Abstract
Restrictive cardiomyopathy (RCM) in children is associated with a greater risk of embolic stroke than are other congenital heart diseases. After diagnosis, 50% of children with RCM die within 2 years without heart transplantation. As such, all RCM patients are placed on the heart transplantation list and must wait for an appropriate heart for transplantation. Every type of embolic stroke can occur while waiting for a donor heart; therefore, the cardiovascular team must initiate antithrombotic therapy at time RCM is diagnosed. Some pediatric RCM patients experience embolic stroke (50% are the cerebral type) despite antithrombotic therapy, including acetylsalicylic acid, warfarin, and heparine. Neurosurgeons working in hospitals that perform organ transplantation expect to see RCM cases with restrictive large cerebral infarct. We think that decompressive craniectomy should be performed as soon as possible after determining the clinical condition of any patient with RCM and a large right middle cerebral artery (MCA) infarct.
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