What are the signs and symptoms of pulmonary hypertension?

Signs and Symptoms of Pulmonary Hypertension

Exertional dyspnea is the hallmark 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

Dyspnea and Exercise Intolerance:

• Exertional dyspnea reflects early right ventricular dysfunction and impaired oxygen delivery, making it the most frequent initial complaint 2, 1
• Fatigue and weakness commonly accompany dyspnea due to reduced cardiac output and impaired oxygen transport 2, 1
• Exercise intolerance progressively worsens as the disease advances 3

Cardiovascular Symptoms:

• Chest pain (angina-like discomfort) occurs in approximately 40% of patients during the disease course 2, 1
• Syncope or presyncope, especially with exertion, signals severely compromised cardiac output and occurs in approximately 40% of patients—this warrants urgent referral 2, 1

Less Common Early Symptoms:

• Dry cough can be present but is less frequent 2
• Exercise-induced nausea or vomiting may occur 2

Physical Examination Findings

Primary Cardiovascular Signs:

• An accentuated pulmonary component of the second heart sound (loud P2) is the most consistently associated sign with pulmonary hypertension, though sensitivity remains modest at 55-70% 2
• A left parasternal lift (right ventricular heave) indicates right ventricular hypertrophy and pressure overload 2
• A pansystolic murmur of tricuspid regurgitation reflects elevated right ventricular pressures 2
• A diastolic murmur of pulmonary regurgitation occurs due to pulmonary valve incompetence from elevated pulmonary artery pressures 2
• A right ventricular third heart sound (S3) suggests advanced right ventricular dysfunction 2

Signs of Advanced Disease and Right Heart Failure:

• Elevated jugular venous pressure with prominent V waves indicates right atrial pressure elevation 2, 4
• Hepatomegaly with pulsatile liver reflects hepatic congestion from right heart failure 2, 4
• Peripheral edema in dependent areas (leg swelling) indicates fluid retention and right ventricular failure 2, 1, 4
• Ascites develops with severe right ventricular failure and hepatic congestion 2, 4
• Cool extremities result from low cardiac output and peripheral vasoconstriction 2, 4
• Abdominal distension from hepatic congestion 2, 4
• Dyspnea at rest indicates disease progression beyond early stages 2, 1

Respiratory Examination:

• Lung sounds are typically normal or clear in isolated pulmonary arterial hypertension 2
• The presence of wheeze or crackles should prompt investigation for alternative diagnoses such as left heart disease, interstitial lung disease, or COPD 2

Mechanical Complications from Pulmonary Artery Dilatation

These complications occur in advanced disease due to massive pulmonary artery enlargement:

• Hemoptysis from rupture of hypertrophied bronchial arteries 2, 4
• Hoarseness from left recurrent laryngeal nerve compression (Ortner syndrome) 2, 4
• Wheeze from large airway compression 2, 4
• Angina from left main coronary artery compression 2, 4
• Signs of cardiac tamponade if pulmonary artery rupture or dissection occurs 2, 4

Clues to Underlying Etiology

Connective Tissue Disease:

• Telangiectasia, digital ulceration, and sclerodactyly suggest scleroderma-associated pulmonary arterial hypertension 2
• Raynaud phenomenon, arthralgias, or swollen hands raise the possibility of connective tissue disease-associated pulmonary arterial hypertension 1

Portopulmonary Hypertension:

• Spider nevi, testicular atrophy, and palmar erythema indicate portopulmonary hypertension 2

Parenchymal Lung Disease:

• Inspiratory crackles point toward parenchymal lung disease as the underlying cause 2

Left Heart Disease:

• Orthopnea and paroxysmal nocturnal dyspnea suggest left-sided cardiac disease with elevated pulmonary venous pressure 1

Critical Diagnostic Distinction

Digital clubbing is NOT typical of idiopathic pulmonary arterial hypertension and should raise suspicion for pulmonary veno-occlusive disease, cyanotic congenital heart disease, interstitial lung disease, or liver disease. 2, 4

WHO Functional Classification

The World Health Organization functional classification system categorizes symptom severity into four classes, guiding 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

Common Diagnostic Pitfalls

The nonspecific nature of pulmonary hypertension symptoms frequently leads to diagnostic delays. 2

• A normal chest radiograph or electrocardiogram does NOT exclude pulmonary hypertension; persistent unexplained dyspnea or risk factors should prompt advanced imaging such as echocardiography 2
• Lack of response to conventional dyspnea therapies should alert clinicians to consider pulmonary hypertension as an alternative cause 2, 5
• Syncope may be the presenting symptom, especially in children 5
• Patients may present with complaints unrelated to pulmonary hypertension, but this condition will affect all aspects of care 6