AUTHOR=Ditaranto Raffaello , Caponetti Angelo Giuseppe , Ferrara Valentina , Parisi Vanda , Minnucci Matteo , Chiti Chiara , Baldassarre Riccardo , Di Nicola Federico , Bonetti Simone , Hasan Tammam , Potena Luciano , Galiè Nazzareno , Ragni Luca , Biagini Elena 

TITLE=Pediatric Restrictive Cardiomyopathies

JOURNAL=Frontiers in Pediatrics

VOLUME=9

YEAR=2022

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.745365

DOI=10.3389/fped.2021.745365

ISSN=2296-2360

ABSTRACT=<p>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 <italic>scenario</italic>, 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.</p>