ABSTRACT Introduction: Pediatric heart transplantation is the definitive therapy for children with end-stage heart failure. This paper describes our initial experience in pediatric heart transplantation in a tertiary center in Brazil Methods: This is a historical prospective descriptive cohort study based on a review of the medical records of children undergoing heart transplantation at Hospital de Base and Hospital da Criança e Maternidade de São José do Rio Preto. Variables were displayed as frequency, mean, or median. Statistical analysis and Kaplan-Meier actuarial curve were obtained with the aid of Microsoft® Excel® 2019 and STATSDirect version 3.3.5. Results: Between January 2010 and December 2020, ten children underwent bicaval orthotopic heart transplantation, 30% of which were under one year of age. Nine patients had end-stage heart failure (International Society for Heart and Lung Transplantation-Heart Failure D) and 50% of the recipients were transplanted under conditions of progressive clinical deterioration (Interagency Registry for Mechanically Assisted Circulatory Support ≤ 2). Forty percent of the recipients had a panel-reactive antibody > 20% on virtual crossmatch. In the postoperative period, 80% of patients required high dose of inotropic support (vasoactive-inotropic score > 10) for > 48 hours. The death-free survival rate at 131 months was 77.1±14.4%. Most patients (88.9%) in late follow-up had an episode of active cytomegalovirus infection. Cellular rejection, with or without clinical repercussion, was present in 44.4% of the patients. Conclusion: Pediatric heart transplantation produces acceptable and feasible outcomes as definitive therapy for children with end-stage heart failure.

INTRODUÇÃO: A abordagem percutânea é opção de escolha em neonatos portadores de estenose pulmonar crítica (EP) e atresia pulmonar com septo interventricular íntegro (APSI). Neste trabalho são descritos casos tratados consecutivamente em centro de referência e seu seguimento a médio prazo. MÉTODOS: A maioria dos pacientes recebeu infusão endovenosa de prostaglandina pré-procedimento, independentemente da necessidade hemodinâmica. Na abordagem da APSI, foi utilizada guia de ponta rígida e, mais recentemente, valvotomia com cateter de radiofrequência. Na maioria dos casos, foi realizada dilatação sequencial com balão até atingir 110% a 120% do diâmetro do anel valvar pulmonar. RESULTADOS: Entre 1998 e 2011, 17 neonatos com APSI (idade mediana de 5 dias, peso de 3,1 + 0,6 kg) e 30 neonatos com EP (idade mediana de 12 dias, peso médio de 3 + 1,4 kg) foram tratados em um serviço terciário. Na APSI, 14 pacientes foram abordados com guia rígida, com sucesso de 71,4%, e 3 com radiofrequência, com sucesso de 100%. Na EP, o sucesso foi alcançado em todos os casos. O óbito hospitalar foi de 23,5% no grupo com APSI e de 3,3% no grupo com EP, nenhum relacionado ao procedimento percutâneo. Na evolução a médio prazo, a taxa de reintervenção por reestenose foi de 21,4% no grupo com APSI e de 10% no grupo com EP. CONCLUSÕES: A valvoplastia pulmonar na EP e na APSI apresenta resultados clínicos e hemodinâmicos aceitáveis, desde que se observem características anatômicas favoráveis e se mantenha a patência do fluxo pulmonar até o procedimento. A morbidade e a mortalidade dos portadores de EP crítica são mais baixas que as de portadores de APSI.

BACKGROUND: The percutaneous approach is the therapy of choice in neonates with critical pulmonary stenosis (PS) and pulmonary atresia with intact interventricular septum (PAIVS). Consecutive cases treated at a reference center and their midterm follow-up was reported in this study. METHODS: Most of the patients received an intravenous infusion of prostaglandin preoperatively, regardless of the need. For the PAIVS approach, a stiff-tip guidewire was used and more recently, radiofrequency valvotomy. In most cases, sequential balloon dilation was performed until 110% to 120% of the pulmonary valve ring diameter was reached. RESULTS: Between 1998 and 2011, 17 neonates with PAIVS (median age of 5 days, mean weight of 3.1 + 0.6 kg) and 30 neonates with PS (median age of 12 days, mean weight of 3 + 1.4 kg) were treated at a tertiary center. In PAIVS patients, a stiff-tip guidewire was used in 14 cases, with a success rate of 71.4%, and radiofrequency perforation in 3 cases, with a success rate of 100%. In PS patients, success was achieved in all of the cases. Hospital death was 23.5% for the PAIVS group and 3.3% for the PS group, none related to the percutaneous procedure. In the midterm follow-up, the reintervention rate due to restenosis was 21.4% in the PAIVS group and 10% in the PS group. CONCLUSIONS: Pulmonary valvuloplasty in PS or PAIVS has acceptable clinical and hemodynamic results, as long as favorable anatomic characteristics are observed and patent pulmonary flow is maintained until the procedure. Morbidity and mortality of patients with critical PS are lower than patients with PAIVS.

