The Natural History of a Man With Ovotesticular 46,XX DSD Caused by a Novel 3-Mb 15q26.2 Deletion Containing NR2F2 Gene.

Gonadal sex determination is a complex genetic process by which an embryonic primordium is driven to form an ovary or a testis, which requires a delicate dosage balance involving many genes. Disruption in this molecular pathway can lead to differences of sex development (DSD). Although some genetic mechanisms leading to 46,XY DSD have been elucidated, little is known about copy-number variation (CNV) causing testicular or ovotesticular 46,XX DSD. We describe a 20-year natural history of a man with SRY-negative 46,XX who was born with atypical male external genitalia, aortic coarctation, and bilateral blepharophimosis-ptosis. The molecular study identified a de novo heterozygous 3-Mb 15q26.2 deletion, a gene-poor locus containing NR2F2, which encodes the nuclear receptor COUP-TFII that is highly expressed in ovary and cardiac arteries. Immunohistochemistry confirmed the low COUP-TFII expression on his ovotestis tissue. Monosomy of 15q26.2, encompassing the NR2F2 gene, may act as a Z-factor regulating the male sex determination negatively. This finding supports a novel type of CNV resulting in DSD in an individual who developed male puberty spontaneously.

Cardiovascular Surgery Residency Program: Training Coronary Anastomosis Using the Arroyo Simulator and UNIFESP Models

ABSTRACT OBJECTIVE: Engage the UNIFESP Cardiovascular Surgery residents in coronary anastomosis, assess their skills and certify results, using the Arroyo Anastomosis Simulator and UNIFESP surgical models. METHODS: First to 6th year residents attended a weekly program of technical training in coronary anastomosis, using 4 simulation models: 1. Arroyo simulator; 2. Dummy with a plastic heart; 3. Dummy with a bovine heart; and 4. Dummy with a beating pig heart. The assessment test was comprised of 10 items, using a scale from 1 to 5 points in each of them, creating a global score of 50 points maximum. RESULTS: The technical performance of the candidate showed improvement in all items, especially manual skill and technical progress, critical sense of the work performed, confidence in the procedure and reduction of the time needed to perform the anastomosis after 12 weeks practice. In response to the multiplicity of factors that currently influence the cardiovascular surgeon training, there have been combined efforts to reform the practices of surgical medical training. CONCLUSION: 1 - The four models of simulators offer a considerable contribution to the field of cardiovascular surgery, improving the skill and dexterity of the surgeon in training. 2 - Residents have shown interest in training and cooperate in the development of innovative procedures for surgical medical training in the art.

Cardiovascular Surgery Residency Program: Training Coronary Anastomosis Using the Arroyo Simulator and UNIFESP Models.

OBJECTIVE: Engage the UNIFESP Cardiovascular Surgery residents in coronary anastomosis, assess their skills and certify results, using the Arroyo Anastomosis Simulator and UNIFESP surgical models. METHODS: First to 6th year residents attended a weekly program of technical training in coronary anastomosis, using 4 simulation models: 1. Arroyo simulator; 2. Dummy with a plastic heart; 3. Dummy with a bovine heart; and 4. Dummy with a beating pig heart. The assessment test was comprised of 10 items, using a scale from 1 to 5 points in each of them, creating a global score of 50 points maximum. RESULTS: The technical performance of the candidate showed improvement in all items, especially manual skill and technical progress, critical sense of the work performed, confidence in the procedure and reduction of the time needed to perform the anastomosis after 12 weeks practice. In response to the multiplicity of factors that currently influence the cardiovascular surgeon training, there have been combined efforts to reform the practices of surgical medical training. CONCLUSION: 1 - The four models of simulators offer a considerable contribution to the field of cardiovascular surgery, improving the skill and dexterity of the surgeon in training. 2 - Residents have shown interest in training and cooperate in the development of innovative procedures for surgical medical training in the art.

Use of right ventricular remodeling surgery with a porcine pulmonary prosthesis for congenital heart disease.

