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Heart transplantation for dilated cardiomyopathy
S S Adwani1, B F Whitehead, P G Rees
1Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, London.
Insights
Pediatric heart transplantation for dilated cardiomyopathy shows promising short-term results, with a 95% survival rate at one year. This procedure offers a viable option for children with end-stage heart failure.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Immunology
Background:
- Dilated cardiomyopathy is a significant cause of heart failure in children.
- Advanced medical management often proves insufficient for pediatric patients with end-stage heart disease.
- Heart transplantation is a potential life-saving intervention for eligible pediatric candidates.
Purpose of the Study:
- To evaluate the efficacy and outcomes of heart transplantation in pediatric patients with dilated cardiomyopathy.
- To assess the short-term survival rates and complications following heart transplantation in this cohort.
- To determine the suitability of heart transplantation as a therapeutic option for pediatric dilated cardiomyopathy.
Main Methods:
- A retrospective analysis of 23 pediatric patients (8 months to 16 years) who underwent heart transplantation between 1988 and 1994.
- Patients selected based on failure to thrive or inotrope dependence despite maximal medical therapy.
- Standard immunosuppression regimen included cyclosporine, azathioprine, and prednisolone.
Main Results:
- The mean age of patients was 7.1 years, with diverse etiologies of cardiomyopathy including idiopathic, congenital, and anthracycline-induced.
- Mean waiting time for transplantation was 22 days.
- Actuarial survival rates were 95% at one year and 87% at three years post-transplantation.
- Four patients developed coronary artery disease, with one mortality attributed to this complication 15 months post-transplant.
Conclusions:
- Heart transplantation is an acceptable short-term therapeutic option for pediatric patients with dilated cardiomyopathy.
- While effective, long-term complications such as coronary artery disease require ongoing monitoring.
- This study highlights the potential of heart transplantation to improve survival in critically ill pediatric cardiac patients.
Abstract:
Between 1988 and 1994, 23 patients underwent heart transplantation for dilated cardiomyopathy. The age of the 13 boys and 10 girls was from 8 months to 16 years (mean 7.1 years). Selection criteria included failure to thrive despite maximal antifailure treatment and/or intravenous inotrope dependence. The aetiology of cardiomyopathy was idiopathic (n = 13), congenital (n = 3), anthracycline induced (n = 4), Barth's syndrome (n = 1), and maternal systemic lupus erythematosus (n = 2). The waiting period of heart transplantation ranged from one day to 147 days (mean 22 days). Maintenance immunosuppression included cyclosporin, azathioprine, and prednisolone. Follow up after transplantation was from one month to 62 months (median 27 months) with a mean actuarial survival of 95% at one year and 87% at three years. Four patients developed coronary artery disease, one of whom died as a consequence 15 months after heart transplantation. Heart transplantation has emerged as an acceptable therapeutic option, at least in the short term, for patients with dilated cardiomyopathy.