Double-outlet right ventricle with nonrelated ventricular septal defect: surgical results using the multiple patches technique.

OBJECTIVE: Introduce a new surgical technique for biventricular correction of double-outlet right ventricle with noncommitted ventricular septal defect. METHODS: From April 1987 to February 1996, 15 patients with double-outlet right ventricle with noncommitted ventricular septal defect were operated on using a new technique for biventricular repair with multiple bovine pericardial patches to create a tunnel between the left ventricle and the aorta. Ages ranged from two months to 13 years (mean age 4.8 years). Thirteen patients had situs solitus and levocardia, one patient had situs inversus and dextrocardia, and one patient had situs solitus and dextrocardia. Construction of the tunnel began at the right atrium. The ventricular septal defect (VSD) was enlarged anteriorly, if restrictive or small, and the first patch was sutured in the infero-posterior edge of the VSD. The second, third and sometimes the fourth patches were sutured in sequence, through the right ventriculotomy, directing the tunnel to the aortic annulus. RESULTS: Overall mortality was 20%, with two early and one late death. The surviving patients were followed-up for a period ranging from ten months to nine years (mean 33 months), and all were in functional class I (NYHA). Minimal residual ventricular septal defect was observed in one patient, stenosis in two patients and moderate pulmonary insufficiency in one. There was no obstruction of the intraventricular tunnel between the LV and the aorta. CONCLUSION: Based on these data, we conclude that this technical modification for the biventricular repair of the double-outlet right ventricle with noncommitted VSD allows for the construction of a tunnel with adequate internal diameter, respecting the spatial changes between the VSD and aorta. In addition, the intraventricular bovine pericardial tunnel takes up less space, thus reducing the incidence of right ventricle outlet obstruction.

Valved conduits of bovine pericardium for right ventricle to pulmonary artery connections.

Between February 1991 and October 1994, 29 patients received right heart bovine pericardial valved conduits. Patients' ages ranged from 19 days to 18 years (mean 3.5 years), with 8 patients younger than 1 year of age. Ten patients had truncus arteriosus type II or III, 7 had pulmonary atresia with ventricular septal defect (VSD), 6 had transposition of the great arteries and pulmonary stenosis (PS), 3 had corrected transposition with VSD and PS, 2 had double-outlet right ventricle, and 1 had aortic atresia with normal left ventricle and VSD. Two hospital deaths (6.9%) occurred, but no late deaths. The postoperative serial echocardiographic studies showed a mean transconduit gradient of 16 mm Hg with a range of 2 to 37 mm Hg. All patients are in functional class I. Although the intermediate results are satisfactory, considering the late complications known from others conduits, long-term follow-up is necessary.

[Clinical manifestation of aortopulmonary window as mitral regurgitation caused by secondary dilatation of the valvar anulus]. / Exteriorização clínica de janela aortopulmonar através de insuficiência mitral por dilatação secundária do anel valvar.

Two patients with huge aortopulmonary window, both ten months-old, that showed curiously up at a clinical viewpoint, including echocardiography, as a mitral valve regurgitation with a dilated mitral ring secondary to the accentuated blood volume through it, were reported. Early signs of heart failure, accentuated systolic murmur at mitral area and overload of left heart cavities, characterized clinical impression of a congenital mitral insufficiency, in both cases. The correct diagnosis was established at operation and at necropsy study, bringing it to us the need to a more accurate analysis of the different diagnostic elements, especially in anomalies which clinical manifestations show great similarities.

[Pediatric heart surgery in a general hospital. Procedures and results in a 5 years' experience]. / Cirurgia cardíaca infantil em hospital geral. Procedimentos e resultados em 5 anos de experiência.

PURPOSE: To establish the routines for pediatric cardiac surgery in a general hospital, with a view to accurate diagnostic, a more precise intervention, a reduction of the risks and consequently an improvement of the results. METHODS: Three hundred and ninety surgeries were carried out in children with congenital heart disease. The method use highlighted noninvasive diagnosis, joint lodging, reduced period of hospitalization, care with the collection and preservation of blood, as well as the preference for fresh blood, techniques of monitoring, anesthesia, cardiopulmonary bypass and myocardial protection, concern with aesthetic incision for female patients and postoperative critical care. RESULTS: A high rate of early extubation was observed, with a significant reduction of the pulmonary complications resulting from prolonged mechanical ventilation. Low haemodynamic instability, arrhythmia and postoperative bleeding rates were uncommon. The low mortality rate and the average hospitalization period confirmed the good results. CONCLUSION: With the establishment of routines for pediatric cardiac surgery, we observed a progressive improvement of the results, with low rates of morbidity and mortality in a general hospital.

