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February 1, 2012Acta cardiologica. Supplementum

Spectrum and clinical manifestations of mutations in genes responsible for hypertrophic cardiomyopathy

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Why the study?

What is the frequency and spectrum of mutations in MYH7, MYBPC3, TNNI3, and TNNT2 genes in Central European patients with hypertrophic cardiomyopathy?

Population

100 patients with hypertrophic cardiomyopathy (HCM) from a Central European population

Design

Cross-sectional

Key result

Complete sequencing of sarcomere genes in hypertrophic cardiomyopathy patients identified mutations in 40%, most commonly in MYBPC3 (54.5%) and MYH7 (31.8%).

Authors

KČKarol ČurilaLBLucie BenešováMPMartin Pěnička

Discussion

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Overview

Supports comprehensive sarcomere sequencing in Central European HCM; leaves open clinical utility and generalizability pending prospective validation.

Study Design

Type

Observational (n=100)

Structured PICO

What is the frequency and spectrum of mutations in MYH7, MYBPC3, TNNI3, and TNNT2 genes in Central European patients with hypertrophic cardiomyopathy?

P
Population
100 patients with hypertrophic cardiomyopathy (HCM) from a Central European population
I
Intervention
Complete sequencing of MYH7, MYBPC3, TNNI3 and TNNT2 genes
O
Outcome
Frequency and distribution of mutations in MYH7, MYBPC3, TNNI3 and TNNT2 genessurrogate

In a Central European HCM cohort, MYBPC3 was the most commonly mutated gene, and the high phenotypic heterogeneity and novel mutations suggest targeted pre-screening tests are ineffective.

Cite This Study

Čurila et al. (2012) conducted an observational in Hypertrophic cardiomyopathy (n=100). Complete sequencing of MYH7, MYBPC3, TNNI3 and TNNT2 genes was evaluated on Presence of mutations in MYH7, MYBPC3, TNNI3 and TNNT2 genes. Complete sequencing of sarcomere genes in hypertrophic cardiomyopathy patients identified mutations in 40%, most commonly in MYBPC3 (54.5%) and MYH7 (31.8%).

synapsesocial.com/papers/6a081adcef79633196e8a517https://doi.org/10.1080/ac.67.1.2146562
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