학술논문
Sudden cardiac death in congenital heart disease.
Document Type
Academic Journal
Author
Khairy P; Montreal Heart Institute, Université de Montréal, 5000 Belanger St E., Montreal, QC, Canada H1T 1C8.; Silka MJ; Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.; Moore JP; Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, CA, USA.; DiNardo JA; Boston Children's Hospital, Harvard University, Boston, MA, USA.; Vehmeijer JT; Heart Center, Academic Medical Center-University of Amsterdam, Amsterdam, The Netherlands.; Sheppard MN; Cry Unit of Cardiovascular Pathology, St George's University of London, London, UK.; van de Bruaene A; Department of Cardiovascular Sciences, University of Leuven, Leuven, Belgium.; Chaix MA; Montreal Heart Institute, Université de Montréal, 5000 Belanger St E., Montreal, QC, Canada H1T 1C8.; Brida M; Department of Cardiovascular Medicine, University Hospital Centre, Zagreb, Croatia.; Moore BM; Royal Prince Alfred Hospital, University of Sydney, Camperdown, New South Wales, Australia.; Shah MJ; Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.; Mondésert B; Montreal Heart Institute, Université de Montréal, 5000 Belanger St E., Montreal, QC, Canada H1T 1C8.; Balaji S; Division of Pediatric Cardiology, Oregon Health and Science University, Portland, OR, USA.; Gatzoulis MA; Royal Brompton & Harefield Hospitals, National Heart and Lung Institute, Imperial College, London, UK.; Ladouceur M; Hôpital Européen Georges Pompidou, Université de Paris, Paris, France.
Source
Publisher: Oxford University Press Country of Publication: England NLM ID: 8006263 Publication Model: Print Cited Medium: Internet ISSN: 1522-9645 (Electronic) Linking ISSN: 0195668X NLM ISO Abbreviation: Eur Heart J Subsets: MEDLINE
Subject
Language
English
Abstract
Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.
(© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
(© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)