Key result
Pediatric idiopathic restrictive cardiomyopathy carries a ~26% three-year survival and characteristic obliquely elevated ST-T segments.
Observational (n=12)
No
Idiopathic restrictive cardiomyopathy in children is associated with characteristic ECG repolarization abnormalities and a poor prognosis, with high mortality from sudden death or heart failure.
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High short-term mortality warrants close surveillance; leaves open optimal therapeutic strategies for this rare disease.
Hayashi et al. (2007) conducted an observational in Idiopathic restrictive cardiomyopathy (n=12). Idiopathic restrictive cardiomyopathy was evaluated on Probability of survival at 1, 2, and 3 years. Pediatric idiopathic restrictive cardiomyopathy has a poor prognosis, with survival probabilities of 78%, 52%, and 26% at 1, 2, and 3 years, respectively, and characteristic ECG abnormalities including obliquely elevated ST-T segments.
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