Symptoms of Pulmonary Hypertension
Primary Presenting Symptoms
Exertional dyspnea is the hallmark symptom of pulmonary hypertension, occurring in 60% of patients at presentation and eventually developing in virtually all patients as the disease progresses. 1 This breathlessness on exertion is often the first symptom that prompts medical evaluation and should trigger investigation when it appears disproportionate to any underlying heart or lung disease. 2, 3
Common Early Symptoms
- Fatigue and weakness are frequent complaints reflecting impaired oxygen transport and reduced cardiac output. 2, 1
- Chest pain (angina) is reported by approximately 40% of patients during the disease course, sometimes due to right ventricular ischemia or compression of the left main coronary artery by a dilated pulmonary artery. 2, 1
- Syncope or presyncope occurs in approximately 40% of patients, particularly with exertion, and represents a critical warning sign of severely compromised cardiac output. 2, 1, 3
- Palpitations may occur as the heart attempts to compensate for reduced cardiac output. 2
Advanced Disease Symptoms
- Dyspnea at rest indicates progression beyond early stages and represents advanced disease. 2, 1
- Peripheral edema (ankle swelling) develops with progressing right ventricular failure. 2
- Abdominal distension and hepatomegaly occur as right heart failure worsens, leading to hepatic congestion. 2
- Ascites develops in advanced right ventricular failure. 2
Less Common Symptoms
- Dry cough may occur in some patients. 2
- Exercise-induced nausea and vomiting can develop. 2
- Hemoptysis may result from rupture of hypertrophied bronchial arteries. 2
- Hoarseness can occur from compression of the left recurrent laryngeal nerve by a dilated pulmonary artery. 2
- Wheeze may result from large airway compression by dilated pulmonary arteries. 2
Important Clinical Context
The symptoms of pulmonary hypertension are nonspecific and mainly related to progressive right ventricular dysfunction. 2 Initial symptoms are typically induced by exertion, with symptoms at rest occurring only in advanced cases. 2 The presentation may be modified by underlying diseases that cause or are associated with pulmonary hypertension. 2
Red Flag Symptoms Requiring Urgent Evaluation
- Syncope with exertion demands urgent referral as it indicates critically compromised cardiac output. 3, 4
- Rapidly progressive symptoms including worsening fatigue, weakness, and abdominal distension warrant immediate cardiology or respiratory referral. 3
- Unexplained dyspnea disproportionate to underlying disease should prompt urgent evaluation. 3
Symptoms Suggesting Specific Underlying Causes
- Orthopnea and paroxysmal nocturnal dyspnea suggest left-sided cardiac disease with elevated pulmonary venous pressure rather than primary pulmonary arterial hypertension. 1, 4
- Raynaud phenomenon, arthralgias, or swollen hands raise the possibility of connective tissue disease-associated pulmonary arterial hypertension. 1