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Cardiac transplantation in children
J R Zuberbuhler1, F J Fricker, B P Griffith
1University of Pittsburgh School of Medicine, Pennsylvania.
Insights
Pediatric heart transplantation offers a life-changing treatment for severe cardiomyopathy, but faces challenges like congenital heart disease and donor organ shortages. Despite risks and long-term effects of immunosuppression, most children achieve good quality of life post-transplant.
Area of Science:
- Pediatric Cardiology
- Transplant Surgery
- Immunology
Background:
- Heart transplantation is an increasingly accepted treatment for children with end-stage cardiomyopathy.
- Congenital heart disease and elevated pulmonary vascular resistance significantly increase transplantation risks.
- Specialized procedures like heterotopic or heart-lung transplantation are options for specific patient groups.
Purpose of the Study:
- To review the current status and challenges of heart transplantation in pediatric patients.
- To highlight the management of immunosuppression and rejection in pediatric heart recipients.
- To discuss long-term outcomes, complications, and future considerations in pediatric heart transplantation.
Main Methods:
- Review of current clinical practices and outcomes in pediatric heart transplantation.
- Analysis of immunosuppressive regimens including cyclosporine, prednisone, azathioprine, and anti-thymocyte globulin (ATG).
- Evaluation of complications such as infection, rejection, coronary artery disease, and lymphoproliferative disease.
Main Results:
- Chronic immunosuppression is essential, with cyclosporine and prednisone as primary agents.
- Infection remains a significant postoperative problem, alongside long-term drug effects and rejection.
- Common long-term complications include hypertension and decreased renal function; coronary artery disease and lymphoproliferative disease are leading causes of death.
Conclusions:
- Pediatric heart transplantation can enable children to return to age-appropriate activities.
- Donor heart shortage is a growing concern impacting selection criteria.
- Careful management of immunosuppression and vigilant monitoring for complications are crucial for long-term success.
Abstract:
Heart transplantation is becoming an accepted treatment for children with irreversible and profoundly disabling cardiomyopathy. The risk is much higher when there is underlying congenital heart disease, and even moderately elevated pulmonary vascular resistance is a contraindication to orthotopic heart transplantation. Heterotopic or heart-lung transplantation may be considered in patients with elevated pulmonary vascular resistance. In a few centers, heart transplantation is being performed as an alternative to palliative surgical procedures in children with hypoplastic left heart syndrome. Chronic immune suppression is necessary in all patients postoperatively. Cyclosporine and prednisone are the mainstays of therapy, and azathioprine is often added to the regimen. ATG is used prophylactically in the immediate postoperative period and acute rejection episodes are treated with pulses of prednisone, ATG, or OKT3. Infection continues to be a major problem, and the chronic long-term effects of both rejection and the drugs used to treat it, especially cyclosporine, are also very important. Coronary artery disease and lymphoproliferative disease are causes of death, and hypertension and decreased renal function are present in almost all survivors. The shortage of donor hearts is becoming a progressively more important problem and may affect selection criteria in the future. On the positive side, most children can return to age-appropriate activities following transplantation and they seem to tolerate their chronic illness and its attendant repeated invasive procedures surprisingly well.