Skip to Main Content



Elevation of pulmonary artery (PA) pressure due to pulmonary vascular or parenchymal disease, increased left heart filling pressures, or a combination. Table 136-1 lists etiologies by underlying categories.



Exertional dyspnea, fatigue, angina (due to RV ischemia), syncope, peripheral edema.

Physical Examination

Jugular venous distention, RV lift, increased P2, right-sided S4, tricuspid regurgitation. Peripheral cyanosis and edema are late manifestations.


CXR shows enlarged central PA. ECG may demonstrate RV hypertrophy and RA enlargement. Echocardiogram shows RV and RA enlargement; RV systolic pressure can be estimated from Doppler recording of tricuspid regurgitation (Chap. 120). Pulmonary function tests identify underlying obstructive or restrictive lung disease; impaired CO diffusion capacity is common. Chest CT identifies contributing interstitial lung disease or pulmonary thromboembolic disease. ANA titer is elevated in collagen vascular diseases. HIV testing should be performed in individuals at risk. Cardiac catheterization accurately assesses PA pressures, cardiac output, and pulmonary vascular resistance, and it identifies underlying congenital vascular shunts; during procedure, response to short-acting vasodilators can be assessed.

Figure 136-1 summarizes workup of pt with unexplained pulmonary hypertension.

FIGURE 136-1

An algorithm for the workup of a pt with unexplained pulmonary hypertension. All potential etiologies and associated conditions must be investigated in a pt with clinical findings consistent with pulmonary hypertension. ANA, antinuclear antibodies; CBC, complete blood count; COLD, chronic obstructive lung disease; HIV, human immunodeficiency virus; LFTs, liver function tests; TSH, thyroid-stimulating hormone.


Uncommon (2 cases/million), very serious form of pulmonary hypertension. Most pts present in fourth and fifth decades, female ≫ male predominance; up to 20% of cases are familial. Major symptom is dyspnea, often with insidious onset. Mean survival <3 years in absence of therapy.

Physical Examination

Prominent a wave in jugular venous pulse, right ventricular heave, narrowly split S2 with accentuated P2. Terminal course is characterized by signs of right-sided heart failure.

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.