Pulmonary stenosis with closed ventricular septum

Abstract

The authors have selected fifteen cases of pulmonary stenosis with closed ventricular septum; in six patients could be demonstrated the presence of a patent foramen ovale or a true atrial septal defect; in two other cases there existed an anomaly of the venous return. The diagnosis was verified in two cases by autopsy, in five by surgical intervention, in twelve by cardiac catheterization, and in seven by angiocardiography. The most important clinical data for the diagnosis were the presence of loud systolic murmur accompanied by a thrill and the diminution or the absence of the second pulmonary sound. The most important radiologic finding consists in the poststenotic dilatation of the main trunk of the pulmonary artery, with clear or normal lung fields, the cardiac enlargement varying from case to case, in relation to age and the degree of stenosis. Electrocardiographically there appeared some signs of right ventricular hypertrophy in thirteen cases, and in six cases the pattern extended further than the V 1 lead. From the hemodynamic point of view the most outstanding finding was the pressure gradient between the pulmonary artery and the right ventricle; the change in pressure was found at the valvular level, except one case in which there existed an intermediate pressure zone suggestive of an infundibular chamber. Opposite from the tetralogy of Fallot, the systolic pressure of the right ventricle may be as much above as below the aortic pressure, placing itself above when the stenosis was of a severe degree. Emphasis is placed on the shape of the right intraventricular pressure curve, as well as the appearance of the Venturi curves. The angiocardiography shows poststenotic dilatation of the main trunk of the pulmonary artery, the delayed evacuation of the right cavities, and the early opacification of the left auricle, when the foramen ovale is patent. Finally, the criteria for the surgical selection of the cases is discussed because the patients with only a slight pulmonary stenosis do not call for surgical treatment.