Cardiomyopathies in Children

Abstract

Introduction: Cardiomyopathies (CM) account for 3-5% of patients in the care of pediatric cardiologists. They are found in all age groups, from fetal to adolescent age, and along with cardiology, teams from several other pediatric subspecialties (neurology, metabolism, genetics) are also included. New findings have led to a high survival rate. Goal: The primary goal is to present CM as an important part in the work of pediatric cardiologist through an elaborate epidemiological study, current classifications, the latest diagnostic methods and treatments, as well as the intertwining with other subspecialties. The secondary goal is to show that CM are no longer “uncommon, insignificant and terminal”, but are common, significant and treatable diseases. Results: From January 1988 to December 2016 (28 years) in the Referral Center for Pediatric Cardiology, Department of Pediatrics, Clinical Hospital Centre, 315 patients were diagnosed with cardiomyopathy,183 males (58.1%) and 132 females (41.9%). In three different periods (10 , 12 and six years) a classification from 1996 was used (10). All three periods have features of a population study, whereas the latter two also have features of an epidemiological study. In all three periods there was a predominance of dilated cardiomyopathies (DCM) (42.5%) , followed by hypertrophic cardiomyopathies (HCM) (37.1%) and restrictive cardiomyopathies (RCM) (6.7%) . Their relative relations were in constant balance. A significant increase of some entity forms, arrhythmogenic right ventricular cardiomyopathies (ARVCM) and non-compaction cardimyopathies (NCCM) has been observed, from 5.8% (1988-1998) to 16.2% (2010-2016). Owing to advances in diagnostic methods, number of unclassified CM has been decreasing significantly. The cause remained unknown in only 24.4% of DCM patients, and in 18.8% of HCM patients. In the last 18 years the mortality rate of 7.4% (14/194) has been recorded, 50% (7/14) due to DCM. That is the result of teamwork, targeted medical therapy, electrotherapy (electrical stimulation – ES, cardiac resynchronisation – CRS, implantable cardioverter defibrilator – ICD) and surgical therapy (Morrow, pulmonary artery banding – PAB), including heart transplantaton since 2011 (8 patients). Conclusion: Cardiomyopathies are after congenital heart defects the most severe diseases under care of pediatric cardiologists. They require fluent teamwork of several expert groups, and mastering of numerous diagnostic and therapeutic methods. Dilated cardiomyopathies are the most common cause of death and the indication for heart transplatation in children.