Young children with HCM benefit from similar management strategies as older children: JACC
UK: Early-onset childhood hypertrophic cardiomyopathy (HCM) (before 12 years) bears a similar symptom burden and cardiac phenotype compared to patients presenting later in childhood (≥12 years of age), says a recent study. Also, there was no difference in the incidence of sudden cardiac death (SCD) and mortality. However, patients presenting younger than 12 years were more likely to have a myectomy but less likely to have a primary prevention ICD.
About half of childhood sarcomeric hypertrophic cardiomyopathy is presented before the age of 12 years, but there is no systematic characterization of this patient group. Considering this, Gabrielle Norrish, Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom, and colleagues aimed to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years in their study published in the Journal of the American College of Cardiology (JACC).
For this purpose, the researchers collected data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years. They were compared with 568 children diagnosed between 12 and 16 years.
The study led to the following findings:
- At baseline, 53.6% of patients had family histories of HCM, 20.9% had heart failure symptoms, and 39.2% were prescribed cardiac medications.
- The median maximal left ventricular wall thickness z-score was 8.7, and 27.2% had left ventricular outflow tract obstruction.
- Over a median follow-up period of 5.6 years, 6.6% died, 3.3% underwent cardiac transplantation, and 10.8% had life-threatening arrhythmic events.
- Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%).
- The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age.
"This study supports the notion that preadolescent patients should not be considered a distinct entity for risk stratification and that similar management strategies as used in older patients with HCM should be used," the researchers concluded.
Reference:
