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. .

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 / 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. .

Clinical research in pediatric organ transplantation

Solid organ transplantation has greatly improved survival in children with end-stage disease, becoming one of the main treatment options in this population. Nonetheless, there are significant challenges associated with validating and optimizing the effects of these interventions in clinical trials. Therefore, we reviewed the main issues related to conducting clinical transplantation research in children. We divided these challenges into three different categories: (i) challenges related to surgical techniques and anesthetic procedures, (ii) challenges related to post-transplant care and (iii) challenges specific to a particular population group and disease type. Some of the observed burdens for clinical research in this field are related to the limitations of conducting studies with a placebo or sham procedure, determining the standard of care for a control group, low prevalence of cases, ethical concerns related to use of a placebo control group and lack of generalizability from animal studies and clinical trials conducted in adult populations. To overcome some of these barriers, it is necessary to utilize alternative clinical trial designs, such as observational studies or non-inferiority trials, and to develop multicenter collaborations to increase the recruitment rate. In conclusion, the lack of robust data related to pediatric transplantation remains problematic, and further clinical trials are needed to develop more efficacious and safer treatments.

Clinical recommendations for postoperative care after heart transplantation in children: 21 years of a single-center experience

Heart transplantation is an option for children with complex congenital heart disease and cardiomyopathies. A patient's quality of life and long-term survival depend on successful management of the surgical complications and adverse side effects of immunosuppression. The purpose of this review was to summarize the practical management of postoperative care in this patient population and to make recommendations for the future.

Common challenge topics in pediatric transplantation

This special issue is dedicated to the common challenge topics in pediatric transplantation. It contains 11 chapters, ranging from clinical research in pediatric transplantation to translational research (from bench to bedside). It includes comprehensive reviews from renowned scientists, clinicians and surgeons from five countries from the International Pediatric Transplantation Association (IPTA), Harvard University, the University of Miami and the University of São Paulo Medical School. The clinical management of specific issues, such as sensitized patients and ABO blood type-incompatible transplantation, is addressed. In addition, the challenges facing this patient population and the future perspectives for clinical research are discussed.

Perfil da saúde bucal de crianças e adolescentes submetidos ao transplante cardíaco - estudo piloto / Profile of the oral health of children and adolescents undergoing heart transplantation - pilot study

O transplante cardíaco é considerado a última opção no tratamento de insuficiência cardíaca terminal, miocardiopatias e cardiopatias congênitas, esgotadas as possibilidades cirúrgicas e/ou medicamentosas. Nos últimos anos, o transplante cardíaco infantil tem demonstrado grande desenvolvimento e avanços importantes clínica e cientificamente. Os quadros de rejeição representam a principal causa de morbidade e mortalidade, no primeiro ano pós-transplante. Visando reduzir a taxa de morbidade e mortalidade, uma terapia imunossupressora é instituída. Entretanto, efeitos colaterais importantes estão relacionados à terapia imunossupressora, dentre eles efeitos extraorais e intraorais. É sabido que a má saúde bucal, associada à terapia imunossupressora, promove doenças bucais, bem como aumenta o risco às infecções secundárias. Por isso, o objetivo deste estudo constou de identificar o perfil de saúde bucal de crianças e adolescentes submetidos ao transplante cardíaco nesta instituição, encaminhados rotineiramente à Unidade de Odontologia pela Unidade de Congênitos. Vinte pacientes foram submetidos à entrevista sobre sua percepção de saúde bucal, avaliação da saúde bucal, por meio de questionários e de índices odontológicos. Este estudo evidenciou que a maioria dos pacientes avaliados (98%) realiza acompanhamento odontológico constante e a percepção destes aponta sua saúde bucal como sendo boa. Os índices odontológicos mostram baixo valor de CPO-D(0,1) e CEO-D(0,05) e baixo índice de gengivite, apesar do alto índice de placa (66%), deixando bem clara a importância da atuação do cirurgião-dentista como parte integrante da equipe multiprofissional, no tratamento, acompanhamento e manutenção dos pacientes submetidos ao transplante cardíaco.

The cariac transplantation has been considered the last treatment option for terminal heart failure, cardiomyopathies and congenital heart disease, without any drug or surgical possibilities, Lately, the infant heart transplantation has shown great development and importante clinical and scientific advances. The rejection cases represent the major cause of morbidity and mortality, during the first year post-transplant. In order to reduce the morbidity and mortality index, na immunosuppressive therapy is instituted, However, importante side effects are related to the immunosuppressive therapy, among them are extra-oral and intra-oral effects. It's known that poor oral ealth, associated with the immunosuppressive therapy promote oral disease, as well as increasing the risk to of identifying the oral health status of children and adolescentes undergoing cardiac transplantation, referred routinely to the Unit of Dentistry from the Congenital Unit. Twenty patients underwent na interview about their own oral health perception, evaluation of oral changes resulting from immunosuppressive therapy and oral health evalution, using questionnaires and dental índices. This study showed that most patients (98%) performed constatnt dental monitoring and this perception points their oral health as being good. The dental índices showed low values of DMFT (0,1) and deft (0,05) and low level of gingivitis, despite the high rate of plaque (66%), makin vey clear the importance of the dentist's roel as part of the multidisciplinar team, in treatment, monitoring and maintenance of patients undeergoing heart transplantation.

