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Lameh Fananapazir, MD, FRCP

JAMA. 1999;281:1746-1752.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

CASE PRESENTATION

A 28-year-old woman was diagnosed as having obstructive hypertrophic cardiomyopathy (HCM) during a family screening 13 years ago. The patient was initially treated with atenolol and verapamil for chest pain, but after several years, she complained of increasing angina and dyspnea. Physical examination revealed a prominent apical impulse and a grade 2/6 precordial systolic murmur. The 12-lead electrocardiogram showed sinus rhythm at 79/min, PR interval of 205 milliseconds, normal QRS axis, and left ventricular (LV) hypertrophy plus associated ST-T wave changes. The echocardiogram showed a septal thickness of 19 mm (normal, 11 mm); LV free wall, 15 mm (normal, <11 mm); left atrium, 52 mm (normal, <40 mm); no systolic anterior motion of the mitral valve, but apposition of LV walls at the level of papillary muscles; mild mitral regurgitation; and predicted LV gradient of 85 mm Hg at rest. A cardiac magnetic resonance imaging study . . . [Full Text of this Article]

DISCUSSION

Clinical and Genetic Heterogeneity

Clinical Correlates of Genetic Defects

Gene-Specific Cardiac Morphology.

Mutation-Specific Penetrance and Natural History.

Mutation-Specific Etiologies of Sudden Death.

Mutation-Specific Functional Abnormalities.

Mutation-Specific Skeletal Myopathy.

Cardiac Hypertrophy as a Compensatory Mechanism

Theoretical Strategies to Induce Regression of Cardiac Disease

Risk Evaluation and Mechanisms of Sudden Death

Management of Arrhythmias

Management of Obstructive HCM

CONCLUSION

Author Affiliation: Section of Clinical Electrophysiology and Inherited Cardiac Diseases, Cardiology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md.

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