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.

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.

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.

Revascularizaçäo direta do miocárdio com artéria gastro-omental esquerda: estudo anatômico e histológico e relato de caso / Direct myocardial revascularization with left gastroeploic artery: anatomic and histologic atudy and case report

A veia safena e a artéria mamária interna (AMI) säo os enxertos mais usados, até o momento, para a revascularizaçäo direta do miocárdio. O estudo de sua normalidade de fluxo, a longo prazo, tem mostrado melhor adaptabilidade dos pedículos arteriais. O estudo da viabilidade da artéria gastro-omental (AG-O), na revascularizaçäo direta do miocárdio, foi realizado mediante estudo anatômico em 25 cad=averes adultos, com idades entre 23 e 84 anos, material cedido pelo Departamento de Anatomia Patológica da Escola Paulista de Medicina. A tática cirúrgica consistiu na dissecçäo da AG-O esquerda, ligando suas colaterais e desligando-a da AG-O direita, ao nível do antro pilórico. Foram estudados os diâmetros da artéria e o comprometimento do pedículo. A transferência para a cavidade pericárdica foi realizada rodando o pedículo pela frente do fundo gástrico e passando-o através do orifício praticado no diafragma, anastomosando-o com a artéria coronária direita ou ramos marginais da artéria circunflexa. O estudo histológico da estrutura da AG-O mostrou camada média de tipo músculo-elástico com baixa incidência de aterosclerose. A aplicaçäo clínica desta técnica foi realizada numa paciente de 58 anos de idade, que apresentava ambas as veias safenas internas finas e fibrosadas. Foram dissecadas AMI esquerda, AMI direita e AG-O esquerda, revascularizando as artérias descendentes anterior, coronária direita e marginal esquerda, respectivamente. Após evoluçäo pós-operatória de 12 dias, sem intercorrências, foi realizada angiografia digital seletiva das artérias mamárias e esplênica, demonstrando a normalidade ao fluxo dos enxertos. esta nova possibilidade de revascularizaçäo miocárdica constitui uma opçäo a mais, entre o elenco de alternativas de que o cirurgiäo dispöe, especialmente quando näo existem possibilidades de escolha de encertos venosos

Revascularizaçäo miocárdica sem circulaçäo extracorpórea: resultados cirúrgicos em 1090 pacientes / Coronary Artery Bypass Surgery Without Cardiopulmonary Bypass. Surgical Outcome in 1090 Patients

PURPOSE--To report our experience of 12 years of myocardial revascularization without cardiopulmonary bypass (CPB) and to discuss its expanding surgical indications. METHODS--This technique was employed in 1090 patients, between September/81 and April/93, corresponding to 17.4 per cent of all revascularized patients. Regarding gender, male patients were the majority (73.7 per cent) and ages ranged from 28 to 86 year-old (mean 57). Chronic coronary insufficiency was the main indication (69.4 per cent) and 18.9 per cent of our global experience were operated on during acute ischemia. The average number of grafts per patient was 1.6 and internal mammary artery was utilized in 60.1 per cent of patients. Left anterior descending coronary artery and right coronary artery were the coronary arteries most often treated. RESULTS--Our hospital mortality rate was 2.5 per cent (28/1090) although it was 7 per cent for patients over 70 years. The most common cause of death was cardiogenic shock after failed angioplasty. Postoperative complications included AMI in 4.8 per cent, arrhythmias in 5.5 per cent and pulmonary in 3.2 per cent. Mean of any type of blood derivate utilization was 0.28 units/patients. CONCLUSION--Myocardial revascularization without CPB is a valid alternative in a selected cohort of patients and it is performed with low morbidity and mortality. Nowadays it represents an excellent tactical option for high-risk patients and in failed angioplasties

Operaçäo de Blalock-Taussing modificada para o tratamento paliativo de cardipatias congênitas com hipofluxo pulmonar / Modified Blalock-taussig operation for the palliative of congenital cardiopathies with low pulmonary flow

De janeiro de 1990 a novembro de 1994, 72 pacientes portadores de cardiopatias congênitas com hipofluxo pulmonar foram submetidos à operaçao de Blalock-Taussig. A idade variou entre 2 dias e ll anos (M:9,0 meses); 44 (61,1 por cento) eram do sexo masculino e 28 (38,8 por cento) do feminino; 38 (52,8 por cento) casos portadores de tétrade de Fallot; 7 (9,7 por cento) atresia pulmonar com septo interventricular íntegro (AP c/ SIVI); 6 (8,4 por cento) transposiçao das grandes artérias (TGA) + estenose pulmonar (EP); 6 (8,4 por cento) atresia tricúspide (AT) + EP; 6 (8,4 por cento) dupla via de entrada (DVE) do ventrículo direito (VD) ou ventrículo esquerdo (VE) + (EP); 3 (4,2 por cento) transposiçao corrigida das grandes artérias (TCGA) + comunicaçao interventricular (CIV) + EP; 2 (2,7 por cento) dupla via de saída (DVS) de VD ou VE + EP; 2 (2,7 por cento) defeito septal atrioventricular (DSAV) + EP; 2 (2,7 por cento) isomerismo direito (D) ou (E) + EP. Os critérios de indicaçao foram: a) neonatos com cardiopatias "ducto dependente"; b) lactentes com piora de cianose ou crise de hipóxia; c) crianças durante a infância com cardiopatias sem chance de uma correçao biventricular. A técnica empregada foi a operaçao de Blalock-Taussig modificada, interpondo prótese entre artéria subclávia e artéria pulmonar. A prótese de Polytetrafluoroethylene (PTFE) foi usada em 69 (94,5 por ento) casos, veia umbilical em 3 (4,1 por cento) casos e artéria mamária bovina em l (l,4 por cento) caso. Durante o ato operatório foi administrada dose única de heparina (lmg x kg peso), nao sendo neutralizada no pós-operatório (PO) e no seguimento tardio, dispensado o uso de anticoagulantes ou antiagregantes plaquetários. Houve 8 (l1,1 por cento) óbitos no PO imediato: 5 (6,9 por cento) casos por obstruçao da prótese (3 foram reoperados), 2 (2,7 por cento) casos por morte súbita (AP c/ SIVI) e l (l,3 por cento) devido a processo infeccioso pulmonar. A obstruçao da prótese como principal causa de óbito esteve diretamente relacionada à anatomia dos vasos (calibre da artéria subclávia e pulmonar) e ou problemas de técnica cirúrgica. A operaçao de Blalock-Taussig modificada demonstrou ser um método confiável e seguro no tratamento paliativo das cardiopatias com hipofluxo pulmonar.

