Log in to MyACC
Hypertrophic Cardiomyopathy in Children
Study Questions:
What are the clinical characteristics and outcomes of childhood hypertrophic cardiomyopathy (HCM) in the United Kingdom?
Methods:
A retrospective, longitudinal, multicenter study was performed in 13 pediatric cardiac centers in the United Kingdom. Patients <16 years of age meeting diagnostic criteria for HCM (left ventricular wall thickness >2 standard deviations above the body surface area-corrected population mean, which could not be explained by abnormal loading conditions) were eligible for enrollment. Etiology of HCM as well as clinical outcomes were studied.
Results:
Six hundred and eighty-seven patients with HCM presented at a mean age of 5.2 years (range 0-16 years). Most patients had nonsyndrome HCM (n = 433, 63%). Additional etiologies of HCM included RASopathy (n = 126, 18.3%), Friedreich’s ataxia (n = 59, 8.6%), or inborn errors of metabolism (IEM) (n = 64, 9%). In infants, the underlying etiology of HCM was more likely to be a RASopathy (42% vs. 11.2%, p < 0.0001) or IEM (18.9% vs. 6.4%, p < 0.0001). Freedom from death or transplantation was 90.6% at 5 years (1.5 per 100 patient-years) with no era effect. The most common cause of mortality was sudden cardiac death (n = 20, 2.9%). Children diagnosed in infancy or with an IEM had a worse prognosis (5-year survival, 80.5% or 66.4%). Arrhythmic events occurred at a rate of 1.2 per 100 patient-years and were more likely in nonsyndromic patients (n = 51, 88%).
Conclusions:
The authors concluded that this national study describes a heterogeneous disease whose outcomes depend on the age of presentation and etiology.
Perspective:
This is the largest European study to assess the clinical characteristics and outcomes of infants and children with HCM. Not surprisingly, outcomes are worse in infants and in patients with inborn errors of metabolism. The increasing prevalence of syndromic HCM can likely be attributed to increased screening of patients with metabolic disease for cardiac issues. Although children in this cohort had a relatively good prognosis (death/transplant rate of 1.5 per patient-year), the risk remains higher than in adult cohorts.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, Acute Heart Failure, Heart Transplant, Interventions and Structural Heart Disease
Keywords: Arrhythmias, Cardiac, Ataxia, Body Surface Area, Cardiomyopathy, Hypertrophic, Child, Death, Sudden, Cardiac, Friedreich Ataxia, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Infant, Metabolism, Inborn Errors, Pediatrics
< Back to Listings
