Hypertrophic cardiomyopathy echocardiography: Difference between revisions
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==Frequency of Hypertrophy== | ==Frequency of Hypertrophy== | ||
While LVH is | While LVH is usually present, not all carriers with HCM demonstrate [[left ventricular hypertrophy]]. [[LVH]] may be time dependent and may appear late, particularly in patients with a mutation in the myosin-binding protein C gene. Patients generally present later in life and in general, have a better prognosis than beta myosin heavy chain or cardiac troponin T mutations. Up to 60% of patients at age 50 years have no evidence of [[LVH]]. LVH may appear later in life in these patients. Because of this, a normal EKG and a normal ECHO at maturity does not exclude the presence of an HCM mutation <ref>Erdmann J, Raible J, Maki-Abadi J et al. Spectrum of clinical phenotypes and gene variants in cardiac myosin-binding protein C mutation carriers with hypertrophic cardiomyopathy. J Am Coll Cardiol 2001;38:322–30.</ref>. | ||
==Diagnostic Fefatures== | ==Diagnostic Fefatures== | ||
Revision as of 23:09, 21 August 2011
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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Echocardiography is the imaging modality of choice in the diagnosis of hypertrophic cardiomyopathy. Classically there is a small left ventricular cavity with hypertrophy out of proportion to any underlying condition that would cause LVH. The hypertrophy is often asymmetric.
Frequency of Hypertrophy
While LVH is usually present, not all carriers with HCM demonstrate left ventricular hypertrophy. LVH may be time dependent and may appear late, particularly in patients with a mutation in the myosin-binding protein C gene. Patients generally present later in life and in general, have a better prognosis than beta myosin heavy chain or cardiac troponin T mutations. Up to 60% of patients at age 50 years have no evidence of LVH. LVH may appear later in life in these patients. Because of this, a normal EKG and a normal ECHO at maturity does not exclude the presence of an HCM mutation [1].
Diagnostic Fefatures
- Left ventricular hypertrophy (LVH) most often in an asymmetric distribution,
- Thickening of the left ventricular wall in the absence of cavitary dilation and the presence of hyperdynamic activity (in fact there may be systolic cavity obliteration or near obliteration)
- Absnece of any other cause of left ventricular hypertrophy such as aortic stenosis or hypertension
Left Ventricular Wall Thickness
- The traditional definition requires a wall thickness > 15 mm.
- Borderline wall thicknesses of 13-14 mm must be distinguished from extreme variants of athletes heart
- There are some genetic variants that yield a normal wall thickness [2][3][4][5][6].
- Among children < 13 years of age, LVH is often absent.
References
- ↑ Erdmann J, Raible J, Maki-Abadi J et al. Spectrum of clinical phenotypes and gene variants in cardiac myosin-binding protein C mutation carriers with hypertrophic cardiomyopathy. J Am Coll Cardiol 2001;38:322–30.
- ↑ Niimura H, Bachinski LL, Sangwatanaroj S et al. Mutations in the gene for cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy. N Engl J Med 1998;338:1248–57.
- ↑ Maron BJ, Niimura H, Casey SA et al. Development of left ventricular hypertrophy in adults in hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations. J Am Coll Cardiol 2001;38:315–21.
- ↑ Charron P, Dubourg O, Desnos M et al. Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in a genotyped adult population. Circulation 1997; 96:214–9.
- ↑ Charron P, Dubourg O, Desnos M et al. Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in genotyped children. Eur Heart J 1998;19:1377–82.
- ↑ Panza JA, Maron BJ. Relation of electrocardiographic abnormalities to evolving left ventricular hypertrophy in hypertrophic cardiomyopathy during childhood. Am J Cardiol 1989;63:1258–65.