Regression of Left Ventricular Hypertrophy in a Case of Sarcomeric Hypertrophic Cardiomyopathy: An Unexpected Outcome
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Published on: September 25, 2017
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Eva Cabrera Borrego1,2, Francisco José Bermúdez-Jiménez1,2,3, Rosa Macías Ruiz1,2
1Cardiology Department, Hospital Universitario Virgen de las Nieves, Granada, Spain.
JACC. Case Reports
|July 28, 2021
Insights
Sarcomeric hypertrophic cardiomyopathy in a child showed reduced hypertrophy during adolescence. This pediatric case study found no left ventricular dysfunction or increased ventricular diameters over time.
Area of Science:
- Pediatric Cardiology
- Genetics
- Cardiovascular Medicine
Background:
- Sarcomeric hypertrophic cardiomyopathy (HCM) is a primary genetic heart muscle disease.
- HCM is characterized by abnormal thickening of the heart muscle, often leading to diastolic dysfunction.
- Early diagnosis and monitoring are crucial for managing pediatric HCM.
Purpose of the Study:
- To present a unique case of sarcomeric hypertrophic cardiomyopathy diagnosed in childhood.
- To describe the natural history of this condition with regression of hypertrophy during adolescence.
- To highlight the absence of left ventricular dysfunction and ventricular dilation in this specific case.
Main Methods:
- Case report detailing clinical presentation and diagnostic findings.
- Echocardiographic assessment over a period of adolescent development.
- Review of relevant literature on pediatric sarcomeric HCM and its progression.
Main Results:
- Diagnosis of sarcomeric HCM in a pediatric patient.
- Significant regression of myocardial hypertrophy observed during adolescence.
- Sustained normal left ventricular systolic function and unchanged ventricular dimensions throughout follow-up.
Conclusions:
- Sarcomeric HCM can exhibit regression of hypertrophy during adolescence in some pediatric cases.
- Long-term follow-up may reveal favorable remodeling without significant ventricular dysfunction.
- This case underscores the variability in the clinical course of pediatric sarcomeric HCM.
Abstract:
We present a case of sarcomeric hypertrophy cardiomyopathy diagnosed in a child who had hypertrophy degree regression during adolescence, with no left ventricular dysfunction and no increase of the ventricular diameters. (Level of Difficulty: Intermediate.).
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