Symptomatic presentation influences outcomes in pediatric restrictive cardiomyopathy.

ABSTRACT:

Introduction

Children with restrictive cardiomyopathy (RCM) traditionally have a poor prognosis, with most patients either dying or requiring heart transplantation within 2 years of diagnosis. The development of symptoms in RCM suggests advanced disease. However, as screening practices evolve and lead to diagnosis of early disease, identifying appropriate timing of transplant listing becomes increasingly important. In this context we compared outcomes of children with RCM presenting with clinical symptoms to those asymptomatic at initial presentation.

Methods

This retrospective cohort study included 25 patients with RCM presenting to a quaternary care center between 2001 and 2018. Times to transplantation, death, and a composite outcome of adverse cardiac events (CPR, cardio

SUBMITTER: Lorenzo M 

PROVIDER: S-EPMC10620919 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature