Heart Transplantation in Children and Adults With Congenital Heart Disease: 3 Decades of Evolution.

Heart transplantation is the treatment of choice for children and adults with congenital heart disease. We report the heart transplant single-center experience. The number of transplantations has increased over the last 3 decades. The Kaplan-Meier survival curves in the first, second, and third decades at 5 and 10 years were 69% and 59%, 62% and 52%, and 66% and 60%, respectively.

Mechanical Circulatory Assistance in Children: Clinical Outcome.

BACKGROUND: In countries where organ donation is scarce, mortality in the pediatric heart transplant waiting list is high, and ventricular assist devices (VADs) are therapeutic alternatives in these situations. Berlin Heart EXCOR is currently 1 of the few VADs specific for children. METHODS: This retrospective study includes pediatric patients who underwent Berlin Heart EXCOR placement in a Brazilian hospital between 2012 and 2021. Clinical and laboratory data at the time of VAD implantation and the occurrence of complications and outcomes (success as a bridge to transplant or death) were analyzed. RESULTS: Eight patients, from 8 months to 15 years, were included: 6 with cardiomyopathy and 2 with congenital heart disease. Six were on Intermacs 1 and 2 on Intermacs 2. The most common complications observed were stroke and right ventricular dysfunction. Six were transplanted, and 2 died. Those submitted to transplant had a higher mean weight than those who died, with no statistically significant difference. The underlying disease had no impact on the outcome. The group undergoing transplant had lower brain natriuretic peptide and lactate values, but no laboratory variable showed a statistically significant difference in the outcome. CONCLUSION: A VAD is an invasive treatment with potentially serious adverse effects and is still poorly available in Brazil. However, as a bridge to transplant, it is a useful treatment for children in progressive clinical decline. In this study, we did not observe any clinical or laboratory factor at the time of VAD implantation that implied better outcomes.

Brain tuberculoma in pediatric heart transplant recipient.

INTRODUCTION: Heart transplantation is the standard treatment for end-stage heart disease. Despite advances in the field, patients remain under risk of developing complications, including opportunistic infections, such as tuberculosis. We present the unprecedented case of cerebral tuberculoma in a 9-year-old heart transplant recipient. CASE SCENARIO: A 9-year-old female child, who underwent heart transplantation in December 2020, was admitted to the emergency department in September 2021 due to headache and vomiting. She had normal vital signs and a mild left hemiparesis. Laboratory findings included lymphopenia and a low C Reactive Protein and brain images showed expansive lesions. A biopsy of the intracranial lesion was performed and anatomopathological analysis was compatible with tuberculoma. After the diagnosis was established, treatment protocol for neurotuberculosis was initiated, the patient had a satisfactory clinical evolution and was discharged 22 days after admission. DISCUSSION: Clinical manifestation of tuberculosis usually occurs up to 6 months after transplantation, the findings are commonly atypical and symptoms may be mild. We could not find in medical literature any description of the disease in a heart transplant recipient as young as the one presented in this case report. We documented great response to treatment, even though conventional antituberculosis therapy may interfere with immunosuppression. CONCLUSION: Patients in the postoperative period following heart transplantation are at high risk for developing opportunistic infections such as tuberculosis, which may present with atypical symptoms. Therefore the clinician must have a high index of suspicion in order to make the correct diagnosis and promptly start treatment.

Heart Retransplantation for Coronary Allograft Vasculopathy in Children: 25 Years of Single-Center Experience.

BACKGROUND: Pediatric end-stage heart disease is surgically managed by heart transplantation. A major complication of primary transplantation (PTx) is coronary allograft vasculopathy (CAV), a form of accelerated atherosclerosis. Retransplantation (RTx) has been the management of CAV; however, there is limited comprehensive literature on this subject. Here we report 25 years of single-center experience in managing CAV with RTx and place it in the context of recent studies. METHODS: A retrospective cohort study was undertaken on patients who underwent PTx <18 years old and subsequent RTx due to CAV at the Heart Institute (InCor) University of São Paulo Medical School between 1992 and 2018. The maintenance immunosuppression protocol was double immunosuppression. For both PTx and RTx, quantitative and qualitative analyses were conducted for transplantation indication, donor/recipient demographics, post-transplant survival, rejection, infection, and immunosuppression. RESULTS: Between 1992 and 2018, 200 children underwent heart transplantation. Ten re-transplantations were performed, for which 7 (70%) were for CAV. Ages at RTx ranged from 11.5 to 29.3 years (19.1 ± 5.68 years; median 18.2 years). The mean time between PTx and RTx was 12.9 ± 3.4 years (median 13.4 years). The Kaplan-Meier survival rate at 1 month, 3 years, and 5 years was 85.7%, 71.5%, and 47.6%, respectively. CONCLUSION: Cardiac RTx can be a management option for CAV in patients who have undergone PTx in childhood with double immunosuppression therapy.