Article Impact Level: HIGH Data Quality: STRONG Summary of Circulation, CIRCULATIONAHA.122.062517. https://doi.org/10.1161/CIRCULATIONAHA.122.062517 Dr. Sarah Abou Alaiwi et al.
Points
- Patients diagnosed with hypertrophic cardiomyopathy (HCM) during childhood have a significantly higher risk of developing left ventricular systolic dysfunction (LVSD) than those diagnosed as adults.
- The prevalence of LVSD in childhood-diagnosed HCM patients is 14.7%, significantly higher than that of 8.7% observed in adult-diagnosed HCM patients.
- Predictors of developing incident LVSD in childhood-diagnosed HCM include age <12 years at HCM diagnosis, male sex, carrying a pathogenic sarcomere variant, previous septal reduction therapy, and lower initial left ventricular ejection fraction.
- LVSD emerges at a median age of 32.6 years in patients with childhood-diagnosed HCM, much earlier than the median age of 57.2 years in those diagnosed as adults.
- Patients with LVSD and childhood-diagnosed HCM have a poor prognosis, with 40% experiencing the composite outcome of death, cardiac transplantation, or left ventricular assist device implantation. Female participants and patients with a left ventricular ejection fraction <35% have higher composite outcome rates.
Summary
This research paper investigates the prevalence, predictors, and prognosis of left ventricular systolic dysfunction (LVSD) in patients diagnosed with hypertrophic cardiomyopathy (HCM) during childhood. The study utilized data from the international, multicenter SHaRe Registry (Sarcomeric Human Cardiomyopathy) and analyzed a cohort of 1,010 patients with childhood-diagnosed HCM, comparing them with 6,741 patients diagnosed as adults.
The results reveal that in the pediatric HCM cohort, the median age at HCM diagnosis was 12.7 years, and 36% of the patients were female. Upon the initial evaluation, 5.5% of patients with childhood-diagnosed HCM had prevalent LVSD. During a median follow-up of 5.5 years, 9.1% of the pediatric cohort developed incident LVSD. The overall prevalence of LVSD was 14.7% in childhood-diagnosed HCM patients, significantly higher than that of 8.7% observed in adult-diagnosed HCM patients.
Furthermore, the study identified several predictors associated with the development of incident LVSD in childhood-diagnosed HCM. These predictors included an age younger than 12 years at HCM diagnosis, male sex, carrying a pathogenic sarcomere variant, previous septal reduction therapy, and a lower initial left ventricular ejection fraction. Notably, patients with LVSD and childhood-diagnosed HCM had a poor prognosis, with 40% experiencing the composite outcome of death, cardiac transplantation, or left ventricular assist device implantation. Female participants and patients with a left ventricular ejection fraction below 35% had higher composite outcome rates.
Overall, this research demonstrates that patients diagnosed with HCM during childhood have a significantly higher lifetime risk of developing LVSD than those diagnosed as adults. Moreover, LVSD tends to emerge earlier in the pediatric cohort. Given the poor prognosis associated with LVSD, irrespective of age at diagnosis, it emphasizes the need for diligent monitoring of LVSD in children with HCM, particularly during the transition to adult care.
Link to the article: https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.122.062517
References Alaiwi, S. A., Roston, T. M., Marstrand, P., Claggett, B. L., Parikh, V. N., Helms, A. S., Ingles, J., Lampert, R., Lakdawala, N. K., Michels, M., Owens, A. T., Rossano, J. W., Saberi, S., Abrams, D. J., Ashley, E. A., Semsarian, C., Stendahl, J. C., Ware, J. S., Miller, E., … Ho, C. Y. (2023). Left ventricular systolic dysfunction in patients diagnosed with hypertrophic cardiomyopathy during childhood: Insights from the share registry(Sarcomeric human cardiomyopathy). Circulation, CIRCULATIONAHA.122.062517. https://doi.org/10.1161/CIRCULATIONAHA.122.062517
