A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Intra-operative trans-esophageal echocardiography in congenital heart disease.

Intra-operative trans-esophageal echocardiography (TEE) is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool. Before TEE was available for intra-operative use, significant residual abnormalities were frequently not detected. The result was often substantial post-operative morbidity and mortality and sometimes the need for re-operation. According to practice guidelines established by the Society of Cardiovascular Anesthesiologists and the American Society of Anesthesiologists, there is strong evidence for the usefulness of TEE in surgery for congenital heart disease because it significantly improves the clinical outcome of these patients. Before surgical correction, TEE helps confirm diagnosis and spot any additional lesion, while after the surgical correction, it provides baseline parameters for comparison after the surgical correction.

Tetralogía de Fallot, operación de Taussig-Blalock (18 casos operados) / Tetralogy of Fallot: surgical repair using the Taussig-Blalock procedure: revision of an article in this journal in 1948

Tetralogy of Fallot is the most common cause of cyanotic congenital heart disease in adults. This malformation consists of a ture anatomopathologic type represented by the following tetralogy: stenosis of the pulmonary artery... Surgical repair using the Taussig-Blalock procedure. Revision of an article published in this journal in 1948 with a revision of 18 cases

Ten-year experience with the arterial switch operation.

BACKGROUND: Arterial level repair is considered the most appropriate procedure for transposition of the great arteries. This report describes our experience with the arterial switch operation over the past decade. METHODS AND RESULTS: From January 1991 to January 2001, a total of 299 patients underwent an arterial switch operation for transposition of the great arteries or double-outlet right ventricle. Group I (n=169, 56.5%) comprised patients with transposition of the great arteries in whom the ventricular septum was essentially intact. Group II patients (n=130, 43.5%) had transposition of the great arteries with an additional significant ventricular septal defect or had double-outlet right ventricle with a subpulmonic ventricular septal defect. Of the total, 245 (82%) were males and 54 (18%) were females. In group I, the ages ranged from 2 days to 18 years (median 19 days) and weight ranged from 1.7 to 68 kg (median 2.5 kg). In group II, the ages ranged from 4 days to 4 years (median 90 days) and weight ranged from 2.5 to 17 kg (median 4 kg). Fifteen percent of the patients (25/169) in group I and 30% of the patients (39/130) in group II had features of bacteriologic infection. Arterial switch operation was performed on standard lines. In group I, 141 patients (83.4%) had a primary arterial switch operation while 28 (16.6%) underwent a rapid two-stage repair. Twenty-three patients required concomitant relief of associated anatomic left ventricular outflow tract obstruction. Operative mortality was 8.8% (15/169) in group I and 33% (44/130) in group II with an overall mortality of 19% (59/299). The major causes of operative mortality included pulmonary arterial hypertensive crisis (n=21), sepsis and related complications (n=16), and left ventricular failure (n=8). Coexisting arch anomalies, longer cross-clamp time, late presentation, and preoperative bacteriologic infections were incremental risk factors. Follow-up ranged from 1 to 10 years and was 87% complete. On follow-up, 91% of the patients were asymptomatic and off all medications. There were 3 late deaths and 5 patients required reoperation. CONCLUSIONS: Excellent long-term results are obtained in operative survivors following the arterial switch operation. However. operative mortality remains a concern in our set-up.

The use of nonfluoroscopic catheter-based mapping system to perform radiofrequency ablation in complex ventricular tachycardia after cardiac surgery in congenital heart disease: a case report.

A nonfluoroscopic electroanatomical cardiac mapping system (CARTO) integrates anatomical and electrophysiological information to reconstruct a three-dimensional activation map. Information from the CARTO system helps to reveal the mechanism and perform successful ablation in scar re-entry ventricular tachycardia after cardiac surgery. Three-dimensional activation and propagation mapping was performed in a patient with ventricular tachycardia after surgical correction of a double outlet right ventricle. The ventricular tachycardia appeared in two morphologies and were refractory to antiarrhythmic medication including amiodarone. Both ventricular tachycardias were re-entered using the ventriculotomy scar but rotated in different directions. Successful radiofrequency ablation was performed by creating a line of conduction block from the pulmonic valve to the ventriculotomy scar using entrainment mapping and the ablation lesion tagging technique. The CARTO system is useful in mapping and guiding the ablation of complex ventricular tachycardia after surgical correction in congenital heart disease

