Elevation of pulmonary artery (PA) pressure due to pulmonary vascular or parenchymal disease, increased left heart filling pressures, or a combination. Table 127-1 lists etiologies by categories.
Exertional dyspnea, fatigue, angina (due to RV ischemia), syncope, peripheral edema.
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 hypertrophy; RV systolic pressure can be estimated from Doppler recording of tricuspid regurgitation. Pulmonary function tests (PFTs) 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, rheumatoid factor, anti-Scl-70 antibodies are elevated in specific collagen vascular conditions that can result in pulmonary hypertension. HIV testing should be performed in individuals at risk. Cardiac catheterization accurately assesses PA pressures, cardiac output, and pulmonary vascular resistance, and quantifies underlying congenital vascular shunts; during procedure, response to short-acting vasodilators can be assessed.
Figure 127-1 summarizes a testing approach for pts with unexplained pulmonary hypertension.
Outline