BACKGROUND: The reconstruction of the right ventricular outflow tract (RVOT) in congenital heart disease has attracted the interest of cardiac surgeons determined to alleviate the anatomic obstruction and restore RV function. METHODS: From June 1991 to September 2008, 203 consecutive patients (mean, 3.0 years; range, 2 months to 35 years) underwent operations. These patients were classified into 5 groups: group 1, tetralogy of Fallot with pulmonary hypoplasia (144 cases, 70.9%); group 2, pulmonary atresia (PA) with ventricular septal defect (VSD) (32 cases, 15.7%); group 3, truncus arteriosus (12 cases, 5.9%); group 4, transposition of the great arteries with left ventricular outflow tract obstructions (8 cases, 3.9%); and group 5, PA with intact ventricular septum (7 cases, 3.4%). Remodeling surgery of the RV consisted of patch closure of the VSD (n = 176), tricuspid valvoplasty repair (n = 25), infundibulum muscle resection, and reconstruction of the RVOT (all patients). The Lecompte procedure was performed in 8 patients in group 4, and the one and a half ventricle technique was performed in 7 patients in group 5. RESULTS: There were 21 hospital deaths (10.3%); 180 patients (88.6%) survived. Patients were followed up from 4 to 206 months (mean, 98.0 months). Sixteen patients (8.8%) underwent reoperation for prosthesis dysfunction, with 2 in-hospital deaths (12.5%). The rest of the patients (164, 80.7%) remain free of reoperation. CONCLUSION: Earlier reconstruction of the pulmonary valve and the RVOT may preserve ventricular performance for a long period. Nevertheless, the porcine pulmonary prosthesis has shown satisfactory results when it has been used for the reconstruction of different types of RV obstructions.

Intracardiac cavopulmonary connection in patients with univentricular heart using intra-atrial lateral tunnel and intra-atrial conduit techniques.

BACKGROUND: In this study, we analyzed the time course of hemodynamic efficiency and follow-up in Fontan candidates who underwent the bidirectional Glenn procedure for staged intracardiac cavopulmonary connection (ICPC). METHODS: Between 1991 and 2008, 52 patients with univentricular heart (mean age, 3.3 years; range, 2-8 years; 27 female patients [51.9%]) underwent ICPC. The cardiac malformations were as follows: tricuspid atresia, 25 cases (48.0%); common ventricle, 16 cases (30.7%); and pulmonary atresia with intact ventricular septum, 11 cases (21.1%). The intracardiac cavopulmonary procedure was indicated for all 52 cases. In 42 patients (80.7%), an intra-atrial lateral tunnel was constructed with a bovine pericardium patch. In the last 10 consecutive cases (19.3%), we performed a modified surgical technique in which we implanted an intra-atrial corrugated bovine pericardium tube sutured around the superior and inferior vena cava ostium. In all cases, a 4-mm fenestration was made to reduce the intratunnel pressure. All 52 patients had previously undergone a Glenn operation. RESULTS: There were 2 hospital deaths (3.8%) and no recorded late deaths. During the follow-up, all patients were medicated with antiplatelet drugs. To evaluate the hemodynamic performance, we used Doppler echocardiography, computed tomography, and magnetic nuclear resonance studies. There were no prosthesis thromboses during this followup period. To evaluate cardiac arrhythmias, we conducted a Holter study. The last 10 patients with an intra-atrial conduit (IAC) presented with sinus rhythm and no arrhythmias during the last 4 years. The 50 surviving patients (96.1%) have been followed up for 6 to 204 months; all these patients are free of reoperation. CONCLUSION: The Glenn operation, which is performed at an early age, prepares the pulmonary bed to receive the ICPC. The midterm results of the intracardiac Fontan procedure seem to be good. The modified surgical procedure (IAC) can be a good alternative technique to the Fontan procedure in suitable patients.

One and a half ventricular repair as an alternative for hypoplastic right ventricle / Operação de um ventrículo e meio como uma alternativa para o ventrículo direito hipoplásico

OBJECTIVE: Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. METHODS: From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). RESULTS: There was one (11.1 percent) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5 percent) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.