Dupla via de saída do ventrículo direito com comunicaçäo interventricular näo relacionada: resultados da correçäo cirúrgica com técnica de múltiplos retalhos / Double-outlet right ventricle with non-committed ventricular septal defect: surgical results using the multiple patches technique

Objetivo: Apresentar uma modificaçäo técnica para a correçäo cirúrgica biventricular da dupla via de saída de ventrículo direito com comunicaçäo interventricular näo relacionada. Métodos: No período de abril de 1987 a fevereiro de 1996, 15 pacientes portadores desta anomalia foram submetidos à técnica de reconstruçäo biventricular com "tunelizaçäo do ventrículo esquerdo para a aorta utilizando-se 2 ou mais retalhos de pericárdio bovino. A idade variou entre 2 a 13 anos (média = 4,8 anos; média = 5 anos). Treze pacientes apresentavam situs solitus e levocardia, 1 paciente situs inversus e dextrocardia e 1 paciente situs solitus e dextrocardia. A construçäo do túnel inicia-se no átrio direito. Sendo a CIV pequena ou restritiva, é feita a ampliaçäo em sentido anterior e o primeiro retalho é suturado na borda póstero-inferior do defeito direcionando-o para o septo, em posiçäo anterior e superior. A seguir, através de ventriculotomia direita o segundo, terceiro e, por vezes, o quarto retalhos säo suturados direcionando o túnel para o anel aórtico. Resultados: Houve 3 óbitos (mortalidade de 20 por cento), 2 hospitalares, 1 tardio e os 12 pacientes restantes foram seguidos por um período variável de 10 meses a 9 anos (média 33 meses) encontrando-se todos em classe funcional I (NYHA). Comunicaçäo interventricular residual, mínima, foi observada em 1 paciente, estenose pulmonar residual em 2 pacientes e insuficiência pulmonar em 1 paciente. Em nenhum dos pacientes demonstrou-se obstruçäo do túnel ventrículo esquerdo - aorta. Conclusäo: Baseados nestes resultados, concluímos que esta técnica para reparo biventricular permite a construçäo de um túnel com diâmetro interno adequado, respeitando as mudanças de direçäo existentes entre a comunicaçäo interventricular e a aorta. O túnel de pericárdio bovino ocupa menor espaço intraventricular causando, portanto, menor risco de obstruçäo da via de saída de ventrículo direito.

Esternotomia mediana como via preferencial na anastomose de Blalock-Taussig modificada / Median sternotomy as a preferencial approach for modified Blalock-Taussig anastomosis

A abordagem usual para a realizaçäo da anastomose de Blalock-Taussig modificada (ABTM) tem sido a toractomia lateral. Esta via acarreta necessariamente trauma ao parênquima pulmonar e acesso difícil por ocasiäo da operaçäo definitiva. A esternotomia mediana (EM) apresenta-se como uma alternativa com certas vantagens. Este trabalho visa avaliar a viabilidade técnica e os resultados da realizaçäo da ABTM por EM. Dez pacientes foram submetidos a esta técnica, interpondo-se enxertos de PTFE. A mortalidade imediata foi de 30 por cento, e a tardia de 10 por cento. Complicaçäo imediata ocorreu em 10 por cento. O tempo médio de internaçäo foi 7,0 + 0,5 dias. A variaçäo da saturaçäo da hemoglobina pré e pós-operatória foi 27,5 + 11,7 por cento (p<0,001). A ABTM é factível por EM com vantagens de acesso fácil e rápido, evita dano pulmonar, possibilita alternativas para anastomoses ao tronco ou artéria pulmonar esquerda, viabiliza a canulaçäo e perfusäo, se necessário, e talvez facilitar a dissecçäo na reoperaçäo.

Single lung Rastelli repair.

A four year old severely cyanotic child was diagnosed to have double outlet right ventricle, large subpulmonic ventricular septal defect, severe pulmonic valve stenosis with absent left pulmonary artery and bilateral superior vena cavae without any connecting vein. A Rastelli type repair was performed utilizing the right pulmonary artery as the only run-off for the right ventricle. Immediate post-pump right ventricular (RV) pressures were 90 percent of the systemic. Six months later, echocardiographically estimated RV pressure was 60 percent of the systemic. The child is doing well at 6 months follow-up.

[Operative results of open heart surgery to 47 cases of congenital cardiopathies-Second part: cyanogen heart diseases]

The second part of the surgical results of 47 congenital heart diseases deals with the cyanotic type. From 1970 to 1976, 18 patients were operated on [among them, 15 cases of Fallot's tetralogy]. The immediate and longer term results of this surgery are analysed