[Submammary incision for median sternotomy]. / Incisão submamária para esternotomia mediana.

PURPOSE: To evaluate the indications, complications, cosmetic and functional results with submammary skin incision for a median sternotomy in females. METHODS: Two hundred and eighty female patients have undergone submammary skin incision for a median sternotomy between 1981 to 1991 ages ranged from 2 months to 42 years (m = 5 +/- 9 years). RESULTS: Adequate exposure of the heart was achieved in every case, there were no technical problems, no major complications and the cosmetic and functional results are excellent. CONCLUSION: Complications associated with this incision are insignificant and the cosmetic and functional results are excellent justified routinely this approach for young women.

[An extracardiac valved graft in the establishment of continuity between the right ventricle and the pulmonary arteries. The technics and the results of their use in correcting common truncus arteriosus]. / Enxerto valvulado extracardíaco no estabelecimento da continuidade entre o ventrículo direito e as artérias pulmonares. Técnicas e resultados de sua aplicação na correção do tronco arterioso comum.

PURPOSE: To evaluate the results of valved extracardiac conduits for establishment of right ventricle pulmonary artery continuity in patients with truncus arteriosus communis. METHODS: between January 1981-January 1991, 15 patients with persistent truncus arteriosus communis underwent surgical repair with valved extracardiac conduits for establishment of right ventricle-pulmonary artery continuity. Mean age was 2 years 9 months and mean weight 9.6 kg. Eight patients were females and seven males. The diagnosis was established by hemodynamics and/or echocardiographic studies and surgical indication was based in presence of cardiac insufficiency and prevention of pulmonary vascular disease. Valved conduits were performed with insertion of pericardial xenograft in woven dacron prosthesis. Diameters ranged between 12 to 22 mm. RESULTS: The in-hospital mortality was 33% and the low output syndrome was the main cause. Post-operative complications, with variable gravity, occurred in 80% of the patients. Reoperation occurred in four patients during the long term follow-up due to stenotic complications of the valved conduit, mean 5 years 2 months after implant. Late mortality was 30%, with correspondent actuarial life table of 66.6% in 1st year, 53.3%, 3rd and 46.2% in 5 and 10 years. CONCLUSION: Although the valved extracardiac conduits have significantly improved the survival of patients with truncus arteriosus communis, late stenotic complications were high determining continuous development of direct ventricle-arterial anastomosis techniques.

Reconstruction of stenotic or nonconfluent pulmonary arteries simultaneously with a Blalock-Taussig shunt.

Stenosis or discontinuity of the pulmonary arteries associated with congenital cardiac defects was repaired in 12 patients, in conjunction with the Blalock-Taussig shunt. This approach was based on the following concepts: (1) The shunted blood is more uniformly distributed to both lungs, which avoids predominance of flow to the ipsilateral lung and long-term pulmonary vascular damage; (2) because adequate blood flow is maintained, the contralateral lung growth will be near normal; (3) the possibility of future surgical correction is enhanced. The ages of the patients ranged from 2 to 24 months. Five had a primary diagnosis of tetralogy of Fallot, three had double outlet of the right ventricle, three had single ventricle, and one had a truncus type anomaly. All operations were performed without cardiopulmonary bypass. Dilation of the stenotic segment was performed in two patients, enlargement of the stenotic segment in three, and resection of the segment and end-to-end anastomosis in four. Nonconfluent pulmonary arteries were corrected in three patients. In one, the stenotic segment was resected and an anastomosis was made between the left pulmonary artery and pulmonary trunk. In another patient, a segment of the right subclavian artery was interposed between the pulmonary trunk and intrahilar left pulmonary artery. In the third patient, the right pulmonary artery was disconnected from the aorta and a tube was interposed between the right and left pulmonary arteries. There were no intraoperative or late deaths, and postoperative angiographic evaluations were satisfactory. We believe that in infants needing a Blalock-Taussig shunt the pulmonary artery anomalies, if present, should be corrected simultaneously.

