (scleroderma — highest risk), HIV, portal hypertension

• WHO Group 2: Left heart disease (LV dysfunction, valvular — most common cause of PH overall)

• WHO Group 3: Lung disease (COPD, ILD, hypoxia) → chronic hypoxic vasoconstriction

• WHO Group 4: CTEPH (chronic thromboembolic PH) — incompletely resolved PE → organized thrombus → fixed obstruction; surgically curable (PEA)

• WHO Group 5: Multifactorial (sarcoidosis, hemolytic anemias, thyroid)

• Decompensation triggers: infection, arrhythmia, PE (always exclude), medication noncompliance, aggressive diuresis, pregnancy, surgery

Most Common DDx

• Pulmonary embolism (acute-on-chronic PH from new PE; CTPA rules in/out; PE is also a cause of PH → CTEPH; anticoagulate)

• Left heart failure (Group 2 PH — elevated PCWP >15 on RHC; BNP elevated; LV dysfunction on echo; treat CHF, not vasodilators)

• COPD with cor pulmonale (Group 3 — O2 + COPD treatment; pulmonary vasodilators not effective and may worsen V/Q mismatch)

• Pericardial tamponade (obstructive shock; JVD + muffled sounds + hypotension; echo confirms; pericardiocentesis)

• RV infarction (inferior MI with RV involvement; EKG — right-sided leads; ST elevation V4R; hypotension + JVD + clear lungs; IV fluids, not diuretics)

• Severe hypoxia from lung disease causing reactive PH (treat hypoxia; PH secondary and will improve with O2/treatment of underlying lung disease)

DATA

• Echo (RV dilation + RV hypokinesis; D-shaped LV in severe RV pressure overload; estimated RVSP from TR jet; pericardial effusion)

• Right heart catheterization (gold standard for PH diagnosis and WHO group classification — mPAP ≥20 mmHg at rest; PCWP ≤15 = pre-capillary PAH; PCWP >15 = post-capillary/Group 2)

• BNP/NT-proBNP (RV wall stress marker; elevated = decompensation; trend for treatment response)

• Troponin (RV ischemia; elevated in severe decompensation — poor prognosis)

• CBC, BMP (polycythemia; renal function — right heart failure → cardiorenal syndrome)

• CT pulmonary angiography (CTPA — rule out acute PE; pulmonary artery dilation; mosaic perfusion pattern)

• CT with chronic thromboembolic disease protocol (CTEPH evaluation)

• Liver function (hepatic congestion from right heart failure)

• 6-minute walk test (deferred during acute; functional capacity baseline)

• ANA, anti-Scl70, anti-centromere (CTD-associated PAH screening — scleroderma)

• HIV, LFTs, hepatitis panel (secondary causes)

Home Meds

• Pulmonary vasodilators (assess compliance — noncompliance is a common decompensation trigger):

  • PDE5 inhibitors: sildenafil 20 mg TID, tadalafil 40 mg daily

  • Endothelin receptor antagonists: bosentan 125 mg BID, ambrisentan 10 mg daily, macitentan 10 mg daily

  • Soluble guanylate cyclase stimulator: riociguat 2.5 mg TID

  • Prostacyclin analogues: epoprostenol IV (continuous pump — NEVER abruptly stop; life-threatening rebound); treprostinil SQ/IV/inhaled; iloprost inhaled

• Warfarin (Group 1 — some centers anticoagulate; INR 1.5–2.5)

• Diuretics (furosemide, spironolactone — right heart failure management)

Plan

• Identify and treat trigger (infection → antibiotics; PE → anticoagulate; arrhythmia → rate/rhythm control; noncompliance → resume medication)

• O2 supplementation: Target SpO2 ≥92% (hypoxia causes pulmonary vasoconstriction worsening PH); supplemental O2 is a pulmonary vasodilator

• Diuresis (volume management):

  • Furosemide 40–80 mg IV (right heart failure with volume overload)

  • Target weight loss 0.5–1 kg/day; avoid aggressive diuresis (RV preload-dependent; hypovolemia worsens RV failure)

  • Monitor creatinine daily (cardiorenal syndrome)

