The clinical and pathological features of two cases in which physiologically advantageous ventricular septal defects closed spontaneously are presented. The first patient, with tricuspid atresia, Type I(c), developed symptoms and signs of increasing systemic hypoxemia, decreasing pulmonary blood flow, and a systolic murmur of decreasing intensity. His ventricular septal defect, previously demonstrated angiocardiographically, could not be found at autopsy; it is presumed to have closed by fusion of its muscular rims with subsequent covering by endocardial proliferation. The second patient, with a double-outlet right ventricle, demonstrated progressive left ventricular enlargement and congestive failure despite increasing pulmonary vascular resistance. Postmortem examination showed that this defect was sealed by adherence of the septal leaflet of the tricuspid valve to the edges of the defect. Appreciation of the true nature of the changing anatomical situation would have resulted in more rational effective therapeutic approaches.The cases presented and review of pertinent literature contribute to more complete understanding of circumstances surrounding the spontaneous closure of ventricular septal defects.
A comparison of pressure levels and pressure-time relationships in pulmonary artery pressure (PAP) and pulmonary venous wedge pressure (PVWP) tracings was made from cardiac catheterization records of 60 patients, aged 1 day to 16 years. In 50 patients with normal or low PAP's there was close correlation between systolic, diastolic, and mean pressures in the two sites. In all 10 patients with pulmonary hypertension the PVWP's were elevated but were significantly different from corresponding PAP's. The wave contours showed qualitative similarities in most cases, but the duration of the rise in systolic pressure was longer, and the Q-pressure intervals were delayed in the PVWP tracings. Satisfactory PVWP's were obtained in 42 of 67 (63%) consecutive patients approached from the leg. Lack of correlation of pressures from the two sites in the presence of pulmonary hypertension may be attributed, in part, to decreased compliance of the pulmonary arterial system. It is concluded that the PVWP is useful in catheter evaluation of heart disease: when the PVWP is normal it usually reflects accurately the PAP; an elevated PVWP suggests an elevated PAP but cannot be used reliably as an indication of the actual arterial pressure.
AdditionalPulmonary hypertension * 572 r-1 at CONS CALIFORNIA DIG LIB on April 12, 2015 http://circ.ahajournals.org/ Downloaded from
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