Clinical spectrum of restrictive cardiomyopathy in children

We reviewed the clinical spectrum and possible prognostic factors in 14 chi ldren with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respir atory symptoms, thromboembolism, increased cardiothoracic ratio on chest ra diogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.