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Published on: July 18, 2014
Sudden death prior to pediatric heart transplantation: would implantable defibrillators improve outcome?
Edward K Rhee1, Charles E Canter, Susan Basile
1Division of Pediatric Cardiology, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, Missouri 63110, USA. rhee_e@kids.wustl.edu
Insights
Sudden cardiac death is uncommon in children awaiting heart transplants. However, those with ischemic cardiomyopathy face a higher risk, suggesting potential benefit from implantable cardioverter-defibrillators (ICDs).
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Transplantation Medicine
Background:
- Implantable cardioverter-defibrillator (ICD) therapy has demonstrated survival benefits in adults with cardiomyopathy.
- The incidence of sudden death in pediatric cardiomyopathy patients is not well-established.
- Understanding sudden death risk is crucial for evaluating ICD efficacy in children.
Purpose of the Study:
- To determine the incidence of sudden cardiac death (SCD) in children with cardiomyopathy before heart transplantation.
- To identify risk factors associated with SCD in this pediatric population.
- To inform decisions regarding primary prevention strategies for SCD in pediatric heart transplant candidates.
Main Methods:
- Retrospective analysis of the Pediatric Heart Transplant Study (PHTS) database.
- Inclusion of patients aged 18 years or younger who died after listing for heart transplantation but before the procedure.
- Focus on patients experiencing sudden or arrhythmic death, with examination of clinical and demographic variables.
Main Results:
- Out of 2,392 patients, 420 (17.6%) died pre-transplant; only 32 (1.3%) deaths were sudden or arrhythmic.
- Patients with ischemic cardiomyopathy exhibited a significantly increased risk of sudden death (relative risk = 6.92).
- Congenital heart disease and UNOS status at listing were not linked to increased sudden death risk.
Conclusions:
- The overall incidence of sudden death in children awaiting heart transplantation is low.
- Widespread ICD implantation for primary prevention is unlikely to improve survival in the general pediatric population.
- Children with ischemic cardiomyopathy represent a subgroup with higher sudden death risk who may benefit from ICD therapy.
Objectives:
The goal of this study is to determine the incidence of sudden cardiac death in children with cardiomyopathy prior to pediatric heart transplantation.
Background:
Recent primary prevention trials of implantable cardiac defibrillator (ICD) therapy in adults with ischemic and non-ischemic cardiomyopathy have shown a survival benefit. The incidence of sudden death, and thus the likelihood of improved survival with ICD therapy, in children with cardiomyopathy is currently unknown.
Methods:
The Pediatric Heart Transplant Study (PHTS) database was retrospectively queried for patients < or =18 years of age who died from any cause after listing for but prior to heart transplantation. Patients having arrhythmic or sudden death were included for study. Clinical and demographic variables were examined to identify higher-risk sub-groups.
Results:
Of the 2,392 patients in the PHTS database, 420 (17.6%) died prior to heart transplantation. Only 32 deaths (1.3% of total listed, 7.6% of total deaths) were sudden or arrhythmic in nature. Patients with ischemic cardiomyopathy had an increased risk of sudden death (relative risk = 6.92). Presence of congenital heart disease and United Network for Organ Sharing (UNOS) status at listing were not associated with an increased risk of sudden death.
Conclusions:
The overall incidence of sudden death in children awaiting heart transplantation is low; therefore, uniform implantation of ICDs for the primary prevention of sudden death is unlikely to improve survival in this population. Children with ischemic cardiomyopathy appear to have a higher risk of sudden death and may benefit from ICD therapy.
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