What are the signs and symptoms of pulmonary hypertension?

Signs and Symptoms of Pulmonary Hypertension

Exertional dyspnea is the cardinal presenting symptom of pulmonary hypertension, occurring in 60% of patients at presentation and eventually developing in virtually all patients, and should trigger urgent evaluation when unexplained by underlying disease severity. 1, 2

Most Common Presenting Symptoms

• Dyspnea on exertion is the most frequent initial complaint, warranting evaluation particularly when it occurs without overt signs of specific heart or lung disease or when disproportionate to the severity of known underlying conditions 1, 2
• Fatigue and weakness are common early complaints reflecting impaired oxygen transport and reduced cardiac output 2
• Chest pain (angina) is reported by approximately 40% of patients during the disease course 2
• Syncope or presyncope, especially with exertion, occurs in approximately 40% of patients and represents a critical warning sign indicating severely compromised cardiac output that warrants urgent referral 1, 2

Physical Examination Findings

Cardiac Signs

• Accentuated pulmonary component of S2 (loud P2) is the most consistently associated sign with pulmonary arterial hypertension, though sensitivity is only 55-70% 1
• Left parasternal lift (RV heave) indicating right ventricular hypertrophy and pressure overload 1
• Pansystolic murmur of tricuspid regurgitation reflecting elevated right ventricular pressures 1
• Diastolic murmur of pulmonary insufficiency from pulmonary valve incompetence 1

Signs of Right Ventricular Failure (Advanced Disease)

• Elevated jugular venous pressure with prominent V waves 3
• Hepatomegaly and pulsatile liver from hepatic congestion 3
• Peripheral edema and ascites 2, 3
• Cool extremities due to low cardiac output and peripheral vasoconstriction 3
• Right ventricular S3 gallop 3
• Hypotension and diminished pulse pressure 3

Progressive Disease Indicators

• Rapidly progressing symptoms including fatigue, weakness, and abdominal distension should prompt immediate cardiology or respiratory referral 1
• Dyspnea at rest indicates very advanced disease and represents a late, ominous finding 1, 2
• Symptoms at rest generally indicate disease progression beyond early stages 1

Associated Condition Clues

• Orthopnea and paroxysmal nocturnal dyspnea suggest left-sided cardiac disease with elevated pulmonary venous pressure 2
• Raynaud phenomenon, arthralgias, or swollen hands raise the possibility of connective tissue disease-associated pulmonary arterial hypertension 2
• 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

Mechanical Complications (Advanced Disease)

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

Arrhythmic Manifestations

• Supraventricular arrhythmias (atrial flutter and atrial fibrillation) occur in advanced disease, compromising cardiac output and leading to clinical deterioration 3
• Ventricular arrhythmias are rare but may occur 3

High-Risk Populations Requiring Screening

Patients with the following conditions warrant periodic screening assessments even when asymptomatic: 1

• Connective tissue diseases
• Portal hypertension
• HIV infection
• Congenital heart diseases with systemic-to-pulmonary shunts
• Family history of pulmonary hypertension

Critical Warning Signs Requiring Urgent Referral

Immediate referral to cardiology/respiratory or a pulmonary hypertension center is indicated for: 1

• Syncope, particularly with exertion
• Rapidly progressing symptoms
• Signs of right heart failure
• Clinical or echocardiographic signs of severe pulmonary hypertension and/or severe right ventricular dysfunction

Common Pitfalls

• The European Society of Cardiology emphasizes that unexplained dyspnea disproportionate to underlying disease should prompt urgent evaluation rather than being dismissed as deconditioning or anxiety 1
• Symptoms are often nonspecific and can be mistaken for other cardiopulmonary conditions, contributing to diagnostic delays averaging more than one year, and exceeding three years in 20% of patients 4
• The presence of normal ECG and (NT-pro-)BNP makes pulmonary hypertension unlikely and can help avoid unnecessary testing 4