Case Report: Sustained ventricular arrhythmia in a child supported by a Berlin heart EXCOR ventricular assist device.

Mechanical circulatory support is an established therapy to support failing hearts as a bridge to transplantation. Although tolerated overall, arrhythmias may occur after ventricular assist device implantation and can complicate patient management. We report on an infant with dilated cardiomyopathy who developed ventricular tachycardia followed by recalcitrant ventricular fibrillation, refractory to comprehensive medical therapy post Berlin Heart EXCOR® (BHE) implant.

Serologic Evaluation for Severe Acute Respiratory Syndrome Coronavirus 2 in Pediatric Heart Transplantation Recipients and Patients on a Pediatric Heart Transplantation Waiting List in a Quaternary Hospital.

BACKGROUND: The Coronavirus disease 2019 (COVID-19) pandemic has been a global reality for longer than 3 years. Serologic studies have great importance for understanding the virus's behavior in populations, as it can suggest the status of the epidemic in a community. This cross-sectional study aimed to analyze the serologic profile for COVID-19 in patients before and after pediatric heart transplantation. METHODS: Serology data on IgG and IgM antibodies for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were collected in patients of the Pediatric Cardiology and Congenital Heart Diseases unit of a Brazilian hospital between January and August 2022. A total of 174 patients were recruited, including 28 on the transplantation waiting list and 146 heart transplant recipients. Information for each patient, including demographics (age, sex, state of origin), type of heart disease (congenital or acquired), and time after transplantation, was analyzed. RESULTS: Overall, 72 patients had a positive serology for anti-N antibodies (48.0%), including 62 heart transplant recipients and 10 patients on the transplantation waiting list, The positivity rates in these 2 groups were 48.1% and 47.6%, respectively. Positivity rates for previously infected individuals were 62.5% and 62.1%, respectively. CONCLUSIONS: Approximately one-half of our study sample had IgM or IgG antibodies against the SARS-CoV-2 virus. Serologic studies on the duration and level of protection provided by these antibodies are relevant public health tools for health promotion of vulnerable groups and can be useful for future studies on antibody behavior.

Case Report: The Challenge for Diagnosis of Myocarditis and Transplant Rejection After COVID Infection in a Heart-Transplanted Adolescent.

Patients who have undergone organ transplantation are immunosuppressed hosts, leaving them at a higher risk of infections. SARS-COV-2 has been shown to affect heart-transplanted patients. In this case report, we present the case of a 14-year-old heart transplant recipient who developed signs and symptoms of heart failure, along with fatigue, after a COVID-19 infection. An endomyocardial biopsy was performed to diagnose rejection and to evaluate whether this was myocarditis due to SARS-COV-2. The biopsy showed intense acute cellular rejection (3R) and antibody rejection PAMR1 H+ but was negative for the SARS-CoV-2 virus. The patient received organ rejection therapy with high-dose methylprednisolone and human immunoglobulin. After treatment, her heart function recovered, with biopsy investigations showing a lower level of cellular rejection (1R).

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.

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant.

We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation. The progression to end-stage heart disease culminated in a heart transplant at 12 years old. One year after the transplant, at the age of 13 years, he presented a progressively growing mass on the generator site of the resynchronization device. The initial decision was to remove the device. During the removal surgery, there was no haematoma or fluid collection. However, there was a progression of the lesion. The lesion was biopsied with the anatomopathological diagnosis of a DT. Resection surgery happened 4 months after the start of the mass growth. At that time, the tumour reached 20 cm in diameter. The lesion infiltrated the pectoralis major muscle and this muscle was resected partially en bloc with the lesion. The defect had primary closure. The patient evolved without postoperative complications and was discharged on the 14th postoperative day. The surgical specimen came with negative circumferential margins. However, the deep margin was microscopically positive. Due to deep involvement, the patient underwent adjuvant radiotherapy. Currently, the patient is under clinical follow-up and has no evidence of tumour recurrence. DT is a rare tumour, with unpredictable courses. Surgery can be considered in the progression of lesions. Treatment is justified by long survival after a heart transplant and in DT patients. DT is a differential diagnosis to be considered in progressive growth lesions.

Case Report: Dilated Cardiomyopathy in a Newborn, a Potential Association With SARS-COV-2.

Objective: The objective of this study was to describe the clinical course of a newborn who developed dilated cardiomyopathy (DCM) after COVID-19 infection. Methods: We retrospectively assessed the clinical notes of a pediatric patient with decompensated heart failure and who was previously positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Results: A 23-day-old newborn presented with diarrhea, hypoactivity, tachypnea, and lethargy. The infant progressed to develop respiratory failure and required orotracheal intubation due to apnea. A nasopharyngeal swab tested positive for SARS-COV-2. An echocardiogram (ECHO) demonstrated severe left ventricular dysfunction. The patient was discharged after 18 days with furosemide and angiotensin-converting enzyme inhibitors. During the follow-up period, the infant had two episodes of decompensated heart failure, with evidence of DCM. Investigations for known causes of secondary DCM were negative. The infant was promptly referred for heart transplantation. Conclusion: Although rare, we have observed a case of DCM in a newborn following COVID-19 disease. DCM may be a complication following COVID-19 disease in newborns.

A Comprehensive and Contemporary Review on Immunosuppression Therapy for Heart Transplantation.

Heart transplantation is the standard of therapy for patients with end-stage heart disease. Since the first human-to-human heart transplantation, performed in 1967, advances in organ donation, surgical techniques, organ preservation, perioperative care, immunologic risk assessment, immunosuppression agents, monitoring of graft function and surveillance of long-term complications have drastically increased recipient survival. However, there are yet many challenges in the modern era of heart transplantation in which immunosuppression may play a key role in further advances in the field. A fine-tuning of immune modulation to prevent graft rejection while avoiding side effects from over immunosuppression has been the vital goal of basic and clinical research. Individualization of drug choices and strategies, taking into account the recipient's clinical characteristics, underlying heart failure diagnosis, immunologic risk and comorbidities seem to be the ideal approaches to improve post-transplant morbidity and survival while preventing both rejection and complications of immunosuppression. The aim of the present review is to provide a practical, comprehensive overview of contemporary immunosuppression in heart transplantation. Clinical evidence for immunosuppressive drugs is reviewed and practical approaches are provided. Cardiac allograft rejection classification and up-to-date management are summarized. Expanding therapies, such as photophoresis, are outlined. Drug-to-drug interactions of immunosuppressive agents focused on cardiovascular medications are summarized. Special situations involving heart transplantation such as sarcoidosis, Chagas diseases and pediatric immunosuppression are also reviewed. The evolution of phamacogenomics to individualize immunosuppressive therapy is described. Finally, future perspectives in the field of immunosuppression in heart transplantation are highlighted.

Long-Term Evaluation of Post-transplant Lymphoproliferative Disorders in Paediatric Heart Transplantation in Sao Paulo, Brazil.

We sought to better define the demographics and characteristics of post-transplant lymphoproliferative disorders (PTLD) in a cohort of paediatric OHT patients from a developing country. Data were collected from the Heart Institute, Sao Paulo, for all paediatric OHT recipients from October 1992 to October 2018. Group differences between the PTLD and non-PTLD cohorts were assessed by Fisher exact and Mann-Whitney U tests. Kaplan-Meier curves analysed the survival in each group. Data were reviewed for 202 paediatric OHT recipients. Overall 1-, 5- and 10-year survival for the entire cohort was 76.5%, 68.3% and 62.9%; 24 patients (11.9%) developed PTLD at a median 3.1 years (IQR 0.8-9.0) after OHT. Cases were evenly spread over the follow-up period, with PTLD diagnosed in 9.8% (n = 137) of patients who were alive at 3 years, 15.3% (n = 78) of patients who were alive at 5 years and 29.3% (n = 41) of patients who were alive at 10 years. The commonest form of PTLD was diffuse large B cell lymphoma (n = 9), and most patients received rituximab with immunosuppression and chemotherapy as treatment (n = 15). We identified no increased risk in mortality amongst the PTLD vs. non-PTLD cohorts in multivariate analysis (P = 0.365). PTLD after paediatric OHT had acceptable outcomes. However, risk factors for PTLD were not identified and warrant further investigation.

Rational Use of Mechanical Circulatory Support as a Bridge to Pediatric and Congenital Heart Transplantation.

INTRODUCTION: Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. METHODS: All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. RESULTS: Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). CONCLUSION: Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

Rational use of mechanical circulatory support as a bridge to pediatric and congenital heart transplantation

Abstract Introduction: Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. Methods: All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. Results: Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). Conclusion: Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

Transplante cardíaco em crianças: visão atual e perspectivas futuras / Heart transplant in children: current view and future perspectives

O transplante cardíaco em crianças tem sido opção nas cardiopatias congênitas complexas e nas cardiomiopatias refratárias a terapêutica convencional. A investigação diagnóstica da etiologia das cardiopatias tem avançado na última década como, por exemplo, a sequência de exons. A assistência circulatória tem sido utilizada como ponte para transplante e no tratamento de rejeições agudas e crônicas. Do ponto de vista imunológico, a prova cruzada virtual no momento do transplante principalmente para os pacientes sensibilizados tem sido útil e fundamental. Novas drogas imunossupressoras como tacrolimo, sirolimo e rituximabe apresentam-se como promissoras na prevenção e tratamento da rejeição. O transplante com sitema ABO incompativel é uma possibilidade para recém-nascidos pela escassez de doadores.

