Pulmonology

Pulmonary Hypertension song

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Pulmonary Hypertension

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Pulmonary hypertension is defined as mean pulmonary arterial pressure ≥20 mmHg at rest measured by right heart catheterization, which is the gold standard for diagnosis.
Group 1 (PAH) is associated with idiopathic disease, heritable mutations in BMPR2 (a TGF-β receptor), connective tissue diseases (especially scleroderma), HIV, portal hypertension, and anorexigens such as fenfluramine.
The most common cause of pulmonary hypertension overall is left heart disease (Group 2), where elevated left-sided filling pressures are transmitted backward into the pulmonary vasculature.
Hypoxic pulmonary vasoconstriction is the key mechanism in Group 3 (lung disease/hypoxia), making COPD and obstructive sleep apnea major contributors in this category.
Chronic thromboembolic pulmonary hypertension (Group 4) is the only potentially surgically curable form, treated with pulmonary thromboendarterectomy.
Classic exam presentation includes progressive exertional dyspnea, loud P2, right ventricular heave, tricuspid regurgitation murmur, and signs of right heart failure such as JVD, peripheral edema, and ascites.
Echocardiography is the preferred initial screening test, showing right ventricular hypertrophy and elevated estimated right ventricular systolic pressure via tricuspid regurgitant jet velocity.
Vasoreactivity testing with inhaled nitric oxide during right heart catheterization identifies patients who may respond to calcium channel blockers such as nifedipine or diltiazem.
Prostacyclin analogs (epoprostenol), endothelin receptor antagonists (bosentan, ambrisentan), and PDE-5 inhibitors (sildenafil, tadalafil) are the three major pharmacologic classes used in Group 1 PAH treatment.
Bosentan is teratogenic and requires REMS enrollment; sildenafil is contraindicated with nitrates due to risk of severe hypotension.