Tratamento do aumento crônico do limiar de estimulação com o emprego de corticosteroides em pacientes portadores de marca-passo / Treatment of the chronic increase of the stimulation threshold with the use of corticosteroids in patients with artificial pacemakers

Dez pacientes portadores de marca-passo, tipo demanda ventricular inibido, que receberam implante via epimiocardica, apresentavam aumento crônico do limiar de estimulação num período de 3 a 24 meses após o implante. A terapêutica medicamentosa consistiu no emprego de dexametasona (16 mg/dia), com redução gradativa da dose, ate suspensão após 3 semanas de tratamento. Com este esquema terapêutico, ocorreram modificações significativas no limiar de estimulação, com retorno ao funcionamento normal do sistema num período máximo de 36 horas, em todos os pacientes. Para comprovar a eficácia do método, foi realizada em uma paciente troca de fonte geradora por unidade multiprogramavel, previamente a administração da dexametasona e determinação indireta do limiar de estimulação, variando-se a largura do pulso, ocorrendo redução gradativa durante o tratamento sem perdas de comando. Com a suspensão do tratamento, não foram observadas perdas de comando em nenhum dos pacientes, encontrando-se o mais antigo do estudo com 27 meses e o mais recente com 1 mês de acompanhamento. Concluiu-se que o tratamento do aumento crônico do limiar de estimulação pode ser realizado por método incruento em implantes epimiocardicos, com excelentes resultados imediatos, sem ocorrerem recidivas tardias

Atresia mitral associada à transposição das grandes artérias, com ventrículo esquerdo bem desenvolvido. Análise clínica e angiográfica e resultado da septostomia atrial / Mitral atresia associated with transposition of great arteries, with well developed left ventricle. Clinical and angiographic analysis and results of atrial septostomy

Os autores relatam o caso de um lactente com associação rara de defeitos congênitos no coração, mencionada, ate hoje, sete vezes: atresia da valva mitral, transposição das grandes artérias sendo ambos os ventriculos bem desenvolvidos. Atribuiu-se o bom desenvolvimento do ventrículo esquerdo, raramente encontrado na atresia mitral, a presença de grande comunicação interventricular que permitiu bom fluxo de sangue durante a vida intra-uterina. Paciente mostrava quadro clinico semelhante ao encontrado na transposicao das grandes arterias. A insuficiencia cardiaca melhorou com septostomia atrial pelo cateter ballao. O óbito, por insuficiência cardíaca, aos 65 dias de vida, decorreu de provável tamanho inadequado da comunicação interarterial criada 30 dias antes. São discutidos os planos de conduta

Estenose congênita da veia cava inferior na região supra-hepática. Correção cirúrgica de um caso / Congenital stenosis of the inferior vena cava in the suprahepatic region. Surgical correction of a case

Menino de 4 anos de idade, portador de estenose congenita da regiao supra-hepatica da veia cava inferior, associada a cirrose hepatica precoce, apresentava hepato e esplenonegalia, ascite e circulacao venosa colateral abdominal como principais sinais clinicos. A angiografia da veia cava inferior mostrou lesao obstrutiva com reducao concentrica por diafragma membranoso. A remocao cirurgica da membrana foi realizada por abordagem da veia cava, atraves de atriotomia direita, com auxilio de circulacao extracorporea. No periodo pos-operatorio, apresentou derrame pericardico seroso importante e ascite recorrente tratados com puncao evacuadora, albumina humana e diureticos. Tres meses apos a operacao, o paciente encontrava-se assintomatico.Destaca-se a importancia de diagnosticar e tratar precocemente esta rara anomalia congenita, que pode provocar lesao hepatica irreversivel

Tratamento cirúrgico da comunicação interventricular em 68 crianças menores de 12 meses de idade / Surgical treatment of ventricular septal defect in 68 children with less than 12 months of age

Sessenta e oito criancas portadoras de comunicacao intraventricular (CIV) com hipodesenvolvimento acentuado, hipertensao pulmonar grave ou insufuciencia cardiaca rebelde ao tratamento clinico, foram operadas de janeiro de 1974 a novembro de 1981. Quarenta e quatro apresentavam CIV isolada, 7 associada a banda anomala, 6 a persistencia do canal arterial,4 a comunicacao interatrial, 2 a constricao previa do tronco pulmonar, 2 a coarctacao da aorta, 1 a insufuciencia aortica, 1 a insuficiencia mitral e 1 a estenose mitral. Os defeitos associados foram corrigidos simultaneamente, exceto a coarctacao da aorta e a insuficiencia mitral por nao serem hemodinamicamente significantes. Em 7 pacientes, em precario estado geral ou com grave infeccao pulmonar, foi realizada a contricao do tronco pulmonar. Nos casos corrigidos, o tipo de comunicacao encontrada foi interinfundibular em 6 casos, perimembranosa em 57, muscular em 2 e posterior em 3. A via de abordagem foi atrial em 70,5% dos casos. Houve um obito, 30 dias apos a correcao, um paciente apresentou bloqueio atrioventricular total e 2 tiveram reabertura da CIV. Conclui-se que o tratamento cirurgico pode ser realizado nesta faixa com baixo risco e excelente resultado