Abstract

Objective: To define the natural history of idiopathic restrictive cardiomyopathy in a paediatric population and to identify any factors predictive of outcome.

Design: Retrospective analysis of patients born between 1970 and 2002 were identified from the Children’s Hospital of Pittsburgh cardiology database. Demographic data, mode of presentation, echocardiographic and haemodynamic findings at diagnosis, survival time, and manner of death were evaluated.

Setting: Tertiary referral and transplant centre for paediatric patients with cardiac disease.

Patients: All local and referred patients with idiopathic restrictive cardiomyopathy born after 1970 and under 21 years of age at time of diagnosis.

Results: 21 patients were identified. Probability of survival at 1, 5, and 10 years was 80.5% (95% confidence interval (CI) 58 to 100), 39% (95% CI 17 to 61), and 20% (95% CI 0 to 42), respectively. Median age of presentation was 3.8 years (mean (SD) 5.7 (6.1) years). Median survival without transplantation was 2.2 years (mean (SD) 4.6 (5.4) years). Age at presentation, sex, and presence or absence of heart failure symptoms at presentation were not associated with clinical course. Right (p  =  0.05) and left ventricular end diastolic pressures (p  =  0.04) and ratio of left atrial to aortic root dimensions (LA:Ao) (p  =  0.03) at presentation had a significantly negative correlation with survival time after diagnosis.

Conclusions: Without transplantation, most children with restrictive cardiomyopathy have a very poor prognosis. Longer survival from diagnosis was correlated with lower LA:Ao and cardiac filling pressures at diagnosis. Survival time was not influenced by the symptoms present at diagnosis.