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Outcomes of Childhood-Onset Hypertrophic Cardiomyopathy
Quick Takes
- Patients with childhood-onset hypertrophic cardiomyopathy are more likely to have sarcomeric disease, carry a higher risk of life-threatening ventricular arrhythmias, and have a greater need for advanced heart failure therapies.
- Patients with adult-onset hypertrophic cardiomyopathy have a higher risk of death, NYHA class III or IV, atrial fibrillation, and stroke, as well as the composite endpoint.
Study Questions:
What are the characteristics and outcomes for patients with childhood-onset hypertrophic cardiomyopathy (HCM)?
Methods:
An observational cohort study was performed using the Sarcomeric Human Cardiomyopathy Registry (SHaRe). A total of 7,677 HCM patients were studied and stratified by age at diagnosis (<1 year [infancy], 1-18 years [childhood], >18 years [adulthood]). Endpoints included heart failure (HF), life-threatening ventricular arrhythmias, atrial fibrillation (AF), and an overall composite that also included stroke and death.
Results:
Stratifying by age at diagnosis, 184 (2.4%) patients were diagnosed in infancy, 1,128 (14.7%) in childhood, and 6,365 (82.9%) in adulthood. Childhood-onset HCM patients had an approximately 2% per year event rate for the composite endpoint. Sarcomeric variants were more common in childhood-onset HCM (63%) and carried a worse prognosis than nonsarcomeric disease with a greater risk of HF and the composite outcome. When compared with adult-onset HCM, childhood-onset was 36% more likely to develop life-threatening ventricular arrhythmias and twice as likely to require transplant or ventricular assist device.
Conclusions:
The authors concluded that patients with childhood-onset HCM are more likely to have sarcomeric disease, carry a higher risk of life-threatening arrhythmias, and have a greater need for advanced HF therapies.
Perspective:
This study investigated the differences in outcomes based on age of onset of HCM. Adult-onset HCM was associated with higher risk for the HF composite endpoint, death, New York Heart Association (NYHA) class III or IV symptoms, AF, and stroke. Childhood-onset HCM was associated with greater risk of ventricular arrhythmias and need for transplant/ventricular assist device. The genotype was different in patients with childhood-onset, with a greater proportion of patients with a sarcomeric variant. The study adds to our knowledge of the natural history of HCM and may assist with clinical risk stratification.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Prevention, 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 Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Mechanical Circulatory Support
Keywords: Age of Onset, Arrhythmias, Cardiac, Atrial Fibrillation, Cardiomyopathies, Cardiomyopathy, Hypertrophic, Genotype, Heart Defects, Congenital, Heart Failure, Heart-Assist Devices, Pediatrics, Risk Assessment, Sarcomeres, Secondary Prevention, Stroke
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