ABSTRACT Objective: To define a reference chart comparing pressure drop vs. flow generated by a set of arterial cannulae currently utilized in cardiopulmonary bypass conditions in pediatric surgery. Methods: Cannulae from two manufacturers were selected considering their design and outer and inner diameters. Cannula performance was evaluated in terms of pressure drop vs. flow during simulated cardiopulmonary bypass conditions. The experimental circuits consisted of a Jostra HL-20 roller pump, a Quadrox-i pediatric oxygenator (Maquet Cardiopulmonary AG, Rastatt, Germany), and a custom pediatric tubing set. The circuit was primed with lactated Ringer’s solution only (first condition) and with human packed red blood cells added (second condition) to achieve a hematocrit of 30%. Cannula sizes 8 to 16 Fr were inserted into the cardiopulmonary bypass circuit with a “Y” connector. The flow was adjusted in 100 ml/min increments within typical flow ranges for each cannula. Pre-cannula and post-cannula pressures were measured to calculate the pressure drop. Results: Utilizing a pressure drop limit of 100 mmHg, our results suggest a recommended flow limit of 500, 900, 1400, 2600, and 3100 mL/min for Braile arterial cannulae sizes 8, 10, 12, 14, and 16 Fr, respectively. For Medtronic DLP arterial cannulae sizes 8, 10, 12, 14, and 16 Fr, the recommended flow limit is 600, 1100, 1700, 2700, and 3300 mL/min, respectively. Conclusion: This study reinforces discrepancies in pressure drop between cannulae of the same diameter supplied by different manufacturers and the importance of independent translational research to evaluate components’ performance.

Abstract Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

Abstract Objective: This study aimed to evaluate Ebstein’s anomaly surgical correction and its early and long-term outcomes. Methods: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein’s anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. Results: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients’ mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. Conclusion: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.

Abstract Objective: Hemodilution is a concern in cardiopulmonary bypass (CPB). Using a smaller dual tubing rather than a single larger inner diameter (ID) tubing in the venous limb to decrease prime volume has been a standard practice. The purpose of this study is to evaluate these tubing options. Methods: Four different CPB circuits primed with blood (hematocrit 30%) were investigated. Two setups were used with two circuits for each one. In Setup I, a neonatal oxygenator was connected to dual 3/16" ID venous limbs (Circuit A) or to a single 1/4" ID venous limb (Circuit B); and in Setup II, a pediatric oxygenator was connected to dual 1/4" ID venous limbs (Circuit C) or a single 3/8" ID venous limb (Circuit D). Trials were conducted at arterial flow rates of 500 ml/min up to 1500 ml/min (Setup I) and up to 3000 ml/min (Setup II), at 36°C and 28°C. Results: Circuit B exhibited a higher venous flow rate than Circuit A, and Circuit D exhibited a higher venous flow rate than Circuit C, at both temperatures. Flow resistance was significantly higher in Circuits A and C than in Circuits B (P<0.001) and D (P<0.001), respectively. Conclusion: A single 1/4" venous limb is better than dual 3/16" venous limbs at all flow rates, up to 1500 ml/min. Moreover, a single 3/8" venous limb is better than dual 1/4" venous limbs, up to 3000 ml/min. Our findings strongly suggest a revision of perfusion practice to include single venous limb circuits for CPB.

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.

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.

FUNDAMENTO: Há uma paucidade de dados comparando o método percutâneo e o cirúrgico para tratamento da comunicação interatrial tipo ostium secundum. OBJETIVOS: Análise de segurança e eficácia comparando ambos os métodos de tratamento em um hospital de excelência com vínculo com o Ministério de Saúde. MÉTODOS: Estudo observacional, prospectivo, não randomizado de duas coortes de crianças e adolescentes < 14 anos tratadas por meio do cateterismo intervencionista (grupo A) ou da cirurgia cardíaca convencional (grupo B). A coleta dos dados foi prospectiva no grupo A e retrospectiva no B. RESULTADOS: De abr/2009 a out/2011 foram alocados 75 pts no grupo A e entre jan/2006 e jan/2011 foram tratados 105 pts no grupo B. A idade e o peso dos pacientes foram maiores no grupo B e o diâmetro da comunicação interatrial do tipo ostium secundum foi semelhante entre os grupos. Sucesso técnico foi observado em todos os procedimentos e não houve óbitos. Complicações (a maioria menores) foram encontradas em 68% no grupo B e em 4% do grupo A (p < 0,001). As taxas de fluxo residual não significativo ou de oclusão total do defeito foram semelhantes nos dois grupos. A mediana de internação foi de 1,2 dias após o procedimento percutâneo e 8,4 dias após a correção cirúrgica (p < 0,001). CONCLUSÃO: Ambos os tratamentos são seguros e eficazes com ótimos desfechos, porém o tratamento percutâneo apresenta menor morbidade e tempo de internação. Tais observações embasam a visão que essa forma de tratamento deve ser, hoje em dia, o método de escolha para pacientes selecionados com CIA do tipo ostium secundum.

BACKGROUND: There is a scarcity of data comparing percutaneous and surgical closure of the secundum atrial septal defect (ASD). OBJECTIVES: Assessment of safety and efficacy of both methods of treatment in a referral center affiliated with the Ministry of Health. METHODS: Observational, prospective, non-randomized study of two cohorts of children and adolescents younger than 14 years, treated by catheterization or surgery. Data was collected prospectively in the percutaneous group (A) and retrospectively in the surgical group (B). RESULTS: A total of 75 patients (pts) were enrolled in group A from April 2009 to October 2011 and 105 pts were treated in group B from January 2006 to January 2011. Age was older and weight was higher in group B and the ASD diameter was similar in both groups. Technical success was achieved in all procedures and there were no deaths. Complications (most minor) occurred in 68% of group B and 4% of A (p < 0.001). Rates of total occlusion or non-significant residual shunts were similar in both groups. Median hospitalization time was 1.2 days in group A and 8.4 days in group B (p < 0.001). CONCLUSION: Both treatment modalities are safe and effective, showing excellent outcomes. However, the percutaneous treatment has lower morbidity and shorter in-hospital stay length. These observations support the concept that percutaneous treatment of atrial septal defects should be regarded as the method of choice to manage selected patients with this condition.

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.

INTRODUCTION: Plantar hyperhidrosis is present in 50% of patients with hyperhidrosis. Thoracic sympathectomy is an important tool for the treatment of this condition, which is successful in about 60% of patients. For the remaining patients, lumbar sympathectomy is the procedure of choice. As new minimally invasive techniques have been developed, a significant demand for this type of access has led to its adaptation to the lumbar sympathectomy. The objective of this study was to evaluate the effectiveness of endoscopic retroperitoneal lumbar sympathectomy in controlling plantar hyperhidrosis and its effects on compensatory sweat. MATERIALS AND METHODS: Thirty female patients with persistent plantar hyperhidrosis after thoracic sympathectomy were enrolled. They were randomly assigned to laparoscopic retroperitoneal lumbar sympathectomy (Group A) or no surgical intervention (Group B - control) groups. Quality-of-life modifications were assessed by specific questionnaires before and after surgery. In the same manner, direct sweat measurements were also performed pre- and post-intervention by evaluating trans-epidermal water loss. Despite the lack of intervention, the control group was evaluated at similar timepoints. RESULTS: In Group A, no major complications occurred in the peri-operative period. During the immediate post-operative period, three patients (20%) experienced prolonged pain (more than ten days). Eight patients suffered from worsened compensatory sweating (53.3%). In Group A, after lumbar sympathectomy, the quality of life significantly improved (p<0.05, intra-group comparison) beyond that of the control group (p<0.05, inter-group comparison). Also, lumbar sympathectomy resulted in significantly lower values of foot sweat (pre- vs. post-operative periods, p<0.05; Group A vs. Group B, p<0.05). These patients also developed higher values of sweat measurements on specific points of their dorsal and abdominal regions after the procedure (p<0.05). CONCLUSIONS: The endoscopic retroperitoneal lumbar sympathectomy diminishes plantar sweat and improves the quality of life of women with plantar hyperhidrosis. However, about half of the patients develop increased compensatory hyperhidrosis in other areas of the body.

OBJETIVO: Com base nos estudos cineangiocardiográficos de pacientes portadores de atresia pulmonar (AP) com comunicação interventricular (CIV), identificar nos grupos propostos pela classificação de BARBERO MARCIAL, subgrupos com suprimento sangüíneo vascular pulmonar que apresentem características morfológicas semelhantes, mensurar suas artérias pulmonares centrais (AAPP), bem como as artérias colaterais sistêmico-pulmonares (ACSP), procurando estabelecer as implicações no tratamento cirúrgico. MÉTODO: Sessenta e três pacientes classificados em grupos A (15), B (40) e C (8) entre janeiro de 1990 e junho de 2001. Foram incluídos os pacientes com estudo cineangiocardiográfico completo e prévio à primeira intervenção cirúrgica, calculando-se o índice arterial pulmonar (IAAPP), índice arterial colateral sistêmico-pulmonar (IACSP) e índice arterial neopulmonar total (IANPT = IAAPP + IACSP). O tratamento cirúrgico foi considerado paliativo (TP), paliativo definitivo (TPD) e definitivo (TD). RESULTADOS:Nove subgrupos foram identificados, A (A1 e A2), B (B1, B2, B3, B4 e B5) e C (C1 e C2). No grupo A, o IAAPP dos pacientes que chegaram ao TD foi maior que o dos com TP (p=0,0092). No grupo B, o IANPT dos pacientes em TD foi maior que os em TP (p=0,0959). No grupo C, o IACSP dos pacientes em TPD foi menor que os TP e TD. No grupo A não houve mortalidade, no grupo B foi de 17,5% e no grupo C de 12,5%. CONCLUSÕES: Dentre os grupos A, B e C foi possível identificar nove subgrupos, sendo que as características morfológicas e morfométricas permitiram sugerir que o tratamento cirúrgico nos pacientes do grupo A têm maior chance de TD, nos do grupo B de TP e nos do grupo C de TPD. A mortalidade apresentou maior correlação com as características morfológicas que com as morfométricas.

OBJECTIVE: With base in the studies cineangiocardiography of pacients with pulmonary atresia (PA) with ventricular septal defect (VSD), to identify in the groups proposed by BARBERO MARCIAL, subgroups with similar morphological characteristics, to measure their central pulmonary arteries (CPA) and major aortopulmonary collateral arteries (MAPCA), thereby establishing their implications in surgical treatment. METHOD: Sixty three patients were classified in groups A (15), B (40) and C (8) between january 1990 and june 2001. Patients with complete cineangiocardiograms prior to the first surgical intervention were included in this study, being calculated the pulmonary arterial index (PAI), the major aortopulmonary collateral arterial index (MAPCAI) and the total neopulmonary arterial index (TNPAI = PAI + MAPCAI). Surgical treatment was considered palliative (PT), definitively palliative (DPT) and definitive (DT). RESULTS: Nine subgroups were identified, A (A1 and A2), B (B1, B2, B3, B4 and B5) and C (C1 and C2). In group A, the PAI of patients for DT was higher than for PT patients (p=0,0092). In group B, the TNPAI of DT patients was greater than for PT patients (p=0,0959). In group C, the MAPCAI in DPT patients was lower than in PT and DT patients. In the group A was not mortality, in the group B was of 17,5% and in the group C was of 12,5%. CONCLUSIONS:Among the groups A, B e C was possible to identifiy nine subgroups, the morphologic and morphometric characteristics allowed to suggest the surgical treatment in the patients of the group A had larger chance of TD, the group B of TP and the group C of TPD. The mortality presented larger correlation with the morphologic characteristics that with the morphometric.

OBJETIVO: Analisar as características anatômicas, o resultado das técnicas empregadas na correção cirúrgica de acordo com o número de procedimentos, assim como a mortalidade em cada grupo da classificação de Barbero-Marcial para atresia pulmonar com comunicação interventricular. CASUÍSTICA E MÉTODOS: De janeiro de 1990 a novembro de 1999, 73 pacientes que foram submetidos a estudo cineangiocardiográfico previamente à primeira intervenção cirúrgica, foram analisados. As características anatômicas das artérias pulmonares e artérias colaterais sistêmico-pulmonares, assim como as técnicas cirúrgicas que propiciaram tratamento paliativo, "paliativo definitivo" e definitivo foram estudadas. As causas de mortalidade também foram descritas. RESULTADOS: Dezenove pacientes apresentavam os segmentos pulmonares supridos por artérias pulmonares (grupo A), 45 por artérias pulmonares e artérias colaterais sistêmico-pulmonares (grupo B) e 9 somente por artérias colaterais sistêmico-pulmonares (grupo C). O grupo A apresentou maior proporção de tratamentos definitivos, o grupo B maior proporção de paliativos e o grupo C, maior proporção de "paliativos definitivos" (p< 0,0001). Não houve diferença estatística significante de mortalidade entre os grupos (p=0,860), embora a proporção de óbitos nos grupos B e C foi mais que o dobro da proporção do grupo A. CONCLUSÕES: A classificação de Barbero-Marcial permite estimar a probabilidade de um paciente ser submetido a tratamento paliativo, "paliativo definitivo" ou definitivo, assim como o risco de mortalidade em pacientes portadores de atresia pulmonar com comunicação interventricular.

OBJECTIVE: To analyze the morphological aspects, the surgical results obtained according to the number of procedures, and the mortality in each group of Barbero-Marcial´s classification of the pulmonary atresia with ventricular septal defect. MATERIAL E METHODS: From January 1990 to November 1999, 73 patients submitted to cardiac catheterization and detailed pulmonary angiographic study before the first surgical intervention were analyzed. The anatomical characteristics of the pulmonary arteries and major aorticopulmonary collaterals, as the surgical techniques of definitive, palliative and "definitive palliative" were studied. The causes of mortality were also described. RESULTS: Nineteen patients had all the pulmonary segments supplied by pulmonary arteries (group A), 45 patients had pulmonary segments supplied by pulmonary arteries and major aorticopulmonary collaterals (group B) and 9 had only major aorticopulmonary collaterals (group C). The group A has the major incidence of definitive surgical repair, the group B more palliative procedures and the group C more "definitive palliative" procedures (p< 0.0001). There is no statistically significant difference of mortality among the groups (p=0.860), although the mortality of group B and C was more than twice that of group A. CONCLUSIONS: Barbero-Marcial´s classification allows to estimate the probability of definitive, palliative or "definitive palliative" treatment, as well as the risk of mortality in patients with pulmonary atresia with ventricular septal defect.

OBJETIVO: Avaliação de uma nova abordagem cirúrgica para fechamento da comunicação interatrial. MÉTODOS: A abordagem da comunicação interatrial através da janela xifóidea foi realizada em 6 pacientes do sexo masculino e 1 do sexo feminino e a idade variou de 6 meses a 14 anos, com média de idades de 5,1 anos. Em todos, foi utilizada acirculação extracorpórea, através de canulação da artéria femoral e átrio direito e videotoracoscopia para auxiliar a visibilização da aorta e do canal arterial, assim como das estruturas correspondentes às cavidades esquerdas. RESULTADOS: Não houve complicações intra-operatórias ou pós-operatórias e, em todos os pacientes, foi possível a extubação na sala de operações. CONCLUSÃO: A janela xifóidea, sem a abertura do esterno, permitiu a correção da comunicação interatrial com bons resultados, podendo constituir-se em uma nova abordagem, minimamente invasiva.

PURPOSE: Evaluate a new access for surgical correction of atrial septal defects. METHODS: The access by xiphoid window was done in 7 cases with atria! septal defect type ostium secundum. The age ranged from 6 months to 14 years, mean-age 5.1 years old. In all cases the extracorporeal circulation was done with femoral artery and right atrial cannulation and aortic clamping. The video-assisted endoscopy was utilized to help the visualization of the aorta, ductus arteriosus and the anatomy of the left intracardiac structures. RESULTS: There was no intraoperative or postoperative complications and in our 7 cases the extubation at the operating room was possible. CONCLUSION: The xiphoid window without opening the sternum permited correction of the atrial septal defect with good results and without complications, and could be used as a new technique, less invasive, to correct these defects.

Com o objetivo de evitar complicações de reestenose da aorta na evolução tardia dos pacientes submetidos ao tratamento cirúrgico da estenose supravalvar aórtica localizada, provocada por calcificação e enrijecimento de material protético utilizado para ampliação de um ou mais seios de Valsalva, com ou sem secção transversal da aorta, desenvolvemos, de outubro de 1991 a dezembro de 1995, uma modificação técnica, que, sem utilização de enxertos artificiais, apenas com tecido sadio da aorta ascendente, permite ampliação adequada da porção inicial da aorta. Neste período, foram operados 10 pacientes, com diagnóstico clínico e hemodinâmico de estenose supravalvar aórtica localizada. As idades variaram de 11 meses a 38 anos (m = 13,2 anos), o peso variou de 7,500 kg a 56 kg (m = 29,1 kg) e a altura variou de 72 cm a 1,68 m (m = 1,5 m). Seis pacientes eram do sexo masculino. Três eram assintomáticos, 4 tinham dispnéia, 2 cansaço aos esforços, 2 palpitações, 1 parestesia de membros inferiores e 1 cianose ao choro. Seis pacientes eram portadores de síndrome de Williams. O gradiente sistólico entre a cavidade livre do ventrículo esquerdo e aorta variou de 50 mmHg a 100 mmHg (m = 73,5). Os pacientes foram operados com auxílio de circulação extracorpórea, hipotermia moderada, cardioplegia cristalóide, nos 7 primeiros casos, e cardioplegia sangüínea nos 3 últimos. A aorta ascendente foi amplamente dissecada até os vasos da base. Após a transecção total da aorta e ressecção do tecido fibrótico estenosante, realizamos incisões longitudinais do bordo da porção proximal da aorta até o fundo dos seios de Valsalva; a seguir, foram feitas incisões longitudinais na porção distai, nas regiões correspondentes aos postes comissurais, de maneira que cada prolongamento da aorta distai ampliasse um fundo de seio de Valsalva, obtendo uma aorta inicial de aspecto anatômico e dimensões normais.Atualmente, com um período pós-operatório de 3 meses a 4 anos e 5 meses, não houve óbito; todos os pacientes estão assintomáticos, evoluindo satisfatoriamente, sem gradiente entre a cavidade livre do ventrículo esquerdo e aorta ascendente, conforme ecocardiograma, Doppler, ressonância nuclear magnética e estudo hemodinâmico. Estes resultados nos permitem concluir ser esta técnica adequada para a correção cirúrgica da estenose supravalvar aórtica localizada, por não utilizar enxertos artificiais e realizar a sutura da aorta em uma linha sinusoidal, evitando, assim, reestenose.

A technical modification in the surgery of supravalvar aortic stenosis has been developed since October 1991 to December 1995, without using artificial grafts, but only healthy tissue of the ascending aorta thus permitting a suitable enlargement of the aortic root. The aim of this technique is to avoid complications of re-estenosis of aortic root in the late evolution of patientes submitted to surgical treatment of localized supravalvar aortic stenosis which was provoked by calcification and hardening of prosthetic material used for the enlargement of one or more Valsalva sinuses, with or whitout transversal section of the aorta. Ten patients underwent a surgery in this meantime, with clinical and hemodynamic diagnosis of localized supravalvar aortic stenosis. Their ages varied from 11 months to 38 years (mean = 13.2 years), the weight varied from 7.500 kg to 56 kg (mean = 29.1 kg), and the height varied from 72 cm to 1.68 m (mean = 1.5 m). Six of these patients were male. Three of them were asymptomatic; 4 had dyspnea, 2 were tired at efforts, 2 had palpitations, 1 had paresthesia in the lower limbs, and 1 cyanosis at crying; 6 of them bore Williams syndrome. The systolic gradient between free cavity of left ventricle and aorta varied from 50 to 100 mmHg (mean = 73.5).The patients were operated on with extracorporeal circulation, moderate hypothermia, crystalloid cardioplegia in the 7 first cases and bloody cardioplegia in the 3 last ones. The ascending aorta was widely dissected till the supra-aortic vessels. After the aorta total transection and the resection of the stenosis f ibrotic tissue, longitudinal incisions were performed at the edge of the aortic proximal portion till the bottom of the Valsalva sinuses. Next to it, longitudinal incisions were performed in the distal portion, in the regions corresponding to the comissural points, in such a way that each stretching of the distal aorta may widen a bottom of Valsalva sinus, in this way obtaining an aortic root with an anatomic aspect and regular sizes. Presently, no death has occurred in a post-operative period from 3 months to 4 years and 5 months. All the patients are asymptomatic, having a satisfactory evolution, checked by Doppler Echocardiogram, Magnetic Nuclear Ressonance and Hemodynamic study. These results allow us to conclude that this technique is suitable to the surgical correction of the localized supravalvar aortic stenosis, by the point of not using artificial grafts and accomplishing the aorta suture in a sinusoidal line, avoiding reestenosis.

OBJETIVO: Relatar as características do transplante cardíaco neonatal e pediátrico em crianças portadoras de cardiopatias complexas e cardiomiopatias retratarias à terapêutica convencional. O presente trabalho mostra a experiência de três anos e meio deste procedimento no Instituto do Coração HC-FMUSP. MÉTODOS: A metodologia empregada consistiu-se na técnica cirúrgica nos casos de cardiopatias congênitas, critérios de indicação para o transplante e viabilidade do doador, manuseio pós-operatório, imunosupressão, prevenção e tratamento de potenciais complicações. RESULTADOS: De novembro de 1992 a junho de 1996, 14 crianças foram transplantadas com idade de 12 dias a seis anos (média de 2,2 anos); 57% do sexo masculino; peso de 3,5 kg a 17,8 kg (média de 10,3 kg). Os doadores tinham de 21 dias a dez anos de idade(média de 4,4 anos); 80% do sexo masculino; peso variando de 3,8 a 20 kg (média de 14,3 kg). A sobrevida foi de 85,7% (2 óbitos em 14 pacientes). O tempo de seguimento foi de um mês a três anos e seis meses (média de 16 meses). As principais complicações foram hipertensão arterial sistêmica, rejeição aguda e infecção. O número de rejeições por paciente foi de 3,5 episódios e de infecção foi de 3,3 episódios. CONCLUSÃO: O transplante cardíaco consistiu-se em promissora opção terapêutica com sobrevida de 85,7% a médio prazo.

OBJECTIVE: Relate the characteristics of the heart transplantation in children with complex congenital heart diseases and severe cardiomyopathies. The present article was written to show the three year experience of this procedure at the Instituto do Coração HC-FMUSP. METHODS: The methodology used was based on surgical procedure in congenital heart disease, heart transplant indication criteria, inclusion criteria for donors, postoperative management, immunosupression and prophylaxis as well as treatment for potential complications. RESULTS: From November 1992 to June 1996, fourteen children, aged 12 days old to six years of age (mean: 2.2 years), underwent transplantation. Fifty-seven percent of recipients were male; weight ranged from 3.5 to 17.8 kg (mean: 10.3 kg). The mean age of donors was 4.4 years (a range of three weeks to ten years), 80% male, weight ranging from 3.8 to 20 kg (median 14.3 kg). The survival rate was 85.7% (two deaths in 14 patients). The follow-up was between one month to three years (average 16 months). The most important complications were systemic hypertension, acute rejection and infection. The number of rejections and infections per patient were 3.5 and 3.3 episodes respectively. CONCLUSIONS: In this experience heart transplantation has been giving a chance for children with complex congenital heart diseases and cardiomyopathies, with a survival rate of 85.7%.

O objetivo do presente estudo é apresentar um sistema de cardioplegia sangüínea para cirurgia de cardiopatia congênita. Foram analisados, prospectivamente, 71 pacientes com 10.12 kg em média, 34 eram do sexo feminino e a idade média foi de 2,1 anos. Da linha arterial, passando por um trocador de calor, aspiramos para uma seringa de 50 cc sangue a 8ºc, a qual é conectada a outra seringa de 10 cc com uma solução decimal de potássio, através de duas torneirinhas. A mistura da solução decimal (3 ml) com 47 ml de sangue a 8o C origina uma solução de sangue com 15 meq/1 de potássio. Esta solução é infundida na raiz da aorta, de acordo com o peso do paciente (10 cc/kg). Em todos obtivemos parada cardioplégica. O tempo médio de extracorpórea foi de 87,2 minutos e de pinçamento aórtico, de 60,7 minutos. Treze pacientes evoluíram para óbito, 6 por falência miocárdica, 3 por síndrome de baixo débito, 2 com arritmia, 1 com falência renal e 1 com coagulopatia. Os 58 demais pacientes receberam alta hospitalar sem complicações. Em conclusão, este método mostrou ser eficiente na preservação miocárdica e com baixa morbi-mortalidade

The purpose of this study is to present a simple Delivery System of Blood Cardioplegia for myocardial preservation during congenital cardiac operation. We prospectively analysed 71 patients (pts) with 10/12 kg of body mean weight, 34 were female and mean age was 2/1 years. From the arterial line, passing through a mini heat exchanger, blood at 8 C is aspirated to a 50 cc syringe, which is conected to a 10 cc syringe with a KCL + decimal solución using 2 stopcocks. The mixture originates a cold blood with 15 mEq/l of KCL + that is infused into the aortic root, according to the pts weight (10 cc/kg). In all pts cardiac arrest was obtained. The mean extracorporeal circulation time and crossclamping was 87.2 and 60.7 min. All pts recovered sinus rhythm with good ventricular function. Thirteen patients died, 6 with respiratory failure, 3 with low cardiac output, 2 with dysrhythmias, 1 with renal failure and 1 with hematologic disorder; the other 58 patients left the hospital with no complications. In conclusion, this method showed good results with efficient myocardial preservation and low morbi-mortality