The malignant course of acute rheumatic Fever in the modern era: implications for early surgical intervention in cases of bivalvular insufficiency with impaired ventricular function.

In recent years, the incidence of acute rheumatic fever (ARF) has drastically decreased in the United States while remaining common in developing nations. However, immigration to the United States from developing nations has resulted in the continued prevalence of ARF. Patients with ARF face the potential development of significant valvular insufficiency and rheumatic carditis, which are associated with significant morbidity and mortality. Potentially adverse outcomes may be avoided with advanced interventions such as aortic and/or mitral valve replacement or repair, the use of inotropic agents, and ventricular assist devices for mechanical circulatory support. This report describes a series of three ARF carditis cases. This series serves not only to emphasize proper ARF prevention, but also to demonstrate the need for timely surgical intervention once medical therapy has failed.

Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006.

Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI) >6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. x m(2) (chi(2)= 7.4, p < 0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI > 6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.