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April 20, 2021Circulation189 citationsOpen Access

Genetic and Phenotypic Landscape of Peripartum Cardiomyopathy

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Why the study?

Do truncating variants in TTN and other cardiomyopathy genes affect clinical presentation and outcomes in women with peripartum cardiomyopathy?

Population

469 women with peripartum cardiomyopathy (PPCM) from US and international academic centers

Comparison

Next-generation sequencing of 67 genes to… vs Reference population and women without TTN…

Design

Cohort

Key result

Truncating variants in TTN were significantly more prevalent in women with peripartum cardiomyopathy compared to a reference population (10.4% vs 1.2%; OR 9.4, P=1.2x10^-46).

Authors

RGRahul GoliJLJian LiJBJeff Brandimarto

Discussion

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Overview

TTN variants may identify higher-risk peripartum cardiomyopathy; extends genetic overlap with dilated cardiomyopathy but leaves outcomes unresolved.

Study Design

Type

Observational (n=469)

Multicenter

Yes

Structured PICO

Do truncating variants in TTN and other cardiomyopathy genes affect clinical presentation and outcomes in women with peripartum cardiomyopathy?

P
Population
469 women with peripartum cardiomyopathy retrospectively identified from US and international academic centers to evaluate the prevalence of genetic variants.
E
Exposure
Next-generation sequencing of 67 genes to evaluate the burden of truncating and missense variants (including TTN, FLNC, DSP, and BAG3)
C
Comparator
Reference population (for genetic prevalence) and women without TTN truncating variants (for phenotypic comparison)
O
Outcome
Prevalence of truncating variants in TTN and other genes, and the impact of TTN truncating variants on the severity of clinical presentation and clinical outcomessurrogate

Peripartum cardiomyopathy shares significant genetic overlap with nonischemic dilated cardiomyopathy, and the presence of TTN truncating variants is associated with a lower ejection fraction at presentation.

Main Result

Odds Ratio: 9.4

Absolute Event Rate: 10.4% vs 1.2%

p-value: p=1.2x10^-46

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Cite This Study

Goli et al. (2021) conducted an observational in Peripartum cardiomyopathy (n=469). Truncating variants in TTN (TTNtvs) vs. Reference population was evaluated on Presence of TTN truncating variants (TTNtvs) (OR 9.4, p=1.2x10^-46). Truncating variants in TTN were significantly more prevalent in women with peripartum cardiomyopathy compared to a reference population (10.4% vs 1.2%; OR 9.4, P=1.2x10^-46).

synapsesocial.com/papers/6a9de8fec448d60cdc367ccbhttps://doi.org/10.1161/circulationaha.120.052395
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