What are the symptoms of pulmonary arterial hypertension (PAH) and pulmonary hypertension (PH)?

Symptoms of Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension (PH)

The symptoms of PAH and PH are nonspecific and primarily reflect progressive right ventricular dysfunction, with exertional dyspnea being the most common presenting symptom, followed by fatigue, weakness, chest pain, and syncope—symptoms at rest indicate advanced disease. 1

Primary Symptoms (Exertion-Induced)

Most Common Presenting Symptoms

• Exertional dyspnea is the hallmark symptom, occurring in 60% of patients at presentation and eventually developing in virtually all patients as disease progresses 2
• Fatigue and weakness reflect impaired oxygen transport and reduced cardiac output 1, 2
• Chest pain (angina) occurs in approximately 40% of patients during the disease course 2
• Syncope or presyncope occurs in approximately 40% of patients, especially with exertion, and represents a critical warning sign indicating severely compromised cardiac output that warrants urgent referral 3, 2

Less Common Symptoms

• Dry cough may be present 1
• Exercise-induced nausea and vomiting can occur 1

Advanced Disease Symptoms (Rest Symptoms)

Symptoms at rest occur only in advanced cases and represent a late, ominous finding. 1, 3

• Dyspnea at rest indicates disease progression beyond early stages 2
• Abdominal distension develops with progressing right ventricular failure 1
• Ankle edema (peripheral edema) reflects right ventricular failure 1, 2

Physical Examination Findings

Cardiovascular Signs

• Accentuated pulmonary component of S2 (loud P2) is the most consistently associated sign with PAH, though sensitivity is only 55-70% 3, 4
• Left parasternal lift (RV heave) indicates right ventricular hypertrophy and pressure overload 3, 4
• Pansystolic murmur of tricuspid regurgitation reflects elevated right ventricular pressures 1, 4
• Diastolic murmur of pulmonary regurgitation occurs from pulmonary valve incompetence 1, 4
• Right ventricular third heart sound (S3) suggests advanced right ventricular dysfunction 4

Signs of Advanced Disease and Right Heart Failure

• Elevated jugular venous pressure with prominent V waves indicates right atrial pressure elevation 4
• Hepatomegaly with pulsatile liver reflects hepatic congestion from right heart failure 1, 4
• Ascites develops with severe right ventricular failure and hepatic congestion 1, 4
• Cool extremities characterize patients with advanced disease 1

Important Respiratory Examination Finding

• Wheeze and crackles are usually absent in isolated PAH 1
• The presence of wheeze or crackles should prompt investigation for alternative diagnoses such as left heart disease, interstitial lung disease, or COPD 4

Mechanical Complications from Pulmonary Artery Dilatation

These symptoms relate to the abnormal distribution of blood flow and mechanical effects of dilated pulmonary arteries: 1

• Hemoptysis related to rupture of hypertrophied bronchial arteries 1
• Hoarseness caused by compression of the left recurrent laryngeal nerve (Ortner syndrome) 1, 4
• Wheeze caused by large airway compression 1, 4
• Angina due to myocardial ischemia from compression of the left main coronary artery 1, 4
• Signs of cardiac tamponade if pulmonary artery rupture or dissection occurs 1, 4

Clues to Underlying Etiology on Physical Examination

• Telangiectasia, digital ulceration, and sclerodactyly suggest scleroderma-associated PAH 1, 4
• Spider nevi, testicular atrophy, and palmar erythema indicate portopulmonary hypertension 1, 4
• Inspiratory crackles point toward parenchymal lung disease as the underlying cause 1, 4
• Digital clubbing is NOT typical of idiopathic PAH and should raise suspicion for pulmonary veno-occlusive disease (PVOD), cyanotic congenital heart disease, interstitial lung disease, or liver disease 1, 4

Critical Warning Signs Requiring Urgent Evaluation

Unexplained dyspnea disproportionate to underlying disease, syncope, and rapidly progressive symptoms should prompt urgent evaluation for pulmonary hypertension. 3

• Syncope, particularly with exertion, warrants urgent referral as it indicates severely compromised cardiac output 3
• Rapidly progressing symptoms, including fatigue, weakness, and abdominal distension, should prompt immediate cardiology or respiratory referral 3
• Dyspnea on exertion should trigger evaluation when it occurs without overt signs of specific heart or lung disease, or when dyspnea is unexplained by the severity of underlying disease 3

Common Pitfalls

The nonspecific nature of symptoms leads to frequent diagnostic delays. 1, 5 A normal chest radiograph or ECG does not exclude PH, and further imaging evaluation should be pursued if there are persistent unexplained symptoms such as dyspnea or risk factors for PH. 1 Lack of response to conventional treatment for dyspnea should alert clinicians to search for another cause, one of which could be PH. 6