• Pulmonary vasodilator therapy (Group 1 PAH):

  • Continue and optimize current regimen; escalate if decompensated despite therapy

  • IV epoprostenol (never stop abruptly — rebound crisis and death); if pump fails → call pharmacy immediately; bridge with IV treprostinil

  • Inhaled NO (nitric oxide) 5–20 ppm via ventilator or high-flow delivery (selective pulmonary vasodilator; used in acute decompensation; requires ICU)

  • IV prostacyclin (epoprostenol or treprostinil) — initiate in ICU for acute decompensation of Group 1 PAH not on IV therapy

• RV failure management:

  • Avoid aggressive fluid loading (worsens RV dilation → septal shift → LV compression)

  • Norepinephrine (vasopressor of choice for RV failure + systemic hypotension — maintains coronary perfusion pressure to ischemic RV)

  • Avoid dopamine at high doses (increases RVSP and worsens RV afterload)

  • Vasopressin 0.03–0.04 units/min (alternative; reduces PVR/SVR ratio favorably)

• CTEPH: Anticoagulation (warfarin or DOAC); refer to CTEPH program for pulmonary endarterectomy (PEA — curative surgery); balloon pulmonary angioplasty (BPA) for inoperable disease; riociguat (Group 4 PAH-specific agent)

• AVOID: sildenafil/tadalafil in Group 2 PH (left heart disease → worsens pulmonary edema); high-flow O2 in Group 3 PH worsened by hyperoxia

• ICU for severe decompensation (troponin rise, hemodynamic instability, syncope)

• Pulmonary hypertension specialist center transfer if unstable or on IV prostacyclin

• Echo q24–48h; daily BNP; daily BMP; continuous SpO2 and hemodynamic monitoring

• PT/OT — graded exercise; respiratory therapy

• Discharge: Optimize pulmonary vasodilator regimen (PH specialist follow-up); diuretic titration; daily weight monitoring; O2 at rest/exertion/sleep; avoid decompensation triggers (infections → vaccinate; pregnancy → contraindication in PAH); PH center follow-up within 1–2 weeks; advance directive discussion (PAH median survival 5–7 years from diagnosis)

Red Flags

• Epoprostenol pump failure → life-threatening rebound pulmonary hypertension → emergency pharmacy + ICU; never allow pump to run dry

• Syncope + known PAH → severely reduced cardiac output from RV failure → ICU; IV epoprostenol ± inhaled NO; ECMO as bridge to transplant

• Troponin rise in PAH + hemodynamic instability → RV infarction pattern → ICU; norepinephrine; inhaled NO; expedited PH center transfer

• Acute PE causing acute-on-chronic PH decompensation → anticoagulation urgently; submassive/massive PE protocol (see PE section)

• Pregnancy in PAH → maternal mortality 30–56%; multidisciplinary immediate management; delivery planning with PH center

Senior IM Resident Pearls

• WHO PH classification drives treatment: Group 1 (PAH) → vasodilators; Group 2 (left heart) → treat CHF, NOT vasodilators (worsen); Group 3 (lung disease) → O2 + treat lung disease; Group 4 (CTEPH) → anticoagulate + PEA surgery or BPA; Group 5 → treat underlying disease

• Epoprostenol has a half-life of 3–5 minutes — abrupt discontinuation causes rebound crisis (massive vasoconstriction) and sudden death; always have emergency backup supply; if pump fails, call pharmacy and ICU simultaneously

• Norepinephrine is preferred over dopamine in RV failure — maintains aortic diastolic pressure (RV coronary perfusion pressure); dopamine increases PVR and worsens RV afterload at higher doses

• CTEPH is surgically curable — PEA (pulmonary endarterectomy) is potentially curative in operable CTEPH; refer all CTEPH patients to experienced PEA center; do not miss this diagnosis (chronic non-resolving PE in anticoagulated patient)

• Common mistake: Giving sildenafil or endothelin antagonists to Group 2 PH (left heart disease) — pulmonary vasodilators in patients with elevated PCWP cause pulmonary edema and can be fatal; always define WHO group before prescribing vasodilators