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Diagnostic yield and long-term outcome of nonischemic sudden cardiac arrest survivors and their relatives: Results from a tertiary referral center

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@article{3d1773ee44b04d3694d73767c6b097e9,
title = "Diagnostic yield and long-term outcome of nonischemic sudden cardiac arrest survivors and their relatives: Results from a tertiary referral center",
abstract = "BACKGROUND: Cardiac arrest may be the first manifestation of most inherited cardiac diseases. International guidelines recommend screening of relatives of sudden cardiac arrest (SCA) survivors if an inherited cardiac disorder is suspected.OBJECTIVE: The purpose of this study was to assess the prevalence and spectrum of inherited cardiac diseases and the long-term outcome in a consecutive cohort of nonischemic SCA survivors (probands) and their relatives.METHODS: This retrospective study consecutively included probands and their relatives referred to our tertiary center for family screening between 2005 and 2018. All participants underwent a systematic workup and follow-up protocol. Data were retrieved from medical records.RESULTS: We included 155 probands (age 41.2 ± 15.5 years; 61% male) and 282 relatives (age 35.7 ± 18.8 years; 51% male). Mean follow-up was 7.1 years for probands and 4.4 years for relatives. We identified an inherited cardiac disease in 76 (49%) probands and 42 (15%) relatives. An implantable cardioverter-defibrillator was inserted in 147 (95%) probands and 9 (3%) relatives. During follow-up, 4 (3%) probands and 3 (1%) relatives died, and 37 probands and 2 relatives received appropriate shock therapy. All relatives received genetic counseling, and 18 (6%) relatives started pharmacologic treatment during follow-up.CONCLUSION: Systematic workup of nonischemic SCA survivors and their relatives identified an inherited cardiac disease in 49% of referred probands and 15% of their relatives. The favorable long-term prognosis of diagnosed relatives probably not only reflects lower age but also the effects of early diagnosis, treatment, and follow-up. These findings support systematic workup of SCA survivors and their relatives.",
keywords = "Cardiomyopathy, Channelopathy, Inherited cardiac disease, Sudden cardiac arrest, Sudden cardiac death",
author = "Jacobsen, {Elisabeth M{\"u}tze} and Hansen, {Benjamin Lautrup} and Amalie Kjerrumgaard and Jacob Tfelt-Hansen and Christian Hassager and Jesper Kjaergaard and Christensen, {Alex H{\o}rby} and Henning Bundgaard and Winkel, {Bo Gregers}",
note = "Copyright {\textcopyright} 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.",
year = "2020",
month = oct,
doi = "10.1016/j.hrthm.2020.06.030",
language = "English",
volume = "17",
pages = "1679--1686",
journal = "Heart Rhythm",
issn = "1547-5271",
publisher = "Elsevier Inc",
number = "10",

}

RIS

TY - JOUR

T1 - Diagnostic yield and long-term outcome of nonischemic sudden cardiac arrest survivors and their relatives

T2 - Results from a tertiary referral center

AU - Jacobsen, Elisabeth Mütze

AU - Hansen, Benjamin Lautrup

AU - Kjerrumgaard, Amalie

AU - Tfelt-Hansen, Jacob

AU - Hassager, Christian

AU - Kjaergaard, Jesper

AU - Christensen, Alex Hørby

AU - Bundgaard, Henning

AU - Winkel, Bo Gregers

N1 - Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

PY - 2020/10

Y1 - 2020/10

N2 - BACKGROUND: Cardiac arrest may be the first manifestation of most inherited cardiac diseases. International guidelines recommend screening of relatives of sudden cardiac arrest (SCA) survivors if an inherited cardiac disorder is suspected.OBJECTIVE: The purpose of this study was to assess the prevalence and spectrum of inherited cardiac diseases and the long-term outcome in a consecutive cohort of nonischemic SCA survivors (probands) and their relatives.METHODS: This retrospective study consecutively included probands and their relatives referred to our tertiary center for family screening between 2005 and 2018. All participants underwent a systematic workup and follow-up protocol. Data were retrieved from medical records.RESULTS: We included 155 probands (age 41.2 ± 15.5 years; 61% male) and 282 relatives (age 35.7 ± 18.8 years; 51% male). Mean follow-up was 7.1 years for probands and 4.4 years for relatives. We identified an inherited cardiac disease in 76 (49%) probands and 42 (15%) relatives. An implantable cardioverter-defibrillator was inserted in 147 (95%) probands and 9 (3%) relatives. During follow-up, 4 (3%) probands and 3 (1%) relatives died, and 37 probands and 2 relatives received appropriate shock therapy. All relatives received genetic counseling, and 18 (6%) relatives started pharmacologic treatment during follow-up.CONCLUSION: Systematic workup of nonischemic SCA survivors and their relatives identified an inherited cardiac disease in 49% of referred probands and 15% of their relatives. The favorable long-term prognosis of diagnosed relatives probably not only reflects lower age but also the effects of early diagnosis, treatment, and follow-up. These findings support systematic workup of SCA survivors and their relatives.

AB - BACKGROUND: Cardiac arrest may be the first manifestation of most inherited cardiac diseases. International guidelines recommend screening of relatives of sudden cardiac arrest (SCA) survivors if an inherited cardiac disorder is suspected.OBJECTIVE: The purpose of this study was to assess the prevalence and spectrum of inherited cardiac diseases and the long-term outcome in a consecutive cohort of nonischemic SCA survivors (probands) and their relatives.METHODS: This retrospective study consecutively included probands and their relatives referred to our tertiary center for family screening between 2005 and 2018. All participants underwent a systematic workup and follow-up protocol. Data were retrieved from medical records.RESULTS: We included 155 probands (age 41.2 ± 15.5 years; 61% male) and 282 relatives (age 35.7 ± 18.8 years; 51% male). Mean follow-up was 7.1 years for probands and 4.4 years for relatives. We identified an inherited cardiac disease in 76 (49%) probands and 42 (15%) relatives. An implantable cardioverter-defibrillator was inserted in 147 (95%) probands and 9 (3%) relatives. During follow-up, 4 (3%) probands and 3 (1%) relatives died, and 37 probands and 2 relatives received appropriate shock therapy. All relatives received genetic counseling, and 18 (6%) relatives started pharmacologic treatment during follow-up.CONCLUSION: Systematic workup of nonischemic SCA survivors and their relatives identified an inherited cardiac disease in 49% of referred probands and 15% of their relatives. The favorable long-term prognosis of diagnosed relatives probably not only reflects lower age but also the effects of early diagnosis, treatment, and follow-up. These findings support systematic workup of SCA survivors and their relatives.

KW - Cardiomyopathy

KW - Channelopathy

KW - Inherited cardiac disease

KW - Sudden cardiac arrest

KW - Sudden cardiac death

UR - http://www.scopus.com/inward/record.url?scp=85089743586&partnerID=8YFLogxK

U2 - 10.1016/j.hrthm.2020.06.030

DO - 10.1016/j.hrthm.2020.06.030

M3 - Journal article

C2 - 32615163

VL - 17

SP - 1679

EP - 1686

JO - Heart Rhythm

JF - Heart Rhythm

SN - 1547-5271

IS - 10

ER -

ID: 62032686