Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions
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
Affiliations
Mattia Zampieri
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Chiara Di Filippo
Local Health Unit, Outpatient Cardiology Clinic, 84131 Salerno, Italy
Chiara Zocchi
Cardiovascular Department, San Donato Hospital, 52100 Arezzo, Italy
Vera Fico
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Cristina Golinelli
Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio—Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero—Universitaria di Bologna, 40138 Bologna, Italy
Gaia Spaziani
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Giovanni Calabri
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Elena Bennati
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Francesca Girolami
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Alberto Marchi
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Silvia Passantino
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Giulio Porcedda
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Guglielmo Capponi
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Alessia Gozzini
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Iacopo Olivotto
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Luca Ragni
Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio—Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero—Universitaria di Bologna, 40138 Bologna, Italy
Silvia Favilli
Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy
Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined by increased myocardial stiffness. While suspicion of RCM is initially raised by clinical evaluation and supported by electrocardiographic and echocardiographic findings, invasive hemodynamic evaluation is often required for diagnosis and management of patients during follow-up. RCM is commonly associated with a poor prognosis and a high incidence of heart failure, and PH is reported in paediatric patients with RCM. Currently, only a few therapies are available for specific RCM aetiologies. Early referral to centres for advanced heart failure treatment is often necessary. The aim of this review is to address questions frequently asked when facing paediatric patients with RCM, including issues related to aetiologies, clinical presentation, diagnostic process and prognosis.
