Pulmonary valve disease consists of an association of two conditions- pulmonary valve stenosis and regurgitation. It can be a congenital or acquired condition, secondary to infective endocarditis, carcinoid disease or surgical interventions for other cardiac conditions. In order to diagnose pulmonary valve disease, both stenosis and regurgitation must be concomitantly present, although they may occur at different moments in time. Furthermore, pulmonary valvular stenosis may be accompanied by subvalvular and supravalvular stenosis, as well as more or less distal pulmonary artery stenosis.
Symptoms of pulmonary valve disease are more often due to the stenosis component and depend on the severity of the condition. They consist of complaints resulting from decreased cardiac output caused by right ventricular dysfunction: dyspnea, fatigability, exertional syncope or dizziness, chest pain, peripheral edema and, in rare cases, sudden death. If a ventricular septal defect coexists, like in tetralogy of Fallot, cyanotic spells may occur. If foramen ovale is patent, right atrial pressure may exceed left atrial pressure, leading to right-to-left shunt and cyanosis .
Physical signs also depend on severity. If the stenosis component is mild, it gives rise to a short presystolic murmur and an ejection click that only appears if the valves are pliable, while in severe stenosis the murmur is more intense and becomes holo-systolic and the click disappears because of the right ventricular end-diastolic pressure being higher than the pulmonary arterial pressure. In mild cases, the second heart sound is widely split, while in severe disease patients the pulmonary component becomes inaudible. The pulmonary regurgitation, especially if severe and caused by pulmonary hypertension, causes a high-pitched diastolic murmur that might be misinterpreted as aortic regurgitation. In mild to moderate pulmonary regurgitation situations, the murmur may be absent. If infundibular or supravalvular stenosis coexists, the systolic murmur changes accordingly: it peaks later during the systole or becomes more prolonged. If the right ventricle is enlarged, in can be palpated in the epigastric region (Hartzer sign). The jugular pulse may have a prominent "A" wave. If the pulmonary regurgitation component is severe, the heart may be enlarged, pulsatile and painful .
Brain natriuretic peptide is measured initially and at follow-up visits, as it is a good indicator of disease evolution . The electrocardiogram shows right ventricular hypertrophy and right atrial dilatation, while posteroanterior and lateral chest radiography highlights a dilated pulmonary trunk and diminished pulmonary vascularization.
More information is added by echocardiography with regard to both stenosis and regurgitation components. The physician should assess valvular morphology, as well as supplementary supravalvular and subvalvular stenosis. Color doppler helps quantify the severity of the stenosis in a qualitative manner and assess the number of regurgitant flows, while pulsed and continuous wave doppler measure jet velocity and pressure gradients . Flow velocities of regurgitant jets should be measured, as well as jet acceleration time. Planimetry is not feasible, and continuity equation and proximal isovelocity surface area methods are not validated for pulmonary valve disease. Still, it is important to measure pulmonary artery pressure: systolic, mean and diastolic ; the fact that systolic pulmonary artery pressure may be as high as or exceed the systemic pressure is also suggested by an immobile or abnormal motion interventricular septum. Right ventricular enlargement should also be quantified .
Computer tomography and magnetic resonance imaging are also useful in assessing disease severity and the cardiac status . Cardiac catheterization is not usually needed in this condition unless echocardiographic and clinical findings are very different. It can more reliable to assess right ventricular and pulmonary artery pressure by "pull back" technique .