History and exam

Key diagnostic factors

common

presence of risk factors

Key risk factors include history of tetralogy of Fallot repair or Ross procedure, pulmonary stenosis or valvuloplasty, history of endocarditis or left-sided heart disease (e.g., left ventricular dysfunction, mitral valve stenosis).

Other diagnostic factors

common

dyspnoea

Patients may experience dyspnoea on exertion.

Worsening dyspnoea associated with leg swelling may signify right heart failure.

decreased exercise tolerance

Important to determine if there is sensation of passing out with exertion. If tolerance is getting progressively worse this requires immediate attention.

diastolic murmur

Low-pitched decrescendo murmur along the left sternal border may be heard.

uncommon

orthopnoea

If present, other valve pathologies that affect the left side of the heart should be sought.

paroxysmal nocturnal dyspnoea

If present, other valve pathologies that affect the left side of the heart should be sought.

palpitations

Atrial fibrillation, flutter, or supraventricular tachycardia are most common and should be ruled out with proper history.

fatigue

Is common when there is pulmonary hypertension leading to decreased cardiac output.

diaphoresis

In severe cases, usually with acute mitral regurgitation.

displaced point of maximal apical impulse

Indicates severe and chronic mitral regurgitation.

systolic murmur

Low-pitched murmur along the left sternal border may be heard.

signs of right-sided heart failure

These are elevated jugular venous pressure, lower extremity oedema, hepatomegaly, and ascites.

Risk factors

strong

pulmonary hypertension

Pulmonary hypertension, both primary and secondary, results in right ventricular failure and dilation and has been associated with pulmonary regurgitation.[1]​​[3]

surgical repair of tetralogy of Fallot, pulmonary stenosis, or pulmonary atresia

Repair of pulmonary atresia or stenosis is a known and significant risk factor for future pulmonary regurgitation.[1][3] Details such as balloon size used at pulmonary valvuloplasty and degree of regurgitation post valvuloplasty should be determined.

endocarditis

Any condition that may destroy the integrity of the valve leaflets may result in pulmonary regurgitation.[1][3]

left-sided heart disease

Any left-sided condition, such as severe left ventricular dysfunction, that results in pulmonary hypertension may cause pulmonary regurgitation.[1][3]

previous Ross procedure (with prosthetic pulmonary valve, homograft valve replacement)

Acquired pulmonary regurgitation has also been associated with the Ross procedure (pulmonary autograft used for aortic valve replacement).[5]

weak

collagen vascular disease

Conditions that result in pulmonary hypertension, such as scleroderma or CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly, and telangiectasia), may be secondary causes of pulmonary hypertension.[1][3]

malignancies that involve the main pulmonary artery

Very rarely, pulmonary regurgitation may be caused by primary or metastatic malignancies that involve the main pulmonary artery.

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