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Key Features

Essentials of Diagnosis

  • Most cases are due to pulmonary hypertension resulting in high-pressure pulmonary valve regurgitation

  • Echocardiogram is definitive in high-pressure pulmonary valve regurgitation but may be less definitive in low-pressure regurgitation

  • Loud diastolic (Graham–Steell) murmur in high-pressure pulmonary valve regurgitation

  • Soft or no murmur in low-pressure pulmonary valve regurgitation

  • Low-pressure pulmonary valve regurgitation is well tolerated

General Considerations

  • Can be divided into high-pressure and low-pressure causes

  • High-pressure causes are due to pulmonary hypertension

  • Low-pressure causes usually due to

    • – A dilated pulmonary annulus

    • – A congenitally abnormal (bicuspid or dysplastic) pulmonary valve

    • – Plaque from carcinoid disease

    • – Surgical pulmonary valve replacement or the residual from a surgical transannular patch used to reduce the outflow gradient in tetralogy of Fallot

  • Because the right ventricle (RV) tolerates a volume load better than a pressure load, it tends to tolerate low pressure pulmonary valve regurgitation for long periods of time without dysfunction

Clinical Findings

  • Most patients are asymptomatic; those with marked PR show symptoms of right heart volume overload

  • A hyperdynamic RV can usually be palpated

  • If the pulmonary artery (PA) is enlarged, it may be palpated along the left sternal border

  • P2 will be palpable in pulmonary hypertension and both systolic and diastolic thrills are occasionally noted

  • In high-pressure pulmonary valve regurgitation, a loud diastolic murmur, often due to a dilated pulmonary annulus, increasing with inspiration and decreasing with Valsalva maneuver

  • The second heart sound may be widely split due to prolonged RV systole or associated right bundle branch block

  • A pulmonary valve systolic click may be noted as well as a right-sided gallop

  • If pulmonary valve stenosis is also present, the ejection click may decline with inspiration while any associated systolic pulmonary murmur increases

Diagnosis

Imaging Studies

  • The chest radiograph may show only the enlarged RV and PA

  • Echocardiography may demonstrate

    • – Evidence of RV volume overload (paradoxic septal motion and an enlarged RV)

    • – Peak systolic RV pressure

    • – Any associated tricuspid regurgitation

    • – Size of PA

  • Cardiac MRI and CT can be useful for

    • – Assessing the size of the PA

    • – Estimating regurgitant flow

    • – Excluding other causes of pulmonary hypertension (eg, thromboembolic disease, peripheral PA stenosis)

    • – Evaluating RV function

Diagnostic Studies

  • ECG is generally of little value except that

    • – A right bundle branch block is common

    • – ECG criteria for right ventricular hypertrophy (RVH) may be present

  • At times, only contrast angiography or MRI of the main PA will show the free flowing regurgitation in low-pressure regurgitation

  • Cardiac catheterization is confirmatory only

Treatment

  • Specific therapy other than treatment of the primary cause rarely needed

  • In high-pressure pulmonary valve regurgitation, controlling the cause of the pulmonary hypertension is key

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