Outcome of pediatric patients with dilated cardiomyopathy listed for transplant: a multi-institutional study

Area of Science:

• Pediatric Cardiology
• Cardiovascular Surgery
• Transplantation Medicine

Background:

• Dilated cardiomyopathy (DCM) in children leads to heart failure with variable outcomes.
• Conventional treatment for pediatric DCM offers a 5-year survival rate of 64%.
• Heart transplantation (HTx) improves survival, but outcomes post-listing require detailed description.

Purpose of the Study:

• To report survival outcomes for pediatric patients with DCM from the time of listing for HTx.
• To evaluate the impact of mechanical support as a bridge to transplant in DCM patients.
• To compare survival rates with conventional treatment and assess factors influencing outcomes.

Main Methods:

• A multi-institutional, prospective registry identified 1,098 pediatric patients (<18 years) with primary DCM listed for HTx between 1993 and 2006.
• Data collected included patient demographics, clinical status at listing, mechanical support use, and transplant details.
• Survival analysis was performed to assess waitlist and post-transplant outcomes.

Main Results:

• Waitlist mortality was 11%; 75% of patients underwent HTx within 2 years.
• Overall 10-year survival post-listing was 72%.
• Higher mortality risk was associated with arrhythmias, mechanical ventilation, and ECMO, but not VAD support. Post-HTx survival was 72% at 10 years, with increased risk linked to black race, older age, mechanical ventilation, longer ischemic time, and earlier transplant era.

Conclusions:

• Heart transplantation offers significantly enhanced survival for pediatric DCM patients compared to the disease's natural course.
• Waitlist mortality for DCM is generally low, except for critically ill patients on ECMO, mechanical ventilation, or with arrhythmias.
• Pediatric DCM patients experience favorable outcomes after HTx, establishing it as a key therapeutic intervention.