Situs inversus totalis asociado a transposición de grandes arterias: un diagnóstico temprano / Situs inversus totalis associated with transposition of the great arteries: an early diagnosis

Resumen Introducción: El situs inversus totalis asociado a la transposición de grandes arterias es una condición médica rara y poco descrita. Existen pocos casos reportados con esta asociación, los cuales, en su mayoría, corresponden a pacientes adultos que presentan una transposición de grandes arterias corregida congénitamente. La complejidad del caso descrito está demarcada tanto por su etiología, como por su baja frecuencia de aparición y reto quirúrgico. Caso clínico: se describe el caso de un neonato con diagnóstico antenatal de transposición de grandes arterias, asociado a hallazgo de dextrocardia y dextrogastria que sugirió situs inversus totalis en radiografía de tórax posnatal, en quien se realizó procedimiento de Jatene de mayor complejidad a la usual por su anatomía. Conclusiones: La presencia de una asociación entre el situs inversus totalis y otra malformación anatómica, como la transposición de grandes arterias, es poco frecuente. Sin embargo, dicha asociación es un factor determinante tanto para la realización oportuna del diagnóstico, como para la elección del tratamiento apropiado y la presentación de posibles complicaciones posteriores.

Abstract Introduction: Situs inversus totalis associated with transposition of the great arteries is a rare and infrequently described medical condition. There are few reported cases of this association, most of which are in adult patients with congenitally corrected transposition of the great arteries. Objective: The complexity of the described case is marked by both its etiology as well as its infrequent presentation and surgical challenge. Clinical case: This was a newborn with a prenatal diagnosis of transposition of the great arteries associated with a finding of dextrocardia and dextrogastria which suggested situs inversus totalis on the postnatal chest x-ray, who underwent a more complex Jatene procedure than usual because of his anatomy. Conclusions: Situs inversus totalis associated with another anatomical malformation, such as transposition of the great arteries, is uncommon. However, this association is a determining factor for both timely diagnosis as well as for choosing the appropriate treatment, and for the development of possible subsequent complications.

Cannot extubate a newborn patient after an arterial switch operation? check major aortopulmonary collaterals!

Abstract The standard treatment of transposition of the great arteries is the arterial switch operation (ASO). Despite successful surgical correction, patients cannot tolerate extubation after the operation. Major aortopulmonary collaterals (MAPCAs) are one of the rare causes of prolonged mechanical ventilation due to significant hemodynamic effects. We report a 28-day-old newborn with transposition of the great arteries and a ventricular septal defect (VSD) who underwent ASO and VSD closure. After postoperative extubation failed twice, four large MAPCAs were revealed during heart catheterization. After transcatheter closure of these four MAPCAs, the patient was extubated and discharged 27 days after the procedure.

Late arterial switch surgery under ecmo support in a patient with transposition of the great arteries with intact ventricular septum: a case report

Abstract A 30-month-old male patient with transposition of the great arteries with intact ventricular septum (TGA/IVS) is presented. Arterial switch operation (ASO) was performed in the light of echocardiographic and angiographic findings. The patient remained under extracorporeal membrane oxygenation support for seven days postoperatively, and his cardiac functions returned to normal at the postoperative 10th day. He was discharged at the postoperative 20th day. The present case, which presents one of the most advanced ages at operation for TGA/IVS among previously reported cases, is used to discuss late ASO in this study.

The increasing importance of percutaneous mechanical circulatory assist device therapy in heart failure management.

Introduction: Advances in medical and surgical care have made it possible for an increasing number of patients with Congenital Heart disease (CHD) to live into adulthood. Transposition of the great vessels (TGV) is the most common cyanotic congenital cardiac disease where the right ventricle serves as systemic ventricle. It is not uncommon for these patients to have systemic ventricular failure requiring transplantation. Study Design: Hemodynamic decompensation in these patients can be swift and difficult to manage. Increasingly percutaneous LVAD’s such as the Impella (Abiomed, Mass, USA) are gaining popularity in these situations owing to their relative ease of placement, both in and outside of the operating room. Conclusion: In this paper we demonstrate that Impella (IMP) CP placement through the axillary artery approach shows to be suitable option for short term cardiac support and improvement of end organ perfusion in anticipation of cardiac transplantation.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension / Senning Paliativo no Tratamento de Cardiopatias Congênitas com Hipertensāo Pulmonar Grave

