Symptoms of Pulmonary Hypertension
Exertional dyspnea is the most frequent presenting symptom of pulmonary arterial hypertension, occurring in 60% of patients at presentation and eventually developing in virtually all patients as the disease progresses. 1
Early/Presenting Symptoms
Pulmonary hypertension may be asymptomatic in its early stages, making early detection challenging. 1 When symptoms do appear, they reflect impaired oxygen transport and reduced cardiac output:
- Exertional dyspnea - the cardinal symptom and most common complaint 1, 2
- Fatigue and weakness - general exertion intolerance is common 1
- Chest pain (angina) - reported by approximately 40% of patients during disease course 1
- Syncope or presyncope - occurs in approximately 40% of patients, especially with exertion 1
Progressive Disease Symptoms
As pulmonary hypertension advances and right ventricular dysfunction develops, additional symptoms emerge:
- Dyspnea at rest - indicates disease progression beyond early stages 1
- Leg swelling (peripheral edema) - reflects right ventricular failure 1, 3
- Abdominal bloating and distension - from hepatic congestion and ascites 1, 3
- Anorexia - develops with right heart failure 1
- More profound fatigue - worsens as cardiac output declines 1
Signs of Advanced Right Ventricular Failure
The European Respiratory Journal describes specific manifestations of severe right ventricular dysfunction:
- Elevated jugular venous pressure with prominent V waves 3
- Hepatomegaly and pulsatile liver 3
- Cool extremities - due to low cardiac output and peripheral vasoconstriction 3
- Hypotension and diminished pulse pressure 3
- Right ventricular S3 gallop 3
Associated Condition Symptoms
Critical caveat: Certain symptoms suggest specific underlying causes rather than idiopathic pulmonary arterial hypertension:
- Orthopnea and paroxysmal nocturnal dyspnea - suggest left-sided cardiac disease with elevated pulmonary venous pressure 1
- Raynaud phenomenon, arthralgias, or swollen hands - raise possibility of connective tissue disease-associated PAH 1
- Snoring or witnessed apnea - warrants evaluation for sleep-disordered breathing 1
- Digital clubbing - should raise suspicion for pulmonary veno-occlusive disease, congenital heart disease, interstitial lung disease, or liver disease rather than idiopathic PAH 3
Mechanical Complications
The European Respiratory Journal notes that pulmonary arterial dilatation can cause:
- Hemoptysis - from rupture of hypertrophied bronchial arteries 3
- Hoarseness - from compression of left recurrent laryngeal nerve 3
- Wheeze - from large airway compression 3
Functional Classification
Symptoms are categorized using the WHO functional classification system, which guides prognosis and treatment decisions 1:
- Class I: No limitation; ordinary activity does not cause symptoms 1
- Class II: Slight limitation; comfortable at rest but ordinary activity causes dyspnea, fatigue, chest pain, or presyncope 1
- Class III: Marked limitation; comfortable at rest but less than ordinary activity causes symptoms 1
- Class IV: Unable to perform any activity without symptoms; signs of right heart failure may be present at rest 1
Important clinical pearl: The presence and rate of progression of symptoms, particularly syncope and signs of right ventricular failure, are critical prognostic indicators, with rapid progression indicating worse prognosis. 1