Journal article

Outcome of idiopathic restrictive cardiomyopathy in children

RJ Weller, R Weintraub, LJ Addonizio, MRK Chrisant, WM Gersony, DT Hsu

The American Journal of Cardiology | EXCERPTA MEDICA INC | Published : 2002


Eighteen children with idiopathic restrictive cardiomyopathy (IRC) were studied in an attempt to identify potential predictors of poor outcome. Four patients presented with low cardiac output (CO) syndrome. Fourteen remaining patients were minimally symptomatic at presentation but developed a low CO syndrome at a mean of 2.8 +/- 2.3 years after diagnosis. At the time of development of low CO in the 18 patients, mean left ventricular end-diastolic pressure was 27 mm Hg, right ventricular end-diastolic pressure was 18 mm Hg, cardiac index was 2.5 L/min/m(2), and pulmonary vascular resistance index (PVRI) was 8.8 U-m(2). Eleven of the 18 patients underwent cardiac transplantation. One died peri..

View full abstract