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September 30, 2005Journal of Medical GeneticsOpen Access

Compound and double mutations in patients with hypertrophic cardiomyopathy: implications for genetic testing and counselling

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Why the study?

Do multiple gene mutations worsen clinical phenotype and increase sudden cardiac death risk in patients with hypertrophic cardiomyopathy compared to single mutations?

Population

80 unrelated probands with hypertrophic cardiomyopathy and their genotyped family members in an Australian…

Comparison

Presence of multiple gene mutations identified… vs Presence of a single gene mutation.

Design

Cohort

Authors

JIJodie Ingles

Discussion

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Overview

May support intensified SCD surveillance in multiple-mutation HCM; hypothesis-generating and requires prospective validation before practice change.

Structured PICO

Do multiple gene mutations worsen clinical phenotype and increase sudden cardiac death risk in patients with hypertrophic cardiomyopathy compared to single mutations?

P
Population
80 unrelated probands with hypertrophic cardiomyopathy (HCM) and their genotyped family members in an Australian cohort.
I
Intervention
Presence of multiple gene mutations (double or compound mutations) identified via genetic screening of seven HCM genes (beta-MHC, MyBP-C, cTnT, cTnI, ACTC, MYL2, and MYL3).
C
Comparator
Presence of a single gene mutation.
O
Outcome
Frequency of single and multiple gene mutations, sudden cardiac death events, and septal wall thickness.hard clinical

Multiple gene mutations in HCM families are associated with a more severe clinical phenotype, including increased risk of sudden cardiac death and greater septal wall thickness, highlighting the need for comprehensive genetic screening.

Cite This Study

Jodie Ingles (2005) studied this question.

synapsesocial.com/papers/6a802429da79b66310cab547https://doi.org/10.1136/jmg.2005.033886
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