Mean pulmonary artery pressure >20 mmHg at rest; 5 WHO groups by etiology.
Also known as: pulmonary hypertension, PH, PAH, pulmonary arterial hypertension, cor pulmonale
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Risk factors Group 1 (PAH): idiopathic, heritable (BMPR2 mutation), connective tissue disease (scleroderma, SLE), HIV, portal hypertension, congenital heart disease, drugs (methamphetamine, anorexigens, dasatinib) Group 2: left-sided heart disease (HFpEF, HFrEF, valvular) Group 3: chronic lung disease (COPD, IPF, OSA, hypoventilation), high altitude Group 4: chronic thromboembolic pulmonary hypertension (CTEPH) — prior PE Group 5: multifactorial (sarcoid, sickle cell, hemolytic anemia, ESRD, glycogen storage) Pathophysiology Sustained elevation of pulmonary vascular resistance causes RV pressure overload, RV hypertrophy and dilation, tricuspid regurgitation, and eventually RV failure. In PAH, pathologic vascular remodeling (intimal hyperplasia, medial hypertrophy, plexiform lesions) and dysregulated nitric oxide/endothelin/prostacyclin pathways drive disease.
Clinical presentation Symptoms Progressive exertional dyspnea (most common, often misattributed to deconditioning early) Fatigue, exercise intolerance Exertional chest pain, presyncope/syncope (severe disease, RV ischemia) Palpitations, hemoptysis (rare) Symptoms of right heart failure: lower-extremity edema, ascites, abdominal fullness Signs / physical exam Loud P2 (palpable in severe), wide split S2, RV heave/lift Right-sided S3 or S4 Tricuspid regurgitation murmur (holosystolic at LLSB, increases with inspiration — Carvallo sign) Pulmonic regurgitation murmur (Graham Steell, diastolic decrescendo) Elevated JVP, hepatomegaly, ascites, peripheral edema, pulsatile liver Classic findings Loud P2 + RV heave + signs of right HF + clear lungs (or findings of underlying lung disease) in a dyspneic patient.
Differential diagnosis Left heart failure (HFpEF or HFrEF) — Elevated PCWP (>15 mmHg) on RHC — defines post-capillary PH (Group 2); orthopnea, edema, S3Chronic obstructive pulmonary disease — Obstructive spirometry, hyperinflation; mild PH common but rarely severeInterstitial lung disease / IPF — Restrictive PFTs, reduced DLCO, fibrosis on HRCTObstructive sleep apnea — Witnessed apneas, daytime sleepiness, BMI elevated; polysomnographyCTEPH — History of PE, mismatched V/Q defects (sensitive screen); pulmonary endarterectomy may cureHigh-output state (anemia, thyrotoxicosis, AV fistula) — Warm extremities, wide pulse pressure; reversible causePulmonary veno-occlusive disease — PAH features with pulmonary edema after vasodilator challenge; centrilobular ground-glass on HRCTDiagnostic workup Diagnostic criteria mPAP >20 mmHg on right heart cath. PAH (Group 1) additionally requires PCWP ≤15 mmHg and PVR >2 Wood units. Vasoreactivity: ≥10 mmHg decrease in mPAP to ≤40 mmHg with maintained cardiac output.
