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Published on: April 10, 2019
Improved early outcome for end-stage dilated cardiomyopathy in children
Anne-Marie McMahon1, Carin van Doorn, Michael Burch
1Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom.
Insights
Recent management changes for pediatric dilated cardiomyopathy significantly reduced early mortality. Aggressive mechanical support and ABO incompatible transplants improved survival rates in children with end-stage heart failure.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Transplantation Medicine
Background:
- End-stage dilated cardiomyopathy presents significant challenges in pediatric care.
- Early outcomes for children with this condition have historically been poor.
Purpose of the Study:
- To evaluate the impact of recent management modifications on early outcomes in pediatric end-stage dilated cardiomyopathy.
- Assess the effectiveness of advanced interventions in improving survival rates.
Main Methods:
- Retrospective analysis of pediatric patients with end-stage dilated cardiomyopathy treated since 1992.
- Comparison of outcomes between two groups: pre-2000 (Group I) and post-2000 (Group II) management strategies.
- Key changes included aggressive mechanical cardiac support, prioritized transplantation listing, and ABO incompatible infant transplants.
Main Results:
- Hospital mortality significantly decreased from 37% in Group I to 11% in Group II (P < .05).
- Increased use of mechanical support (36% vs 26%) and successful ABO incompatible transplants in Group II contributed to improved survival.
- While mechanical assistance showed high morbidity, it was associated with better overall outcomes.
Conclusions:
- Recent management refinements have substantially reduced early mortality in pediatric end-stage dilated cardiomyopathy.
- Further improvements necessitate identifying early myocardial recovery markers and developing advanced, long-term mechanical support devices.
Objective:
To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children.
Methods:
We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II).
Results:
In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P <.05).
Conclusions:
Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.
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