Avaliação do peptídeo natriurético tipo B no diagnóstico de rejeição após transplante cardíaco pediátrico / B-type natriuretic peptide assessment in the diagnosis of rejection after pediatric heart transplant / Evaluación del péptido natriurético tipo B en el diagnóstico de rechazo tras transplante cardiaco pediátrico

FUNDAMENTO: A rejeição constitui-se em uma das principais causas de mortalidade após o transplante cardíaco pediátrico. O peptídeo natriurético tipo B (BNP) tem sido estudado como método no diagnóstico de rejeição aguda, especialmente em pacientes adultos submetidos a transplante cardíaco. OBJETIVO:Correlacionar o nível sérico de BNP à rejeição aguda diagnosticada pela biópsia endomiocárdica em pacientes do grupo de transplante cardíaco pediátrico. MÉTODOS:Foram coletadas 50 amostras de BNP de 33 crianças em pós-operatório de transplante cardíaco e analisados dados de idade, sexo, cor, grupo sangüíneo, painel imunológico, tempo de evolução após o transplante, classe funcional, imunossupressão utilizada e número de rejeições. RESULTADOS:Foram 33 crianças com idade mediana de 10,13 anos, predomínio do sexo feminino (54%) e da cor branca (78%). No momento da dosagem de BNP o tempo médio de transplante foi 4,25 anos. A biópsia endomiocárdica diagnosticou nove rejeições em oito pacientes (27%), sendo três com grau 3 A, cinco com grau 2 e um com rejeição humoral. No momento da biópsia, a maioria dos pacientes encontrava-se assintomática. O nível sérico de BNP foi em média 77,18 pg/ml, sendo 144,22 pg/ml no grupo com rejeição e 62,46 pg/ml no grupo sem rejeição, com p = 0,02. CONCLUSÃO: Crianças assintomáticas podem apresentar rejeição aguda no pós-operatório de transplante cardíaco. O nível sérico de BNP apresentou diferença estatisticamente significante no grupo com rejeição, podendo ser método adicional no diagnóstico de rejeição cardíaca.

BACKGROUND: Rejection is one of the major causes of mortality following pediatric heart transplant. B-type natriuretic peptide (BNP) has been studied as a method for the diagnosis of acute rejection, especially in adult patients undergoing heart transplant. OBJECTIVE: To correlate serum BNP levels with acute rejection as diagnosed by endomyocardial biopsy in patients of the pediatric heart transplant group. METHODS: A total of 50 BNP samples were collected from 33 children in the postoperative period of heart transplant, and data on age, gender, skin color, blood group, immune panel, follow-up time after transplant, functional class, immunosuppressive regimen used and number of rejections were analyzed. RESULTS: Thirty three children with median age of 10.13 years were analyzed; of these, 54% were females and 78% were Caucasians. BNP levels were determined at a mean time from transplant of 4.25 years. Nine episodes of rejection were diagnosed in eight patients (27%) by means of endomyocardial biopsy; of these, three were grade 3A, five were grade 2, and one had humoral rejection. At the moment of biopsy, most patients were asymptomatic. The mean serum BNP level was 77.18 pg/ml, with 144.22 pg/ml in the group with rejection and 62.46 pg/ml in the group without rejection, with p = 0.02. CONCLUSION: Asymptomatic children can present acute rejection in the postoperative period of heart transplant. Serum BNP levels show a statistically significant difference in the group with rejection and thus can be an additional method in the diagnosis of cardiac rejection.

FUNDAMENTO: El rechazo constituye una de las principales causas de mortalidad tras el transplante cardiaco pediátrico. El péptido natriurético tipo B (BNP) ha sido estudiado como método en el diagnóstico de rechazo agudo, sobre todo en pacientes adultos sometidos a transplante cardiaco. OBJETIVO:Correlacionar el nivel sérico de BNP al rechazo agudo diagnosticado por biopsia endomiocárdica en pacientes del grupo de transplante cardiaco pediátrico. MÉTODOS:Se recolectaron 50 muestras de BNP de 33 niños en postoperatorio de transplante cardiaco, y se analizaron datos como edad, sexo, color, grupo sanguíneo, cuadro inmunológico, tiempo de evolución tras el transplante, clase funcional, inmunosupresión utilizada y número de rechazos. RESULTADOS:Se seleccionaron a 33 niños con edad promedio de 10,13 años, predominio del sexo femenino (54%) y de color blanca (78%). Al momento de la dosificación de BNP, el tiempo promedio de transplante fue de 4,25 años. La biopsia endomiocárdica diagnosticó nueve rechazos en ocho pacientes (27%), de ellos tres presentaron grado 3A, cinco grado 2 y uno rechazo humoral. Ya al momento de la biopsia, la mayoría de los pacientes se encontraba asintomática. El nivel sérico de BNP tuvo como promedio, 77,18 pg/ml; 144,22 pg/ml en el grupo con rechazo y 62,46 pg/ml en el grupo sin rechazo, con p = 0,02. CONCLUSIÓN: Niños asintomáticos pueden presentar rechazo agudo en el postoperatorio de transplante cardiaco. El nivel sérico de BNP presentó diferencia estadísticamente significante en el grupo con rechazo, lo que lo convierte en posible método adicional en el diagnóstico de rechazo cardiaco.