Heart transplant in children has been an option in complex congenital heart disease and cardiomyopathies that do not respond to conventional treatment. The diagnostic investigatio nof the etiology of heart disease has advanced in the last decade, an example of this being exon sequencing. Circulatory support has been used as a bridge to transplantation, and in the treatment of acute and chronic rejections. From an immunological point of view,the virtual crossmatch test at the time of transplant, particularly for sensitized patients, has been useful and essential. New immunosuppressant drugs such as tacrolimus, sirolimusand rituximab have shown promise in the prevention and treatment of rejection. ABO--incompatible transplantation is a possibility for newborn infants, due to the lack of donos.

Use of Short-term Circulatory Support as a Bridge in Pediatric Heart Transplantation / Emprego do Suporte Circulatório de Curta Duração como Ponte para Transplante Cardíaco Pediátrico

Background: Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure. Objective: To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy. Methods: Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy. Results: We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and causeof death in group A while neurologic complications were more prevalent en group B. Conclusion: Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2. .

Introdução: O transplante cardíaco é o tratamento de escolha na insuficiência cardíaca terminal, porém a escassez de doadores limita o seu emprego, especialmente na população pediátrica. O implante de dispositivos de assistência circulatória mecânica (ACM) pode aumentar o tempo de espera e contribuir na redução da mortalidade. Objetivo: Estudar a evolução dos pacientes pediátricos com diagnóstico de miocardiopatia, com indicação de transplante e que evoluíram em Intermacs1 ou 2, comparando a utilização ou não de algum tipo de ACM de curta duração. Método: No período de janeiro de 2011 a dezembro de 2013, 40 pacientes com idade média de 4,5 anos foram admitidos com diagnóstico de miocardiopatia dilatada. Desses pacientes, 20 evoluíram em Intermacs 1 ou 2. Um faleceu em menos de 24 horas e 19 foram listados para transplante. Os pacientes foram divididos em dois grupos: A, pacientes manejados clinicamente em espera para o transplante; B, pacientes que utilizaram algum tipo de ACM de curta duração durante o tempo de espera em lista. Resultados: No grupo A (n=10), oito pacientes evoluíram para óbito aguardando o transplante e dois pacientes (20%) foram transplantados, sendo que nenhum recebeu alta hospitalar. No grupo B (n = 9), seis pacientes (66,7%; p = 0,04) foram transplantados e três receberam alta hospitalar (p = 0,049). As principais complicações foram insuficiência renal e sepse, no grupo A, e complicações neurológicas no grupo B. Conclusão: O emprego de ACM de curta duração aumentou a sobrevida dos pacientes priorizados e em fila de espera de transplante cardíaco pediátrico. .

Use of short-term circulatory support as a bridge in pediatric heart transplantation.

BACKGROUND: Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure. OBJECTIVE: To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy. METHODS: Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy. RESULTS: We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984 h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and cause of death in group A while neurologic complications were more prevalent en group B. CONCLUSION: Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2.

Terapia de ressincronização cardíaca em criança com cardiopatia congênita complexa candidata a transplante cardíaco / Cardiac resynchronization therapy in child with complex congenital heart disease candidate for heart transplantation

Os autores relatam o caso de criança de 8 anos de idade com discordâncias atrioventricular e ventriculoarterial (transposição corrigida das grandes artérias) e insuficiência cardíaca congestiva crônica refratária, que apresentava retardo da ativação de ventrículo morfologicamente direito em posição sistêmica. A dissincroniacardíaca foi provocada pela estimulação ventricular exclusiva para o tratamento de bloqueio atrioventricular totalconsequente a fechamento de defeito do septo interventricular e correção de regurgitação da valva atrioventricular.O implante do marcapasso atriobiventricular foi realizado por técnica híbrida, transtorácica e transvenosa, e a tática operatória foi definida com o auxílio de tomografia computadorizada do tórax. Após 4 anos de seguimento, a criança mantém melhora tanto clínica como funcional, a despeito da coexistência de alterações em bioprótese valvar e ventrículo anatomicamente direito sob regime pressórico sistêmico...

We report a case of an eight-year-old child, with congenitally corrected transposition of the great arteries and refractory congestive heart failure, who presented a delay in the activation of morphologically right ventricle in systemic position. Cardiac dyssynchrony was caused by isolated ventricular pacing for treatment of total atrioventricular block due to ventricular septal defect closure and correction of atrioventricular valve regurgitation. The atriobiventricular pacemaker was implanted using hybrid, transthoracic and transvenous access, and the surgical strategy was guided by thoracic computerized tomography. After four years of follow-up, we observed clinical and functional improvement, despite of the co-existence of bioprosthetic valve dysfunction and a morphologically right ventricle under systemic conditions...