OBJETIVO: Pacientes com cardiopatia congênita complexa e caracterizada por hipoplasia do ventrículo direito têm uma opção de correção paliativa com a operação de um ventrículo e meio. MÉTODOS: De julho de 2001 a março de 2009, nove pacientes (idade média de 5,2 anos, variando de 3 a 9 anos), com hipoplasia do ventrículo direito, foram submetidos à correção com a técnica de um ventrículo e meio. Os diagnósticos pré-operatórios foram: atresia pulmonar com septo ventricular intacto, em seis casos, e anomalia de Ebstein, em três casos. Seis pacientes tiveram derivação cavo-pulmonar bidirecional (operação de Glenn) prévia. A abordagem cirúrgica foi realizada com circulação extracorpórea e os defeitos intracardíacos foram corrigidos: fechamento do defeito do septo atrial (nove casos), reconstrução da via de saída do ventrículo direito com prótese pulmonar porcina (sete casos); plástica da valva tricúspide (três casos). RESULTADOS: Houve um (11,1 por cento) óbito. Todos os pacientes receberam alta hospitalar em boas condições clínicas. Um paciente apresentou estenose pulmonar distal à prótese, sendo necessária a reintervenção cirúrgica. Houve um (12,5 por cento) óbito tardio, após a reoperação. Sete (77,8 por cento) pacientes foram seguidos por período médio de 39,8 meses (intervalo de 16 meses para 8,4 anos) e se encontram em boas condições clínicas. CONCLUSÕES: O tratamento cirúrgico de cardiopatias congênita, na presença de um ventrículo direito hipoplásico, por meio da operação de um ventrículo e meio, tem menor risco cirúrgico que a correção biventricular, mantendo o átrio direito com baixa pressão, fluxo sanguíneo pulmonar pulsátil e melhora da saturação sistêmica de oxigênio. Os resultados de curto e médio prazo são satisfatórios; porém será necessário um maior tempo de seguimento para provar a eficácia desta abordagem cirúrgica a longo prazo.

One and a half ventricular repair as an alternative for hypoplastic right ventricle.

OBJECTIVE: Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. METHODS: From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). RESULTS: There was one (11.1%) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5%) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.

Late outcome of right ventricular outflow tract repair using bicuspid pulmonary prosthesis in tetralogy of Fallot surgery repair: case report / Resultado tardio da reconstrução da via de saída do ventrículo direito com prótese pulmonar bicúspide na correção da tetralogia de Fallot: relato de caso

We present a case of a 19 year-old female patient, with tetralogy of Fallot and hypoplastic pulmonary ring. Diagnosis was performed by echocardiography and cardiac catheterization. The surgical repair was performed at the age of 2 years old. A right ventricular outflow tract remodeling was performed using a porcine pulmonary bicuspid prosthesis. The patient presented a uneventful follow-up during 17 years. Postoperative assessment has shown: mild pulmonary insufficiency and mild pulmonary ventricle gradient, satisfactory right ventricular performance and prosthesis not presenting calcification. This is an option to correct the tetralogy of Fallot adopted by our Institution in the last two decades.

É apresentado o caso de uma paciente de 19 anos de idade, sexo feminino, portadora de tetralogia de Fallot com anel pulmonar hipoplásico. O diagnóstico foi feito com ecoDopplercardiograma e cateterismo cardíaco. A correção cirúrgica foi realizada com 2 anos de idade. Para a reconstrução da valva pulmonar e via de saída do ventrículo foi utilizada prótese bivalvular pulmonar porcina. A paciente teve boa evolução clínica durante 17 anos. A avaliação pós-operatória demonstrou: discreta insuficiência pulmonar, discreto gradiente transvalvar pulmonar, desempenho do ventrículo direito preservado e prótese pulmonar sem calcificação. Esse é o procedimento adotado pela nossa Instituição para a correção da tetralogia de Fallot, nos últimos 20 anos.

Late outcome of right ventricular outflow tract repair using bicuspid pulmonary prosthesis in tetralogy of Fallot surgery repair: case report.

We present a case of a 19 year-old female patient, with tetralogy of Fallot and hypoplastic pulmonary ring. Diagnosis was performed by echocardiography and cardiac catheterization. The surgical repair was performed at the age of 2 years old. A right ventricular outflow tract remodeling was performed using a porcine pulmonary bicuspid prosthesis. The patient presented a uneventful follow-up during 17 years. Postoperative assessment has shown: mild pulmonary insufficiency and mild pulmonary ventricle gradient, satisfactory right ventricular performance and prosthesis not presenting calcification. This is an option to correct the tetralogy of Fallot adopted by our Institution in the last two decades.

