10th March 2024

Clinical manifestations

Symptoms and signs of pulmonary hypertension are nonspecific but similar for all types of PH. Initial presenting complaints are usually exertional dyspnoea and fatigue that progress gradually over time with no obvious clinical signs and symptoms. Clinical signs and symptoms become apparent only when overt right ventricular failure develops due to severe pulmonary hypertension.

Classical signs and symptoms of cor pulmonale (i.e. right ventricular failure) include jugular venous distension, often with a prominent V wave and positive Kussmaul sign. Pitting lower limb oedema is usually present bilaterally, as well as ascites and hepatomegaly. On cardiac examination, it may be possible to find a RV heave, a loud P2, or a pansystolic murmur (due to tricuspid regurgitation).

In some cases, when there is an underlying cause for PH, the patient may have signs and symptoms of the disease, i.e. connective tissue disease, heart failure, and chronic lung diseases. PH is an under-recognised disorder that is frequently misdiagnosed until symptoms become severe.