What are the symptoms of pulmonary hypertension?

Symptoms of Pulmonary Hypertension

Exertional dyspnea is the hallmark and most frequent presenting symptom of pulmonary hypertension, occurring in 60% of patients at presentation and eventually developing in virtually all patients as the disease progresses. 1

Early and Presenting Symptoms

The initial symptoms of pulmonary hypertension are often nonspecific and reflect impaired cardiopulmonary function:

• Exertional dyspnea is the cardinal symptom, present in the majority of patients at diagnosis and universally as disease advances 1, 2
• Fatigue and weakness are common early complaints, resulting from impaired oxygen transport and reduced cardiac output 1
• Chest pain (angina) occurs in approximately 40% of patients during the disease course, related to right ventricular ischemia 1
• Syncope or presyncope develops in approximately 40% of patients, particularly with exertion, due to inability to augment cardiac output 1

Progressive Disease Manifestations

As pulmonary hypertension advances, symptoms worsen and new manifestations emerge:

• Dyspnea at rest indicates progression beyond early stages and reflects worsening right ventricular function 1
• Leg swelling (peripheral edema) signals right ventricular failure and systemic venous congestion 1
• Elevated jugular venous pressure with prominent V waves becomes evident on examination 3
• Hepatomegaly with pulsatile liver develops from hepatic congestion 3
• Ascites may occur in advanced right heart failure 3
• Cool extremities result from low cardiac output and peripheral vasoconstriction 3

Advanced Right Ventricular Failure Signs

Severe disease produces additional clinical findings:

• Right ventricular S3 gallop on cardiac auscultation 3
• Hypotension and diminished pulse pressure indicating severely compromised cardiac output 3
• Syncope with minimal exertion reflecting inability to maintain cerebral perfusion 3

Mechanical Complications

Pulmonary arterial dilatation can cause specific complications:

• Hemoptysis from rupture of hypertrophied bronchial arteries 3
• Hoarseness due to compression of the left recurrent laryngeal nerve 3
• Wheeze caused by large airway compression 3
• Angina from compression of the left main coronary artery leading to myocardial ischemia 3

Associated Condition Clues

Certain symptoms suggest specific underlying etiologies:

• Orthopnea and paroxysmal nocturnal dyspnea indicate left-sided cardiac disease with elevated pulmonary venous pressure (Group 2 PH) 1
• Raynaud phenomenon, arthralgias, or swollen hands raise suspicion for connective tissue disease-associated PAH 1

Functional Classification

The WHO functional classification system categorizes symptom severity into four classes that guide prognosis and treatment:

• 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 Pitfall

• Digital clubbing should raise suspicion for pulmonary veno-occlusive disease, congenital heart disease, interstitial lung disease, or liver disease rather than idiopathic pulmonary arterial hypertension 3