Procedimento de Lecompte para a correção de transposição das grandes artérias, associada à comunicação interventricular e obstrução de via de saída do ventrículo esquerdo / Lecompte procedure for correction of the transposition of the great arteries associated with ventricular septal defect and left ventricle outflow tract obstruction

OBJETIVO: Avaliar o procedimento de Lecompte para a correção da transposição das grandes artérias associada à comunicação interventricular e obstrução da via de saída do ventrículo esquerdo (TGA, CIV e OVSVE) e apresentar os resultados no período pós-operatório intermediário e tardio. MÉTODO: Entre fevereiro de 1994 e julho de 2005, sete pacientes, com idade de 2 a 8 anos (mediana -M-: 3,0), portadores de TGA, CIV e OVSVE, foram submetidos a tratamento cirúrgico corretivo. Em seis casos, foi utilizado o procedimento de Lecompte. Esta técnica consiste na abordagem por ventriculotomia direita, ressecção ampla do septo conal e construção de um túnel ventricular conectando o ventrículo esquerdo à aorta; o caso restante apresentava obstrução da prótese valvulada implantada entre o ventrículo direito e a artéria pulmonar (VD-AP) e falência do VD e foi submetido à conversão no procedimento de Lecompte. RESULTADOS: Os tempos de CEC variaram entre 105 e 194 min (M: 130) e os tempos de anoxia entre 65 e 90 min (M: 78). Houve um óbito no pós-operatório imediato devido a coagulopatia, seguido de insuficiência ventricular direita. Os seis pacientes sobreviventes receberam alta hospitalar no período de 5 a 30 dias (M: 11) e permaneceram em acompanhamento entre 12 a 144 meses (M: 73,6). CONCLUSÃO: O procedimento de Lecompte teve como vantagens: 1 - Indicação cirúrgica em pacientes com menor faixa etária; 2 - Baixa morbi-mortalidade; 3 - Expectativa de acompanhamento a longo prazo, sem reoperação; 4 - Possibilidade de converter o procedimento de Rastelli em Lecompte.

OBJECTIVE: To evaluate the Lecompte procedure used for the correction of transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) and left ventricle outflow tract obstruction (LVOTO) and to present the intermediate and long-term results of the surgery. METHODS: Between February 1994 and July 2005, seven patients with ages between 2 and 8 years (median: 3.0) suffering from TGA, VSD and LVOTO underwent corrective surgery. In six cases, the Lecompte procedure was performed. This technique consists in right ventriculotomy, extensive resection of the conal septum and construction of a ventricular tunnel connecting the left ventricle to the aorta (LV-Ao). The remaining case presented with obstruction of a valvular prosthesis implanted between the right ventricle and the pulmonary artery (RV-PA) and RV failure. This case was converted to the Lecompte procedure. RESULTS: The cardiopulmonary bypass time varied from 105 to 194 minutes (Median: 130) and the aortic clamping time varied from 65 to 90 minutes (Median: 78). There was one death in the immediate post-operative period due to coagulopathy followed by RV failure. Six patients were released from hospital between the 5th to 30th postoperative days (Median: 11) and the follow up period was from 12 to 144 months (Median: 73.6). CONCLUSIONS: The Lecompte procedure presents the following advantages: 1. Surgical indication for infants, 2. Low morbidity and mortality rates, 3. Free from reoperation over the long term. 4. Possibility of conversion of the Rastelli procedure into the Lecompte procedure.

Modified ultrafiltration in surgical correction of congenital heart disease with cardiopulmonary bypass.

The surgical correction of congenital heart disease using haemodilution and hypothermia with cardiopulmonary bypass (CPB) may expose patients to tissue ischaemia and initiate a systemic inflammatory response, increasing the total body water and inducing impairment, especially of heart, lung and brain function. It is possible to use ultrafiltration during CPB in the rewarming phase to remove water accumulation in the third space (conventional ultrafiltration). The reduced volume of prime used in children and the ability only to filter the reservoir blood during CPB led the Great Ormond Street Group to modify the method of ultrafiltration with regards to the placement of the filter and the timing of filtration (post-CPB). The main advantage of the modified technique is the ultrafiltration of the patient. A prospective nonrandomized study has been conducted to compare conventional with conventional + modified ultrafiltration. From January 1996 to March 1998, 41 patients underwent correction of congenital heart disease and were submitted to a comparative study (homogeneous groups), using either the conventional or the conventional + modified ultrafiltration techniques. There were no technical complications, no patient required mediastinal re-exploration due to bleeding and it was possible to close all the chests. There were significant differences in the ultrafiltrate volume balance (143.3 +/- 54.3 versus 227 +/- 71.4 mL; P < 0.001) but there were no significant differences in clinical postoperative evolution between the conventional and the conventional + modified ultrafiltration.

Assessment of splanchnic perfusion with gastric tonometry in the immediate postoperative period of cardiac surgery in children

OBJECTIVE - A prospective, nonrandomized clinical study to assess splanchnic perfusion based on intramucosal pH in the postoperative period of cardiac surgery and to check the evolution of patients during hospitalization. METHODS - We studied 10 children, during the immediate postoperative period after elective cardiac surgery. Sequential intramucosal pH measurements were taken, without dobutamine (T0) and with 5mcg/kg/min (T1) and 10 (T2) mcg/kg/min. In the pediatric intensive care unit, intramucosal pH measurements were made on admission and 4, 8, 12, and 24 hours thereafter. RESULTS - The patients had an increase in intramucosal pH values with dobutamine 10mcg/kg/min [7.19+/- 0.09 (T0), 7.16+/-0.13(T1), and 7.32+/-0.16(T2)], (p=0.103). During the hospitalization period, the intramucosal pH values were the following: 7.20+/-0.13 (upon admission), 7.27+/-0.16 (after 4 hours), 7.26+/-0.07 (after 8 hours), 7.32+/-0.12 (after 12 hours), and 7.38+/-0.08 (after 24 hours), (p=0.045). No deaths occurred, and none of the patients developed multiple organ and systems dysfunction. CONCLUSION - An increase in and normalization of intramucosal pH was observed after dobutamine use. Measurement of intramucosal pH is a type of monitoring that is easy to perform and free of complications in children during the postoperative period of cardiac surgery

Estudo angiográfico comparativo da artéria pulmonar no pré e pós-operatório de pacientes submetidos a operaçäo de derivaçäo cavopulmonar bidirecional / Comparative angiographic study of the pulmonary artery in pre and postoperative period in patients submitted to bidirectional cavopulmonary shunt surgery

INTRODUÇÄO: Este trabalho analisou as alteraçöes nos diâmetros da artéria pulmonar (AP) após a derivaçäo cavopulmonar bilateral (DCPB). CASUíSTICA E MÉTODOS: Foram incluídos 18 pacientes submetidos a DCPB, no período de março de 1990 a janeiro de 1997, que possuíam exames cineangiográficos disponíveis no período pré e pós-operatório. As medidas da AP direita e esquerda foram realizadas em três locais: na origem, imediatamente antes da bifurcaçäo e no início da artéria do lobo inferior. Em seguida, os diâmetros de cada local foram indexados à superfície corpórea ou ao diâmetro da aorta, medido na altura do diafragma. Os dados angiográficos e oximétricos foram submetidos a análise estatística. RESULTADOS: Quando analisados os diâmetros absolutos observou-se que a maioria sofreu aumento näo-significante, no período pós-operatório, ao passo que o diâmetro II da AP esquerda apresentou diminuiçäo. Os índices totais I e III apresentaram diminuiçäo significante, no período pós-operatório, e no índice total II a reduçäo näo foi significante. A análise da variável seguimento pós-operatório demonstrou reduçäo significativa dos índices totais nos pacientes com seguimento pós-operatório inferior a 23,6 meses. A presença de fluxo sangüíneo adicional determinou o aumento dos índices AP direita II e III, e pequena reduçäo nos outros índices. As medidas da AP indexadas pelo diâmetro da aorta revelaram comportamento semelhante à indexaçäo pela superfície corpórea. Na ausência de circulaçäo venosa colateral (CVC) observou-se aumento significante da saturaçäo de O2, por outro lado, a sua presença determinou um aumento näo-significante, durante o período de observaçäo

Estudo comparativo da ultrafiltração convencional e associação de ultrafiltração convencional e modificada na correção de cardiopatias congênitas com alto risco cirúrgico / Comparative study of standard ultrafiltration and its association with modification for the correction of high surgical risk congenital heart diseases

A necessidade de correção cirúrgica de má-formações cardíacas complexas, que requerem tempos prolongados de circulação extracorpórea (CEC) aumentou a morbimortalidade devido a retenção hídrica e reação inflamatória sistêmica. O objetivo deste estudo é comparar a evolução pós-operatória imediata de pacientes submetidos a ultrafiltração convencional (UFC) durante a CEC e ultrafiltração modificada (UFM) após CEC. Quarenta e um pacientes submetidos a correção cirúrgica de cardiopatias congênitas foram divididos em 2 grupos: G1: 21 pacientes com idade de 15 dias a 36 meses (mediana: 11 meses) e peso de 3,6 a 13,5 kg (M: 7,27 / 3,07), operados entre 1996 e 1997, foram submetidos a UFC. G2: 20 pacientes com idade de 9 dias a 36 meses (mediana: 5,5 meses) e peso entre 2,2 e 12 kg (M: 5,7 / 2,5), operados entre 1997 e 1998, foram submetidos a UFC+UFM. Dentre as operações mais freqüentes temos: ventriculosseptoplastia, 15 (36,5 por cento) casos; operação de Jatene, 10 (24,3 por cento ) casos; correção de defeito septal A-V total, 7 (17,0 por cento) casos etc. A análise estatística de idade, peso e complexidade cirúrgica mostrou semelhança entre os grupos. Houve 6 (28,5 por cento) óbitos no G1 e 4 (20 por cento) no G2, (p=0,71). O volume médio ultrafiltrado no G1 (UFC) foi 143,3 ml e no G2 (UFC+UFM) foi 227,0 ml, (p<0,001), mostrando diferença estatisticamente significante. Porém o tempo médio de ventilação mecânica (G1: 94.8h, G2: 95.6h, p= 0.97), tempo médio de uso de drogas inotrópicas (G1: 145.2h e G2: 137.6h, p=0.85); tempo médio de permanência em UTIP (G1: 169.6h e G2: 157.8h, p= 0.79) e tempo médio de permanência hospitalar (G1: 14,8 d. e G2 14,6 d., p= 0,95), não mostraram diferenças significantes entre os grupos. A técnica de UFC utilizada a mais de 8 anos no nosso Serviço mostrou resultados semelhantes quando comparada com a associação de UFC+UFM. A UFM mostrou-se eficaz na remoção de água corpórea após CEC, não havendo intercorrências com o método. Talvez um estudo randomizado, recrutando um número maior de pacientes permita detectar diferenças entre os dois métodos

Guia de tomada de decisão em cardiopatias congênitas em página Web na Internet: modelo Atresia Tricúspide / Guidelines to decision-making in congenital heart disease on the WEB interface: Tricuspid Atresia model

Estimulada pelo desafio de gerenciar toda a informação envolvida na formação do conhecimento na área de saúde, a Informática Médica desenvolveu-se em larga escala em todos os países. O recente sucesso da rede Internet como veículo de distribuição de informação incentiva a elaboração de programas médicos para utilização através dessa rede. A elaboração de um programa de apoio à decisão - para cardiopatias congênitas em forma de documento em hipertexto de World-Wide Web - apresentado pela Internet possibilitaria o aproveitamento das características de processamento e armazenamento distribuído dessa rede. Este projeto teve como objetivos: criar um modelo de guia de tomada de decisão em cirurgia cardíaca pediátrica usando como base a Atresia Tricúspide; avaliar o grau de complexidade da criação deste modelo e os benefícios pelo emprego de interface em página WEB; testar sua validação com os casos de 16 pacientes tratados na Universidade Federal de São Paulo, no período de 1980 a março de 1997. Com a utilização de hardware adequado e da linguagem de programação HTML, o programa foi desenvolvido com a utilização de 7 telas de fluxograma, conjunto de textos e 37 ilustrações. Durante a elaboração deste modelo foi possível constatar a possibilidade de fácil desenvolvimento e rápida atualização. O resultado da validação mostrou concordância significativa (91,66 por cento) com as indicações cirúrgicas realizadas pelos especialistas, na Universidade Federal de São Paulo.

Reconstruçäo da continuidade ventrículo-pulmonar (conexäo VD-TP): técnicas e táticas cirúrgicas / Reconstruction of the continuity of the right ventricle and pulmonary artery trunk (connection VD-TP): surgical technics and tactics

Os autores revisam as técnicas cirúrgicas utilizadas para a reconstruçao da continuidade ventrículo direito (VD) e o tronco pulmonar (TP), dando ênfase às diferentes técnicas e associaçao, dependendo das formas anatômicas de cada lesao. Apresentam, para ilustraçao, 2 casos, operados com o uso de um novo conceito de prótese biológica produzida com a valva pulmonar e o tronco pulmonar suíno, preservados em glutaraldeído mediante dois modelos: retalho bivalvulado e tubo valvulado na experiência atual. No período de maio/91 e junho/95, foram operados 48 pacientes, em 43 foi usado retalho bivalvulado e, em 5 casos, prótese tubular valvulada. Houve 5 (10,4 por cento) óbitos imediatos e 2 (4,6 por cento) tardios. Este novo conceito de reconstruçao da valva pulmonar e via de saída do ventrículo direito, usando heteroenxertos valvulados evita a insuficiência pulmonar significativa e apresenta baixa incidência de calcificaçao. É uma técnica facilmente reprodutível.