Cirurgia cardíaca em índios brasileiros / Heart Surgery in Brazilian Indians

PURPOSE: Our experience with surgical treatment of heart diseases in Indians living in the Amazon rain forest in primitive stages was reviewed. METHODS: From 1988 to 1995, 18 patients underwent cardiovascular surgical procedures at the São Paulo Hospital of the Escola Paulista de Medicina. Seven patients had valvar disease, nine congenital heart defects, one submitral aneurysm and one arrhythmia. Thirteen Indians came from tribes of the Amazon rain forest area: three from the Xavante, two from Waiapi, two from Tucano, two from Macuxi, two from Mayoruna, and one of each tribe of Xikrin, Guajajara, Terena, Surui, Galibi, Cinta-Larga and Pataxó. RESULTS: We performed 22 operations, with two hospital deaths. Follow-up was possible in 87.5 of cases, with one late death. The majority of cases were due to congenital heart defects and in this series it was noted the absence of operations to treat coronary artery disease. The incidence of valve disease was higher in accultured or semi-accultured Indians. CONCLUSION: The surgical treatment of cardiovascular disease has made possible to the surviving indians to return to and be accepted by their fellow tribesmen.

Reconstituiçäo da valva pulmonar e via de saída do ventrículo direito, com prótese bivalvular e prótese tubular valvada de tronco pulmonar de porco estudo experimental e aplicaçäo clínica / Pulmonary valve and outlet right ventricle reconstruction with bivalvular prosthesis and valved tubular prosthesis of the pig pulmonary trunk: experimental study and clinical application

A obstruçao da via de saída do ventrículo direito (VSVD) tem gerado muita polêmica em torno da técnica da sua correçao cirúrgica, sendo a reconstituiçao ainda motivo de controvérsias. Com essa finalidade, foram desenvolvidas duas próteses a partir do tronco pulmonar (TP) de porco: 1) a prótese bivalvular: poderia ser usada na correçao da tétrade de Fallot associada a hipoplasia do anel pulmonar: 2) a prótese tubular valvada, possuindo a própria valva pulmonar: poderia ser empregada na correçao de malformaçoes com descontínuidade entre o VD e TP. Estes dois tipos de próteses foram testados em modelo experimental. Seis ovelhas foram submetidas a implante de prótese bivalvular, com o auxílio da circulaçao extracorpórea (CEC), após ampla ressecçao do infundíbulo pulmonar, incluindo duas válvulas da valva pulmonar, procurando-se, com isto, imitar a reconstituiçao empregada no Fallot. O implante da protese tubular valvada foi realizado em 12 ovelhas, sem o auxílio da CEC, mediante pinçamento tangencial do infundíbulo e TP, permitindo o desvio do fluxo sangüíneo através do conduto, após ligadura do TP. A prótese bivalvular implantada foi avaliada mediante parâmetros hemodinâmicos e ecocardiográficos na fase intra-operatória, conferindo desempenho satisfatório (insuficiência pulmonar discreta ou ausente e gradientes VD-TP menores de 10 mmHg). A seguir, os animais foram sacrificados. O desempenho da prótese tubular valvada foi avaliada na fase intra-operatória com medidas hemodinâmicas, mostrando gradientes acima de 1O mmHg em apenas 3 casos. Sete ovelhas tiveram controle ecocardiográfico com 99 a 135 dias de evoluçao, registrando gradientes de 9,85 mmHg a 49 mmHg (média 19,7). Quatro casos foram submetidos a estudo hemodinâmico no 6( mês de evoluçao, registrando discreto aumento do gradiente (média 22,3); a seguir os animais foram sacrificados e encaminhados para estudo anatomopatológico. A aplicaçao clínica da prótese bivalvular foi realizada em 3 pacientes portadores de t.de Fallot associada a hipoplasia do anel pulmonar (2 casos) e agenesia da valva pulmonar (1 caso), com idades de 16, 2 e 7 anos. Após evoluçao de 3 a 10 meses, os gradientes variaram entre 10 mmHg e 20 mmHg e discreta insuficiência pulmonar valvar ao estudo ecodopplercardiográfico...