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November 1, 2005CirculationOpen Access

Gene Mutations in Apical Hypertrophic Cardiomyopathy

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Population

15 probands with apical hypertrophy (nonobstructive hypertrophy localized to the cardiac apex)

Design

Case_series

Key result

Sarcomere gene mutations were identified in 7 of 15 probands with apical hypertrophic cardiomyopathy, with the cardiac actin Glu101Lys mutation consistently producing apical morphology.

Authors

MAMichael AradMPManual Penas-LadoLMLorenzo Monserrat

Discussion

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Overview

Apical HCM genetic testing may refine family counseling; leaves open distinct variant-phenotype drivers versus typical HCM.

Study Design

Type

Observational (n=15)

Structured PICO

P
Population
15 probands with apical hypertrophy evaluated for genetic mutations in 9 sarcomere protein genes and 3 other genes implicated in idiopathic cardiac hypertrophy.
E
Exposure
DNA sequence analyses of 9 sarcomere protein genes and 3 other genes (GLA, PRKAG2, and LAMP2)
O
Outcome
Identification of genetic mutations causing apical hypertrophic cardiomyopathysurrogate

Only a limited number of sarcomere gene defects, such as the cardiac actin Glu101Lys mutation, consistently produce the apical variant of hypertrophic cardiomyopathy.

Cite This Study

Arad et al. (2005) conducted an observational in Apical hypertrophic cardiomyopathy (n=15). Sarcomere protein gene mutations was evaluated on Identification of genetic mutations. Sarcomere gene mutations were identified in 7 of 15 probands with apical hypertrophic cardiomyopathy, with the cardiac actin Glu101Lys mutation consistently producing apical morphology.

synapsesocial.com/papers/6a6310314f5ef41b946a996ehttps://doi.org/10.1161/circulationaha.105.547448
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