Pulmonary valvular stenosis with intact ventricular septum: Isolated valvular stenosis and valvular stenosis associated with interatrial shunt

Abstract

1. 1. Wide-spread application of cardiac catheterization in more accurate definition of congenital heart disease has dispelled the concept of rarity of isolated pulmonary valvular stenosis. 2. 2. Clinical recognition may be fairly easy in the more severe cases. The characteristic clinical features are increasing exertional dyspnea and fatigue with increasing age, normal physical growth and development except where cyanosis is evident, marked systolic thrill and murmur maximal in the second and third left intercostal spaces and decreased or absent pulmonary second sound. Cyanosis is absent when an interatrial shunt is not present. 3. 3. The shunting of blood from right to left through an atrial defect or patent foramen ovale results in slight to severe cyanosis which varies with the amount of shunted blood. These individuals usually are more restricted, and growth and physical impairment are common. 4. 4. When the pulmonary valvular stenosis is less severe, the clinical diagnosis may be more difficult due to absence of fatigue and dyspnea, and great exercise tolerance and physical vigor may be prominent. In these instances the thrill and murmur may not be characteristic and a “septal defect” may be suspected. 5. 5. The anatomic and physiologic alterations can be precicted from the high resistance of the pulmonary valve, and considered in this light, the increasing right ventricular hypertrophy expresses itself in the electrocardiographic, x-ray, and fluoroscopic configuration of the heart, and in catheterization and in angiocardiographic findings. 6. 6. Despite a high degree of clinical suspicion and recognition of this congenital anomaly, we believe it is not possible to differentiate by clinical means among some of the varieties of subvalvular stenosis. Therefore, venous catheterization of the heart and angiocardiography should be utilized whenever possible. Cardiac catheterization characteristically shows a low pressure in the pulmonary artery and a higher pressure in the right ventricle which is proportional to the degree of valvular stenosis. Meticulous exploration of the region of stenosis will characteristically show an increasing negative pressure as the catheter is withdrawn from the pulmonary artery into the stream of the valve orifice (Venturi curves) and an abrupt change to the high pressure of the right ventricle.