Improvement in Hypertrophic Cardiomyopathy
Improvement in Hypertrophic Cardiomyopathy
A 17-year-old obese boy found to have familial apical hypertrophic cardiomyopathy on routine screening was enrolled in a weight loss program on the basis of the hypothesis that significant weight loss would improve his cardiac status. He was followed with serial dual-energy x-ray absorptiometry, electrocardiography, echocardiography, and blood pressure and pulse rate measurements. Within 1 year, he lost 49 kg, with a body mass index reduction from 43.6 to 28.1 kg/m and associated reductions in systolic blood pressure, diastolic blood pressure, pulse pressure, mean heart rate, rate pressure product, and echocardiographic indices of left ventricular mass that resulted in a change from the initial geometric finding of eccentric left ventricular hypertrophy to a "normal" left ventricular mass with minimal asymmetric apical left ventricular thickening. Significant weight loss in an obese adolescent with presumed familial apical hypertrophic cardiomyopathy was associated with striking improvement in cardiac functional indices, which could have profound implications for long-term cardiovascular risk.
Obesity has become the most prevalent chronic medical condition in developed countries. Among the known comorbidities associated with obesity are hypertensive heart disease, cor pulmonale associated with the pickwickian syndrome, coronary heart disease, and obesity-associated cardiomyopathy. These are associated with increased cardiac morbidity and mortality, which may be attributable to heart failure, myocardial infarction, sudden cardiac death, and/or malignant arrhythmia. Obesity may also exacerbate other forms of cardiomyopathy, including the familial hypertrophic cardiomyopathies. The familial hypertrophic cardiomyopathies were previously thought to be uncommon but are now known to be more prevalent, particularly in black male patients such as our patient. This cardiomyopathy has a significant association with sudden cardiac death, especially in younger patients. Some reports suggest a prevalence of up to 0.2% in young adults screened using echocardiography.
The hypertrophic variants may be obstructive or nonobstructive. (Even the so-called nonobstructive variants can have obstructive physiology in settings such as Valsalva maneuver and dehydration, and with posture changes, isometric exercise, and/or sympathomimetic medications.) Treatment modalities include
-blockade and/or calcium channel blockade, pacemaker use, surgical treatment, and other experimental methods. We present the case of a 17-year-old, obese, asymptomatic adolescent who was found, during an evaluation for inclusion in a weight loss program, to have findings consistent with familial apical hypertrophic cardiomyopathy (FAHCM). His physiologic and hemodynamic findings were most consistent with the midcavity obstructive variant because of observed apposition of the interventricular septum with the free wall and papillary muscles during systole. The significant improvement observed in left ventricular mass and other cardiac functional indices during a 1-year period in which this patient lost 49 kg highlights the importance of obesity's contribution to cardiomyopathy severity, even in familial variants, and also the central role that weight loss may play in the management of such patients.
A 17-year-old obese boy found to have familial apical hypertrophic cardiomyopathy on routine screening was enrolled in a weight loss program on the basis of the hypothesis that significant weight loss would improve his cardiac status. He was followed with serial dual-energy x-ray absorptiometry, electrocardiography, echocardiography, and blood pressure and pulse rate measurements. Within 1 year, he lost 49 kg, with a body mass index reduction from 43.6 to 28.1 kg/m and associated reductions in systolic blood pressure, diastolic blood pressure, pulse pressure, mean heart rate, rate pressure product, and echocardiographic indices of left ventricular mass that resulted in a change from the initial geometric finding of eccentric left ventricular hypertrophy to a "normal" left ventricular mass with minimal asymmetric apical left ventricular thickening. Significant weight loss in an obese adolescent with presumed familial apical hypertrophic cardiomyopathy was associated with striking improvement in cardiac functional indices, which could have profound implications for long-term cardiovascular risk.
Obesity has become the most prevalent chronic medical condition in developed countries. Among the known comorbidities associated with obesity are hypertensive heart disease, cor pulmonale associated with the pickwickian syndrome, coronary heart disease, and obesity-associated cardiomyopathy. These are associated with increased cardiac morbidity and mortality, which may be attributable to heart failure, myocardial infarction, sudden cardiac death, and/or malignant arrhythmia. Obesity may also exacerbate other forms of cardiomyopathy, including the familial hypertrophic cardiomyopathies. The familial hypertrophic cardiomyopathies were previously thought to be uncommon but are now known to be more prevalent, particularly in black male patients such as our patient. This cardiomyopathy has a significant association with sudden cardiac death, especially in younger patients. Some reports suggest a prevalence of up to 0.2% in young adults screened using echocardiography.
The hypertrophic variants may be obstructive or nonobstructive. (Even the so-called nonobstructive variants can have obstructive physiology in settings such as Valsalva maneuver and dehydration, and with posture changes, isometric exercise, and/or sympathomimetic medications.) Treatment modalities include
-blockade and/or calcium channel blockade, pacemaker use, surgical treatment, and other experimental methods. We present the case of a 17-year-old, obese, asymptomatic adolescent who was found, during an evaluation for inclusion in a weight loss program, to have findings consistent with familial apical hypertrophic cardiomyopathy (FAHCM). His physiologic and hemodynamic findings were most consistent with the midcavity obstructive variant because of observed apposition of the interventricular septum with the free wall and papillary muscles during systole. The significant improvement observed in left ventricular mass and other cardiac functional indices during a 1-year period in which this patient lost 49 kg highlights the importance of obesity's contribution to cardiomyopathy severity, even in familial variants, and also the central role that weight loss may play in the management of such patients.
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