Registry of Hospital das Clínicas of the University of São Paulo Medical School: first official solid organ and tissue transplantation report - 2008

OBJECTIVE: The aim of this study was to report a single center experience of organ and tissue transplantation INTRODUCTION: This is the first report of organ and tissue transplantation at the Hospital das Clínicas of the University of Sao Paulo Medical School. METHODS: We collected data from each type of organ transplantation from 2002 to 2007. The data collected were patient characteristics and actuarial survival Kaplan-Meier curves at 30 days, one year, and five years RESULTS: There were a total of 3,321 transplants at our institution and the 5-year survival curve ranged from 53 percent to 88 percent. CONCLUSION: This report shows that solid organ and tissue transplants are feasible within the institution and allow us to expect that the quality of transplantation will improve in the future.

Transplante cardíaco pediátrico em vigência de choque cardiogênico refratário: análise crítica da viabilidade, aplicabilidade e resultados / Pediatric heart transplantation in refractory cardiogenic shock: a critical analysis of feasibility, applicability and results

FUNDAMENTO: Considerando crianças com miocardiopatia dilatada, na lista de espera de transplante de coração, podemos avaliar a gravidade do quadro hemodinâmico desses pacientes. Alguns apresentam choque cardiogênico e um elevado índice de mortalidade. Mesmo com suporte inotrópico e respiratório, o transplante de coração é considerado uma condição de extrema gravidade. OBJETIVO: Apresentar nossa experiência com crianças na circunstância de transplante cardíaco em vigência de choque cardiogênico refratário, procurando analisar a viabilidade, a aplicabilidade e os resultados desses transplantes. MÉTODOS: De março de 2001 a fevereiro de 2004, 22 crianças com miocardiopatia dilatada, previamente registradas na lista de transplante, apresentaram choque cardiogênico, necessitando transferência para unidade de terapia intensiva (UTI) pediátrica, intubação e suporte inotrópico. As idades variaram de 11 meses a 11 anos (média = 4,3 idade), com 55 por cento do sexo masculino; 14 poderiam ser listados como prioridade clínica e os outros 8 foram excluídos da lista de espera em razão de condição clínica desfavorável. RESULTADOS: Oito transplantes de coração foram executados, 6 crianças faleceram na fila de espera (42,9 por cento). Duas crianças faleceram (25 por cento) após o transplante; as outras 6 receberam alta hospitalar com boas condições clínicas. As duas principais complicação são rejeição, em 4 casos, e infecção, em 5 casos. Dois apresentaram complicações neurológicas, com recuperação total em um dos casos. CONCLUSÃO: Crianças com miocardiopatia e choque cardiogênico necessitam de transplante imediato; somente 57,1 por cento podiam ser transplantadas, com mortalidade de 25 por cento. Daquelas que sobreviveram ao transplante, a evolução clínica foi boa, similar às crianças transplantas em cirurgias eletivas.

BACKGROUND: In children with dilated cardiomyopathy who are on the waiting list for heart transplantation, we evaluate the seriousness of their hemodynamic conditions. Some develop cardiogenic shock, and the mortality rate is high. Even with inotropic and respiratory support, heart transplantation is considered an extremely grave circumstance. OBJECTIVE: The objective of this study is to report on our experience with children in this condition, in an attempt to analyze the viability, applicability and results of heart transplantation in these children. METHODS: From March 2001 to February 2004, 22 children with dilated cardiomyopathy who were on the waiting list for heart transplantation developed cardiogenic shock, requiring transfer to pediatric intensive care unit (ICU), intubation and inotropic support. Their ages ranged from 11 months to 11 years (mean age: 4.3 years), 55 percent were males, 14 could be listed as clinical priority, and the remaining 8 were removed from the waiting list due to their unfavorable clinical conditions. RESULTS: Eight heart transplantations were performed, and 6 children died while on the waiting list (42.9 percent). Two children died (25 percent) after transplantation and the remaining 6 were discharged from hospital in good clinical condition. The two main complications were organ rejection in 4 cases and infection in 5 cases. Two patients developed neurological complications, and one of them fully recovered. CONCLUSION: Children with cardiomyopathy and cardiogenic shock require immediate heart transplantation; only 57.1 percent could be transplanted, with an early 25 percent mortality rate. Those who survived transplantation showed good clinical progress, similar to that of children transplanted on an elective basis.