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Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy
Insights
Left ventricular hypertrophy in children with hypertrophic cardiomyopathy can develop or worsen over time. Early echocardiograms may not detect the disease in young relatives.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- The developmental trajectory of left ventricular hypertrophy (LVH) in pediatric HCM is not well understood.
- It remains unclear if LVH is congenital or develops postnatally.
Purpose of the Study:
- To investigate the development and progression of LVH in children with suspected or confirmed HCM.
- To determine if LVH is established at birth or evolves during early life.
- To assess the implications for early diagnosis in at-risk children.
Main Methods:
- Serial echocardiographic studies were performed on 39 children with a family history or morphologic evidence of HCM.
- Patients were evaluated at baseline (mean age 11 years) and during follow-up (mean age 16 years).
- Changes in left ventricular wall thickness were compared to normal growth expectations.
Main Results:
- Significant increases in LVH magnitude and extent were observed in 17 patients.
- Five patients showed evolution from a normal cardiac morphology to hypertrophy.
- The increase in left ventricular wall thickness (101% +/- 62%) significantly exceeded normal growth (13% +/- 10%).
Conclusions:
- LVH can develop or progress spontaneously in children with HCM during periods of significant body growth.
- Normal echocardiograms in childhood do not exclude the future development of HCM.
- Serial monitoring is crucial for early detection and management of pediatric HCM.
Abstract:
Whether the magnitude and distribution of left ventricular hypertrophy in patients with hypertrophic cardiomyopathy are established at birth, or whether they evolve during the first years of life, is unknown. Accordingly, we conducted serial echocardiographic studies in 39 children with a family history or morphologic evidence of hypertrophic cardiomyopathy. The patients were initially evaluated at 4 to 15 years of age (mean, 11) and most recently at 9 to 20 years (mean, 16). During a follow-up period of 2.5 to 6.8 years (mean, 4), the magnitude and extent of preexisting left ventricular hypertrophy markedly increased in 17 patients and the morphologic appearance of the heart evolved from normal to hypertrophic in 5 others. In these 22 patients the left ventricular wall thickness increased strikingly (by 6 to 23 mm, a change of 101 +/- 62 percent); these increases significantly exceeded those expected as a consequence of normal growth (13 +/- 10 percent; P less than 0.001) and were not associated with symptomatic deterioration or related to subaortic obstruction. We conclude that left ventricular hypertrophy may develop or progress spontaneously in patients with hypertrophic cardiomyopathy during childhood, when body growth is considerable. Since echocardiograms may be normal during childhood--before the morphologic features of hypertrophic cardiomyopathy develop--a single echocardiographic examination of young relatives of patients with hypertrophic cardiomyopathy may not exclude this disease.