Pediatric and congenital heart transplant: twenty-year experience in a tertiary Brazilian hospital.

INTRODUCTION: Cardiac transplantation remains the gold standard for end-stage cardiomyopathies and congenital heart defects in pediatric patients. OBJECTIVE: This study aims to report on 20 years of experience since the first case and evaluate our results. METHODS: We conducted a retrospective analysis of the database and outpatient follow-up. Between October 1992 and April 2012, 109 patients underwent 114 transplants. 51.8% of them being female. The age of patients ranged from 12 days to 21 years with a mean of 8.8±5.7 years and a median of 5.2 years. The underlying diagnosis was dilated cardiomyopathy in 61.5%, congenital heart disease in 26.6% and restrictive cardiomyopathy in 11.9%. All patients above 17 years old had congenital heart disease. RESULTS: Survival rate at 30 days, 1, 5, 10, 15, and 20 years were 90.4%, 81.3%, 70.9%, 60.5%, 44.4% and 26.7%, respectively. Mean cold ischemic time was 187.9 minutes and it did not correlate with mortality (P>0.05). Infectious complications and rejection episodes were the most common complications (P<0.0001), occurring, respectively, in 66% and 57.4% of the survivors after 10 years. There was no incidence of graft vascular disease and lymphoproliferative disease at year one, but they affected, respectively, 7.4% and 11% of patients within 10 years. CONCLUSION: Twenty-year pediatric heart transplant results at our institution were quite satisfactory and complication rates were acceptable.

Pediatric and congenital heart transplant: twenty-year experience in a tertiary Brazilian Hospital / Experiência de 20 anos com transplante cardíaco pediátrico e em portadores de cardiopatias congênitas

Introduction: Cardiac transplantation remains the gold standard for end-stage cardiomyopathies and congenital heart defects in pediatric patients. Objective: This study aims to report on 20 years of experience since the first case and evaluate our results. Methods: We conducted a retrospective analysis of the database and outpatient follow-up. Between October 1992 and April 2012, 109 patients underwent 114 transplants. 51.8% of them being female. The age of patients ranged from 12 days to 21 years with a mean of 8.8±5.7 years and a median of 5.2 years. The underlying diagnosis was dilated cardiomyopathy in 61.5%, congenital heart disease in 26.6% and restrictive cardiomyopathy in 11.9%. All patients above 17 years old had congenital heart disease. Results: Survival rate at 30 days, 1, 5, 10, 15, and 20 years were 90.4%, 81.3%, 70.9%, 60.5%, 44.4% and 26.7%, respectively. Mean cold ischemic time was 187.9 minutes and it did not correlate with mortality (P>0.05). Infectious complications and rejection episodes were the most common complications (P<0.0001), occurring, respectively, in 66% and 57.4% of the survivors after 10 years. There was no incidence of graft vascular disease and lymphoproliferative disease at year one, but they affected, respectively, 7.4% and 11% of patients within 10 years. Conclusion: Twenty-year pediatric heart transplant results at our institution were quite satisfactory and complication rates were acceptable. .

Introdução: O transplante cardíaco tem sido o tratamento de escolha para pacientes pediátricos portadores de miocardiopatias e portadores de cardiopatias congênitas em fase final da doença. Objetivo: Relatar a experiência de 20 anos do serviço e avaliar seus resultados. Métodos: Estudo retrospectivo por meio de análise do banco de dados. Entre outubro de 1992 e abril de 2012, 109 pacientes foram submetidos a 114 transplantes. 51,8% eram do sexo feminino. A idade dos pacientes variou de 12 dias a 21 anos, com média de 8,8±5,7 anos e mediana de 5,2 anos. O diagnóstico de base dos pacientes foi de miocardiopatia dilatada em 61,5%, cardiopatias congênitas em 26,6% e miocardiopatia restritiva em 11,9%. Todos os pacientes entre 17 e 21 anos eram portadores de cardiopatias congênitas. Resultados: A sobrevida em 30 dias, 1, 5, 10, 15 e 20 anos foi de 90,4%, 81,3%, 70,9%, 60,5%, 44,4 e 26,7%, respectivamente. O tempo médio de isquemia do órgão transplantado foi de 187,9 minutos e não teve correlação com a mortalidade (P>0,05). Intercorrências infecciosas e rejeição foram as complicações mais incidentes (P<0,0001), atingindo 66 e 57,4% dos sobreviventes após 10 anos. A incidência de doença vascular do enxerto e doença linfoproliferativa foi zero no primeiro ano e atingiu, respectivamente, 7,4 e 11% dos pacientes em 10 anos. Conclusão: O Transplante Cardíaco neste grupo de pacientes pediátricos e portadores de cardiopatias congênitas pôde oferecer resultados satisfatórios, com uma taxa de complicações aceitável ao longo do seguimento. .