ESSENTIALS OF DIAGNOSIS
Most cases are due to pulmonary hypertension resulting in high-pressure pulmonary valve regurgitation.
Echocardiogram is definitive in high-pressure, but may be less definitive in low-pressure pulmonary valve regurgitation.
A 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.
Pulmonary valve regurgitation can be divided into high-pressure causes (due to pulmonary hypertension) and 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 physiology following a surgical transannular patch used to reduce the outflow gradient in tetralogy of Fallot). Because the 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.
Most patients are asymptomatic. Those with marked pulmonary valve regurgitation may exhibit symptoms of right heart volume overload. On examination, a hyperdynamic RV can usually be palpated (RV lift). If the PA is enlarged, it also may be palpated along the left sternal border. P2 will be palpable in pulmonary hypertension and both systolic and diastolic thrills are occasionally noted. On auscultation, the second heart sound may be widely split due to prolonged RV systole or an associated right bundle branch block. A pulmonary valve systolic click may be noted as well as a right-sided gallop. If pulmonic stenosis is also present, the ejection click may decline with inspiration, while any associated systolic pulmonary murmur will increase. In high-pressure pulmonary valve regurgitation, the pulmonary diastolic (Graham Steell) murmur is readily audible. It is often contributed to by a dilated pulmonary annulus. The murmur increases with inspiration and diminishes with the Valsalva maneuver. In low-pressure pulmonary valve regurgitation, the PA diastolic pressure may be only a few mm Hg higher than the RV diastolic pressure, and there is little diastolic gradient to produce a murmur or characteristic echocardiography/Doppler findings. At times, only contrast angiography or MRI of the main PA will show the free-flowing pulmonary valve regurgitation in low-pressure pulmonary valve regurgitation. This situation is common in patients following repair of tetralogy of Fallot where, despite little murmur, there may effectively be no pulmonary valve present. This can be suspected by noting an enlarging right ventricle.
The ECG is generally of little value, although right bundle branch block is common, and there may be ECG criteria for RVH. 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), and Doppler can determine peak systolic RV pressure and reveal any associated tricuspid regurgitation. The interventricular septum may appear flattened if there is pulmonary hypertension. The size of the main PA can be determined and color flow ...