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Table 13. Assessment for Pulmonary Arterial Hypertension in
Adults With Congenital Heart Disease
Assessment
Type Findings
Clinical history,
examination
• Reduced functional capacity
• Symptoms:
▶ Functional: dyspnea, fatigue
▶ Right heart failure: congestion, abdominal distention, early
satiety
• History of near-syncope
• Desaturation at rest
• Cyanosis
• Clubbing
• Accentuated S2 heart sound
• Jugular venous distention, distended abdomen, peripheral edema
Exercise
capacity
• Reduced 6-minute walk distance
• Possibly reduced resting or exercising oxygen saturation
• Abnormal lung function, at rest and during exercise
Imaging • Electrocardiogram: right ventricular enlargement
• Transthoracic echocardiography: RVE, flattened IVS, RAE, peak
TR jet >2.9 m/s, RVOT acceleration time <105 ms, TAPSE <18 mm
Biomarkers Elevated BNP or NT-proBNP
Hemodynamics • mPAP >20 mm Hg and
• PVR >2 Wood units and
• PCWP ≤15 mm Hg
BNP indicates B-type natriuretic peptide; CT, computed tomography; IVS, interventricular
septum; JVD, jugular venous distention; mPAP, mean pulmonary artery pressure; NT-
proBNP, N-terminal prohormone of B-type natriuretic peptide; PAH, pulmonary arterial
hypertension; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular
resistance; RAE, right atrial enlargement; RVE, right ventricular enlargement; RVOT,
right ventricular outflow tract; TAPSE, tricuspid annular plane systolic excursion; and TR,
tricuspid regurgitation.
