11th May 2025

Clinical Manifestations

Most commonly, PH affects females between 20 and 50 years. Presentation may be precipitated by use of stimulants such as cocaine. PH presents with progressive dyspnoea and fatigue on exertion. Other exertional symptoms include cardiac chest pain and presyncope/syncope, which may not appear until the onset of right sided heart failure. Given that the symptoms of PH are not specific, diagnosis is often delayed. 

Symptoms can be classified by WHO functional status. This is useful for marking severity, prognosis and outcome to therapies.

Table 1: WHO functional classification of PH

Examination signs are absent or subtle in early disease and again generally occur after onset of right sided cardiac failure.  

Examination findings include: 

• Cyanosis
• Right ventricular heave – indicating right heart hypertrophy
• Loud P2 – accentuated pulmonary component of second heart sound 
• Holosystolic murmur – functional tricuspid regurgitation
• Signs of right heart failure: raised JVP, peripheral oedema, hepatomegaly, ascites. 
• Note that the lungs remain clear on auscultation (unless underlying lung disease)

In secondary PH, additional clinical features will depend on the underlying aetiology. When related to systemic sclerosis, one may expect to see sclerodactyly, Reynaud’s, polyarthralgia, telangiectasia, dysphagia and additional rheumatological manifestations associated with this condition. In the presence of left sided cardiac failure, signs of pulmonary congestion may be present, such as coarse bi-basal crackles, orthopnoea and paroxysmal nocturnal dyspnoea. Interstitial lung disease may reveal fine inspiratory bi-basal crackles. 

This illustrates the importance of a thorough history and examination in guiding the clinician towards the cause of suspected PH.