Children with restrictive cardiomyopathy who present with ischemic symptoms despite appearing well are at risk for sudden death, prompting recommendations for screening and aggressive management including ICDs and transplant listing.
All children with RCM are at risk for ischemia-related complications and death, and some are at risk of sudden death. In the present study, patients at risk of sudden death appeared well and had no evidence of ongoing heart failure but often had signs or symptoms of ischemia characterized by chest pain, syncope, or both. ECGs and Holter monitors may be useful screening tools. The use of beta-blockade, the placement of an implantable cardioverter-defibrillator, and preferential status 1A or B listing for cardiac transplantation are proposed for pediatric patients with RCM and evidence of ongoing ischemia.
Rivenes et al. (2000) studied this question.