Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions

• DOI: 10.3390/diagnostics13243666
• Publication Date: 2023-12-15T11:53:16Z
• Authors: Mattia Zampieri; Chiara Di Filippo; Chiara Zocchi; Vera Fico; Cristina Golinelli; Gaia Spaziani; Giovanni Calabri; Elena Bennati; Francesca Girolami; Alberto Marchi; Silvia Passantino; Giulio Porcedda; Guglielmo Capponi; Alessia Gozzini; Iacopo Olivotto; Luca Ragni; Silvia Favilli
• Keywords: Restrictive cardiomyopathy; Presentation (obstetrics)

Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined increased myocardial stiffness. While suspicion of RCM initially raised clinical evaluation and supported electrocardiographic echocardiographic findings, invasive hemodynamic often required for diagnosis management patients during follow-up. commonly associated with a poor prognosis high incidence heart failure, PH reported in paediatric RCM. Currently, only few therapies are available specific aetiologies. Early referral to centres advanced failure treatment necessary. The aim this review address questions frequently asked when facing RCM, including issues related aetiologies, presentation, diagnostic process prognosis.