INTRODUÇÃO: A introdução da cirurgia de anastomose cavopulmonar total (ACPT) ou cirurgia de Fontan modificou de forma significativa a história natural de pacientes com cardiopatias complexas não passíveis de reparo biventricular. Entretanto, é conhecido o desenvolvimento de estenoses silenciosas. Além disso, a utilização de condutos fenestrados é uma técnica comumente realizada. O presente estudo relata a experiência de um centro terciário no implante percutâneo de dispositivos em condutos de pacientes com circulação do tipo univentricular. MÉTODO: Entre julho de 2000 e julho de 2010, 12 pacientes receberam dispositivos percutâneos em condutos após cirurgia de Fontan. Os pacientes foram divididos em dois grupos, de acordo com a indicação para o procedimento: 5 pacientes receberam implante de oclusores septais para fechamento de fenestração cirúrgica (grupo 1), 6 pacientes receberam stents para alívio de obstrução de condutos (grupo 2), e 1 paciente recebeu ambos os dispositivos simultaneamente. A média de idade dos pacientes do grupo 1 no momento do procedimento era de 174 ± 53,5 meses e o peso médio era de 30,7 ± 6,8 kg. A média de idade dos pacientes do grupo 2 no momento do procedimento era de 148,5 ± 84,6 meses e o peso médio era de 28,9 ± 19,8 kg. RESULTADOS: No grupo 1, a saturação média passou de 82,6 ± 7,5% para 90,4 ± 7,5% logo após o procedimento (P = 0,001). No grupo 2, a saturação média passou de 81,8 ± 8,9% para 91,3 ± 8,7% (P = 0,01). O menor diâmetro do conduto passou de uma média de 6,9 ± 4,8 mm para 16,6 ± 3,5 mm após o procedimento (P = 0,02). O paciente que realizou ambos os procedimentos simultaneamente teve aumento do menor diâmetro do conduto de 11,7 mm para 16 mm e melhora da saturação sistêmica, passando de 60% para 90%. CONCLUSÃO: O tratamento da obstrução de condutos por meio do implante de stents não-recobertos e a oclusão de fenestrações cirúrgicas com stents recobertos ou dispositivos de AmplatzerTM são procedimentos seguros, com altos índices de sucesso imediato e que se mantêm a médio prazo.

BACKGROUND: The introduction of total cavopulmonary anastomosis (TCPA) or Fontan procedure significantly changed the natural history of patients with complex heart diseases inadequate for biventricular repair. However, the development of silent stenosis is well known. In addition, fenestrated conduits are frequently used. The present study reports the experience of a tertiary center with percutaneous devices implanted in the conduits of patients with univentricular circulation. METHOD: From July 2000 to July 2010, 12 patients received percutaneous devices in conduits after Fontan procedure. Patients were divided into two groups, according to the indication for the procedure: 5 patients received septal occluders to close surgical fenestrations (group 1), 6 patients received stents for the relief of conduit obstructions (group 2) and 1 patient received both devices simultaneously. Mean age of group 1 patients at the time of the procedure was 174 ± 53.5 months and mean weight was 30.7 ± 6.8 kg. Mean age of group 2 patients was 148.5 ± 84.6 months and mean weight was 28.9 ± 19.8 kg. RESULTS: In group 1, oxygen saturation rose from 82.6 ± 7.5% to 90.4 ± 7.5% immediately after the procedure (P = 0.001). In group 2, oxygen saturation rose from 81.8 ± 8.9% to 91.3 ± 8.7% (P = 0.01). The minimal conduit diameter changed from 6.9 + 4,8 mm to 16.6 ± 3.5 mm after the procedure (P = 0.02). The patient who was submitted to both procedures simultaneously had an increase of the minimal conduit diameter from 11.7 mm to 16 mm and an improvement in systemic saturation from 60% to 90%. CONCLUSION: Treatment of conduit obstructions with non-covered stents and the occlusion of surgical fenestrations with covered stents or AmplatzerTM devices are safe procedures, with high immediate success rates which are maintained in the mid-term follow-up.