Clinical features

Symptoms include:

• chest pain, exertional dyspnoea, fatigue, syncope
• other symptoms due to the cause of the pulmonary hypertension, if not primary pulmonary hypertension, will be present

Signs of pulmonary hypertension include:

• small volume pulse
• peripheral cyanosis
• a raised jugular venous pulse, with a prominent a wave
• a parasternal - right ventricular - heave
• in the later stages, peripheral oedema

There may also be:

• a loud pulmonary component to the second heart sound
• a right ventricular fourth heart sound click
• a mid-diastolic systolic ejection murmur
• a Graham Steel murmur

All these heart murmurs will probably only ever be heard by the skilled cardiologist.