Childhood-onset hypertrophic cardiomyopathy patients have over two-fold increased risk of heart failure (HRadj 2.39) and require advanced therapies like transplant more frequently than adults.
Does childhood-onset hypertrophic cardiomyopathy increase the risk of heart failure, ventricular arrhythmias, and death in patients with hypertrophic cardiomyopathy compared to adult-onset disease?
Childhood-onset hypertrophic cardiomyopathy is associated with a higher prevalence of sarcomeric variants and an increased risk of life-threatening ventricular arrhythmias and need for advanced heart failure therapies compared to adult-onset disease.
Absolute Event Rate: 0% vs 0%
Abstract Aims Childhood-onset hypertrophic cardiomyopathy (HCM) is far less common than adult-onset disease, thus natural history is not well characterized. We aim to describe the characteristics and outcomes of childhood-onset HCM. Methods and results We performed an observational cohort study of 7677 HCM patients from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Hypertrophic cardiomyopathy patients were stratified by age at diagnosis 1 year (infancy), 1–18 years (childhood), 18 years (adulthood) and assessed for composite endpoints reflecting heart failure (HF), life-threatening ventricular arrhythmias, atrial fibrillation (AF), and an overall composite that also included stroke and death. Stratifying by age of diagnosis, 184 (2.4%) patients were diagnosed in infancy; 1128 (14.7%) in childhood; and 6365 (82.9%) in adulthood. Childhood-onset HCM patients had an ∼2%/year event rate for the overall composite endpoint, with ventricular arrhythmias representing the most common event in the 1st decade following baseline visit, but HF and AF becoming more common by the end of the 2nd decade. Sarcomeric variants were more common in childhood-onset HCM (63%) and carried a worse prognosis than non-sarcomeric disease, including a greater than two-fold increased risk of HF HRadj 2.39 (1.36–4.20), P = 0.003 and 67% increased risk of the overall composite outcome HRadj 1.67 (1.16–2.41), P = 0.006. When compared with adult-onset HCM, childhood-onset was 36% more likely to develop life-threatening ventricular arrhythmias HRadj 1.36 (1.03–1.80) and twice as likely to require transplant or ventricular assist device HRadj 1.99 (1.23–3.23). Conclusion Patients with childhood-onset HCM are more likely to have sarcomeric disease, carry a higher risk of life-threatening ventricular arrythmias, and have greater need for advanced HF therapies. These findings provide insight into the natural history of disease and can help inform clinical risk stratification.
Marston et al. (Wed,) reported a other. Childhood-onset hypertrophic cardiomyopathy patients have over two-fold increased risk of heart failure (HRadj 2.39) and require advanced therapies like transplant more frequently than adults.
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