What are the clinical examination findings in patients with pulmonary hypertension?

Clinical Examination Findings in Pulmonary Hypertension

The most consistently associated physical finding with pulmonary hypertension is an accentuated pulmonary component of the second heart sound (loud P2), though its sensitivity remains modest at 55-70%. 1

Primary Cardiovascular Signs

Right Ventricular Pressure Overload

• Left parasternal lift (RV heave) indicates right ventricular hypertrophy and pressure overload 2, 1
• Accentuated P2 (loud pulmonic component of S2) is the most reliable sign, best heard at the left second intercostal space; may be audible at the apex in severe disease 2, 1, 3
• Pansystolic murmur of tricuspid regurgitation reflects elevated right ventricular pressures, heard best at the left lower sternal border and increases with inspiration 2, 1
• Diastolic murmur of pulmonary regurgitation occurs from pulmonary valve incompetence due to elevated pulmonary artery pressures 2, 1
• Right ventricular S3 gallop suggests advanced right ventricular dysfunction and carries prognostic significance 2, 1

Important caveat: While a loud P2 has the highest positive likelihood ratio (1.9 overall, 3.2 when assessed by specialists), no physical examination finding can reliably rule out pulmonary hypertension (negative likelihood ratios 0.7-1.3). 3

Signs of Advanced Disease and Right Heart Failure

Volume Overload Manifestations

• Elevated jugular venous pressure with prominent V waves indicates right atrial pressure elevation 2, 4, 1
• Hepatomegaly with pulsatile liver reflects hepatic congestion from right heart failure 2, 4, 1
• Peripheral edema in dependent areas indicates fluid retention 2, 1
• Ascites develops with severe right ventricular failure and hepatic congestion 2, 4, 1
• Cool extremities result from low cardiac output and peripheral vasoconstriction 4

Systemic Manifestations

• Syncope, especially with exertion, indicates severely compromised cardiac output and carries poor prognosis 2, 4
• Central cyanosis may be present in advanced cases 2
• Hypotension and diminished pulse pressure occur in advanced right ventricular failure 4

Respiratory Examination

Lung sounds are typically normal or clear in isolated pulmonary arterial hypertension. 2, 1 This is a critical distinguishing feature—the presence of abnormal lung sounds should prompt investigation for alternative diagnoses:

• Wheeze or crackles suggest left heart disease, interstitial lung disease, or COPD rather than isolated PAH 1
• Inspiratory crackles specifically point toward parenchymal lung disease as the underlying cause 1

Clues to Underlying Etiology

Connective Tissue Disease

• Telangiectasia, digital ulceration, and sclerodactyly suggest scleroderma-associated PAH 1

Portopulmonary Hypertension

• Spider nevi, testicular atrophy, and palmar erythema indicate portal hypertension 1

Critical Diagnostic Distinction

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
• Liver disease 4, 1

Mechanical Complications from Pulmonary Artery Dilatation

Advanced pulmonary hypertension can cause massive pulmonary arterial dilatation leading to compression syndromes:

• Hoarseness from left recurrent laryngeal nerve compression (Ortner syndrome) 4, 1
• Wheeze from large airway compression 4, 1
• Angina from left main coronary artery compression 4, 1
• Hemoptysis due to rupture of hypertrophied bronchial arteries 4
• Signs of cardiac tamponade if pulmonary artery rupture or dissection occurs—a potentially fatal complication 4, 1

Clinical Pearls for Examination

The physical examination requires experience to appreciate subtle findings. 2 Key examination strategies include:

• Assess for right-sided S4 on inspiration, which has a positive likelihood ratio of 4.7 when detected by experienced examiners 3
• Listen for P2 specifically during slow inspiration, as this increases diagnostic yield 3
• Recognize that specialist examiners demonstrate significantly higher diagnostic accuracy than generalists (positive LR 3.2 vs 1.9 for loud P2) 3

Common pitfall: Symptoms at rest are reported only in very advanced cases (WHO Functional Class IV); earlier disease may have minimal or subtle physical findings despite significant hemodynamic impairment. 2 Therefore, a normal physical examination does not exclude pulmonary hypertension, and echocardiography should be pursued when clinical suspicion exists based on unexplained dyspnea. 2