Pediatric Restrictive Cardiomyopathies

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2022 Feb 11

PMID 35145940

Authors

Raffaello Ditaranto

Angelo Giuseppe Caponetti

Valentina Ferrara

Vanda Parisi

Matteo Minnucci

Chiara Chiti

Riccardo Baldassarre

Federico Di Nicola

Simone Bonetti

Tammam Hasan

Luciano Potena

Nazzareno Galie

Luca Ragni

Elena Biagini

Affiliations

Soon will be listed here.

Abstract

Restrictive cardiomyopathy (RCM) is the least frequent phenotype among pediatric heart muscle diseases, representing only 2.5-3% of all cardiomyopathies diagnosed during childhood. Pediatric RCM has a poor prognosis, high incidence of pulmonary hypertension (PH), thromboembolic events, and sudden death, is less amenable to medical or surgical treatment with high mortality rates. In this , heart transplantation remains the only successful therapeutic option. Despite a shared hemodynamic profile, characterized by severe diastolic dysfunction and restrictive ventricular filling, with normal ventricle ejection fraction and wall thickness, RCM recognizes a broad etiological spectrum, consisting of genetic/familial and acquired causes, each of which has a distinct pathophysiology and natural course. Hence, the aim of this review is to cover the causes, clinical presentation, diagnostic evaluation, treatment, and prognosis of pediatric RCM.

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