Related Experiment Videos
Cardiomyopathy and heart transplantation in children
1Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, USA. morrowwilliamr@exchange.uams.edu
Insights
Dilated cardiomyopathy in children requires accurate diagnosis and aggressive treatment for survival. Advances in mechanical support and heart transplantation offer improved outcomes, but organ donor shortages persist.
Area of Science:
- Pediatric Cardiology
- Genetics
- Transplantation Medicine
Background:
- Dilated cardiomyopathy is a leading cause of death in pediatric heart disease.
- Familial inheritance is increasingly recognized in pediatric dilated cardiomyopathy.
- Associations with metabolic, dysmorphic, and neuromuscular diseases are critical for pediatric cases.
Purpose of the Study:
- To highlight the importance of accurate diagnosis and aggressive therapy in pediatric dilated cardiomyopathy.
- To discuss the role of mechanical support and heart transplantation in managing pediatric dilated cardiomyopathy.
- To address the challenges of organ donor availability for pediatric heart transplantation.
Main Methods:
- Review of current literature on pediatric dilated cardiomyopathy.
- Analysis of treatment strategies including conventional heart failure therapy and mechanical support.
- Evaluation of outcomes for heart transplantation in pediatric patients.
Main Results:
- Survival depends on accurate diagnosis and aggressive therapy.
- Extracorporeal membrane oxygenation serves as effective mechanical support and a bridge to transplantation.
- Waiting list mortality is approximately 20%, with a 70% intermediate survival rate post-transplantation.
Conclusions:
- Accurate diagnosis and aggressive management are crucial for survival in pediatric dilated cardiomyopathy.
- Mechanical support and heart transplantation improve outcomes but are limited by organ availability.
- Increased organ donation or innovative strategies like xenotransplantation are needed to reduce waiting list mortality.
Abstract:
Cardiomyopathy is one of the most common causes of death in children with heart disease. Increasingly, dilated cardiomyopathy is recognized to be familial, and specific gene products related to the myocyte cytoskeleton and contractile proteins have been identified. Other associations with metabolic disease, dysmorphic syndromes, and neuromuscular disease are important to establish, particularly in pediatric patients, to guide therapy and patient selection for transplantation. Survival in children with dilated cardiomyopathy depends on accurate diagnosis and aggressive therapy. Patients may respond to conventional treatment for heart failure or may deteriorate, requiring mechanical support. Extracorporeal membrane oxygenation has been used effectively for mechanical support in children until improvement occurs or as a bridge to transplantation. For those who are listed, the mortality rate while waiting for a donor organ averages approximately 20%. Survival after transplantation is good, with an intermediate survival rate of approximately 70%. Late survival remains to be determined in the current cyclosporin era but may in fact be improving. However, increased organ donation or strategies to increase the size of the organ donor pool, such as xenotransplantation, are needed to significantly reduce the rate of mortality while waiting.