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.
Abstract

Related Concept Videos

Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
Cardiopulmonary Resuscitation III: AED Use01:23

Cardiopulmonary Resuscitation III: AED Use

Introduction to AEDAn Automated External Defibrillator (AED) is a portable medical device that analyzes the heart's rhythm and, if necessary, delivers an electrical shock to help the heart re-establish an effective rhythm during sudden cardiac arrest (SCA). SCA occurs when the heart suddenly and unexpectedly stops beating, leading to a loss of blood flow to the brain and other vital organs. In such emergencies, time is of the essence, and using an AED, combined with Cardiopulmonary...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...