Labs BNP/NT-proBNP — elevated, prognostic HIV serology, hepatitis serologies (portopulmonary) Connective tissue disease workup: ANA, anti-centromere, anti-Scl-70, RF, anti-CCP if indicated Thrombophilia testing if CTEPH suspected TSH Imaging Echocardiography (TTE) — screening modality: estimated RV systolic pressure from TR jet, RV size/function, signs of pressure overload CXR — enlarged central pulmonary arteries with peripheral pruning, cardiomegaly ECG — RV hypertrophy (R>S in V1), right axis deviation, P pulmonale (tall P in II), RBBB V/Q scan — mandatory to exclude CTEPH (sensitivity > CTPA) HRCT chest — evaluate for ILD; pulmonary CTA if PE/CTEPH suspected PFTs including DLCO (low DLCO with otherwise normal PFTs suggests vasculopathy) Polysomnography if OSA suspected Other studies Right heart catheterization (REQUIRED for confirmation of PAH and treatment decisions): mPAP >20 mmHg + PCWP ≤15 mmHg + PVR >2 Wood units defines pre-capillary PH; vasoreactivity testing with inhaled nitric oxide 6-minute walk distance — functional assessment, prognostic Diagnostic algorithm WHO Group Mechanism Examples 1: PAH Pulmonary arteriolar disease Idiopathic, heritable, CTD (scleroderma), HIV, portopulmonary, drug-induced 2: Left heart disease Post-capillary, elevated PCWP HFpEF, HFrEF, valvular 3: Lung disease/hypoxia Chronic hypoxic vasoconstriction COPD, IPF, OSA, high altitude 4: CTEPH Chronic thromboembolic obstruction Unresolved PE 5: Multifactorial Mixed mechanisms Sarcoid, sickle cell, ESRD, hematologic disorders
WHO classification of pulmonary hypertension (5 groups) — guides workup and treatment. Treatment First-line Treat the underlying cause first: heart failure (Group 2), chronic lung disease (Group 3), CTEPH (Group 4), connective tissue or other (Group 5) PAH-specific therapies are reserved for Group 1 and CTEPH inoperable patients — they can WORSEN Groups 2 and 3 Group 1 PAH general measures: supplemental O2 if hypoxemic, diuretics for RV volume overload, anticoagulation (idiopathic PAH only; controversial), avoid pregnancy Vasoreactive responders (rare, ~10%): high-dose calcium channel blockers — nifedipine, diltiazem, amlodipine Non-vasoreactive (most): combination therapy from 3 pathways: Endothelin receptor antagonist (ERA): bosentan, ambrisentan, macitentan Phosphodiesterase-5 inhibitor (PDE5i): sildenafil, tadalafil — OR soluble guanylate cyclase stimulator: riociguat (also approved for CTEPH) Prostacyclin pathway: epoprostenol (continuous IV), treprostinil (IV/SC/inhaled/oral), iloprost (inhaled), selexipag (oral) Second-line / adjunct Severe/high-risk PAH (AMBITION trial): initial combination ambrisentan + tadalafil Activin signaling inhibitor sotatercept (STELLAR 2023) — added benefit on top of background therapy CTEPH (Group 4): pulmonary endarterectomy (potentially curative) for surgical candidates; riociguat for inoperable; balloon pulmonary angioplasty in select centers Refractory PAH: lung or heart-lung transplantation, atrial septostomy as bridge Group 3 PH from lung disease: inhaled treprostinil approved (INCREASE trial) for PH-ILD; otherwise treat underlying disease and optimize oxygen Complications Right ventricular failure, cardiogenic shock Arrhythmias (atrial flutter/fibrillation poorly tolerated) Hemoptysis from dilated bronchial arteries Sudden cardiac death Maternal mortality 30-50% in PAH — pregnancy contraindicated PANCE pearls Treatment of Group 1 PAH with PAH-specific drugs can WORSEN Groups 2 (left heart) and 3 (lung disease) — always classify before treating. V/Q scan, not CTPA, is the screening test for CTEPH — sensitivity for chronic emboli is higher. Right heart catheterization is required to confirm PAH and guide therapy; echo estimates can over- or underestimate by 20 mmHg. Pregnancy in PAH carries ~30-50% maternal mortality — discuss avoidance/termination and effective contraception (no estrogen). Bosentan is teratogenic and hepatotoxic — monthly LFTs and pregnancy testing required (REMS program). References ESC/ERS 2022 — 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (Humbert et al., Eur Heart J 2022)AMBITION Trial — Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension (Galiè et al., NEJM 2015)STELLAR Trial — Sotatercept for the Treatment of Pulmonary Arterial Hypertension (Hoeper et al., NEJM 2023)INCREASE Trial — Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease (Waxman et al., NEJM 2021)
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