Signs of Impending Pulmonary Hypertension

Clinical Presentation

The European Society of Cardiology recommends that unexplained dyspnea disproportionate to underlying disease, syncope, and rapidly progressive symptoms should prompt urgent evaluation for pulmonary hypertension 1, 2
Dyspnea on exertion is the most common presenting symptom and should trigger evaluation when it occurs without overt signs of specific heart or lung disease, or when dyspnea is unexplained by the severity of underlying disease 1, 2
Syncope, particularly with exertion, is a critical warning sign indicating severely compromised cardiac output and warrants urgent referral 2
Rapidly progressing symptoms, including fatigue, weakness, and abdominal distension, should prompt immediate cardiology or respiratory referral 2
Symptoms at rest indicate very advanced disease and represent a late, ominous finding 2

Accentuated pulmonary component of S2 (loud P2) is the most consistently associated sign with PAH, though sensitivity is only 55-70% 2, 3
Left parasternal lift (RV heave) indicating right ventricular hypertrophy and pressure overload 3
Pansystolic murmur of tricuspid regurgitation reflecting elevated right ventricular pressures 2, 3
Diastolic murmur of pulmonary insufficiency from pulmonary valve incompetence 2, 3

The European Heart Society recommends that patients with connective tissue diseases, portal hypertension, HIV infection, congenital heart diseases with systemic-to-pulmonary shunts, and family history of pulmonary hypertension warrant periodic screening assessments even when asymptomatic 2, 5, 6

Chest radiograph findings that suggest developing PH include central pulmonary arterial dilatation, rapid tapering of peripheral vessels, and right heart chamber enlargement 2, 5, 6
ECG findings may show right ventricular hypertrophy and right axis deviation, but have inadequate sensitivity and specificity as a screening tool 2, 3
Transthoracic Doppler echocardiography should always be performed when PH is suspected and has sensitivity of 79-100% and specificity of 68-98% for detecting moderate PH 5

Immediate referral to cardiology/respiratory or a PH center is indicated for syncope, rapidly progressing symptoms, signs of right heart failure, and clinical or echocardiographic signs of severe PH and/or severe RV dysfunction 1, 4