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Patterns of Left Ventricular Remodelling in Children and Young Patients with Hypertrophic Cardiomyopathy
Emanuele Monda1, Martina Caiazza1, Chiara Cirillo1
1Department of Translational Medical Sciences, Inherited and Rare Cardiovascular Diseases, University of Campania "Luigi Vanvitelli", 80131 Naples, Italy.
Insights
Pediatric hypertrophic cardiomyopathy (HCM) shows varied left ventricular (LV) remodelling patterns. Maximal LV wall thickness (MLVWT) predicts adverse outcomes, with some children experiencing later progression.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Genetics
- Cardiac Imaging
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Understanding left ventricular (LV) remodelling in pediatric HCM is crucial for prognosis.
Purpose of the Study:
- To evaluate age at onset, clinical course, and LV remodelling patterns in pediatric HCM.
- To identify predictors of LV remodelling progression in young HCM patients.
Main Methods:
- Retrospective analysis of 53 pediatric patients (<18 years) with sarcomeric/non-syndromic HCM.
- Assessment of three LV remodelling patterns: thickening, thinning with preserved ejection fraction, and thinning with reduced ejection fraction (hypokinetic end-stage).
Main Results:
- 60% of patients exhibited LV remodelling; 24% progressed to hypokinetic end-stage HCM.
- Maximal LV wall thickness (MLVWT) predicted hypokinetic end-stage remodelling (OR 1.17 per 1 mm increase, p=0.043).
- Two patients showed regression then later progression of MLVWT during adolescence.
Conclusions:
- Diverse LV remodelling patterns exist in pediatric sarcomeric/non-syndromic HCM.
- MLVWT is a key predictor of adverse remodelling in this population.
- Further research into remodelling mechanisms may guide targeted therapies for preclinical HCM.
Abstract:
Introduction: The aim of this study was to evaluate the age at onset, clinical course, and patterns of left ventricular (LV) remodelling during follow-up in children and young patients with hypertrophic cardiomyopathy (HCM). Methods: We included consecutive patients with sarcomeric or non-syndromic HCM below 18 years old. Three pre-specified patterns of LV remodelling were assessed: maximal LV wall thickness (MLVWT) thickening; MLVWT thinning with preserved LV ejection fraction; and MLVWT thinning with progressive reduction in LV ejection fraction (hypokinetic end-stage evolution). Results: Fifty-three patients with sarcomeric/non-syndromic HCM (mean age 9.4 ± 5.5 years, 68% male) fulfilled the inclusion criteria. In total, 32 patients (60%) showed LV remodelling: 3 patients (6%) exhibited MLVWT thinning; 16 patients (30%) showed MLVWT thickening; and 13 patients (24%) progressed to hypokinetic end-stage HCM. Twenty-one patients (40%) had no LV remodelling during follow-up. In multivariate analysis, MLVWT was a predictor of the hypokinetic end-stage remodelling pattern during follow-up (OR 1.17 [95%CI 1.01-1.36] per 1 mm increase, p-value 0.043), regardless of sarcomeric variants and New York Heart Association class. Two patients with sarcomeric HCM, showing a pattern of MLVWT regression during childhood, experienced progression during adolescence. Conclusions: Different patterns of LV remodelling were observed in a cohort of children with sarcomeric/non-syndromic HCM. Interestingly, a pattern of progressive MLVWT thinning during childhood, with new progression of MLVWT during adolescence, was noted. A better understanding of the remodelling mechanisms in children with sarcomeric HCM may be relevant to defining the timing and possible efficacy of new targeted therapies in the preclinical stage of the disease.