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Fundamento:A transposição das grandes artérias (TGA) é a cardiopatia cianogênica mais comum, com incidência variando de 0,2 a 0,4 por 1000 nascidos vivos. Muitos dos pacientes não tratados nos primeiros meses de vida podem evoluir com doença vascular pulmonar grave, e um método terapêutico para o tratamento desses pacientes pode ser realizado por meio de uma cirurgia paliativa para redirecionamento do fluxo em nível atrial.Objetivo:Relatar a experiência institucional com a cirurgia de Senning paliativo em crianças com diagnóstico de TGA e dupla via de saída do ventrículo direito com doença vascular pulmonar severa. Avaliar, também, a evolução clínica precoce e tardia da operação de Senning paliativo.Métodos:Estudo retrospectivo, baseado na avaliação de prontuários no período de 1991 a 2014. Somente pacientes sem indicação para o tratamento cirúrgico definitivo da cardiopatia em razão de elevadas pressões pulmonares.Resultados:Evidenciou-se após um ano de evolução um aumento médio da saturação arterial de oxigênio de 62,1% para 92,5% e uma redução média do hematócrito de 49,4% para 36,3%. A análise da histologia pulmonar foi possível em 16 pacientes. Em oito pacientes foi evidenciada lesão pulmonar grau 3 e 4.Conclusão:A cirurgia de Senning paliativo melhorou a saturação de oxigênio arterial, reduziu a policitemia e proporcionou uma melhora da qualidade de vida em pacientes com TGA com comunicação interventricular, os quais possuíam hipertensão pulmonar severa com prognóstico fechado.

Cirugía de Nikaidoh modificada: experiencia inicial y resultados a corto y a mediano plazos / The Modified Nikaidoh Procedure: Initial Experience and Short and Mid-term Outcomes

Introducción: El tratamiento convencional de los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar es la cirugía de Rastelli. Sin embargo, sus resultados son subóptimos, razón por la cual se han propuesto otras alternativas quirúrgicas. El procedimiento de Nikaidoh es una técnica reciente que surge como una opción terapéutica atractiva. Objetivo: Analizar nuestra experiencia inicial con la cirugía de Nikaidoh modificada: translocación aórtica y reconstrucción de ambos tractos de salida ventriculares. Material y métodos: Se analizaron 11 pacientes consecutivos operados con técnica de Nikaidoh modificada en nuestra institución en el período 2005-2014, con edad mediana de 3 años (intervalo intercuartil 25-75%: 1-5,5 años) y peso medio de 13,8 ± 5,4 kg, 9 de ellos con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar y 2 con doble salida del ventrículo derecho con estenosis pulmonar y fisiología de transposición. Todos tenían comunicación interventricular no relacionada con la aorta y un paciente tenía además ventrículo derecho hipoplásico. Resultados: En el posquirúrgico inmediato no hubo mortalidad, un paciente desarrolló endocarditis infecciosa y requirió reoperación. Seis pacientes tuvieron disfunción ventricular transitoria; solo uno necesitó asistencia ventricular. No se registró obstrucción a los tractos de salida ventriculares ni insuficiencia aórtica mayor que leve. En el seguimiento a mediano plazo, con una media de seguimiento de 5,5 ± 2,1 años (rango: 2-8,6 años), no hubo mortalidad ni reoperaciones. Se efectuó un cateterismo intervencionista para dilatación del conducto ventrículo derecho-arteria pulmonar. Todos los pacientes permanecían en clase funcional I, con buena función biventricular, sin arritmias, sin obstrucción al tracto de salida ventricular izquierdo y sin insuficiencia aórtica significativa. Conclusiones: La cirugía de Nikaidoh y sus modificaciones constituyen una buena opción quirúrgica para los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar, que por su morfología son malos candidatos para cirugía de Rastelli. En esta serie no hubo mortalidad y todos los pacientes se encuentran en clase funcional I. En el seguimiento a mediano plazo, el tracto de salida del ventrículo izquierdo permaneció sin obstrucción y no se registró insuficiencia aórtica significativa.

Background: The Rastelli operation is the conventional procedure for transposition of the great vessels with ventricular septal defect and pulmonary stenosis. Its results, however, are suboptimal and other surgical alternatives have been postulated. A recent surgical technique, the Nikaidoh procedure, appears as a new therapeutic option. Objective: The aim of this study was to review our initial experience using the modified Nikaidoh procedure consisting in aortic translocation and biventricular outflow tract reconstruction. Methods: The study analyzed 11 consecutive patients operated on with the modified Nikaidoh procedure at our institution between 2005 and 2014. Median age was 3 years (25-75% interquartile range: 1-5.5 years) and mean weight was 13.8 ± 5.4 kg. Nine patients had transposition of the great vessels with ventricular septal defect and pulmonary stenosis and 2 had double outlet right ventricle with pulmonary stenosis and transposition physiology. All patients had a ventricular septal defect unrelated to the aorta and one patient also had a hypoplastic right ventricle. Results: There were no deaths in the immediate postoperative period. One patient developed infective endocarditis needing reoperation. Six patients had some degree of transient left ventricular dysfunction, and one patient only needed ventricular assistance. There was no involved right or left ventricular outflow tract obstruction and only mild aortic regurgitation. At mid-term follow-up (mean of 5.5 ± 2.1 years, range: 2-8.6 years) there were no deaths or reoperations. An interventional cardiac catheterization to dilate the right ventricle to pulmonary artery conduit was performed in one patient. All patients remained in functional class I, with good biventricular function, free from arrhythmias, no left ventricular outflow tract obstruction and no significant aortic regurgitation. Conclusions: The Nikaidoh operation and its variants are a satisfactory surgical option for patients presenting transposition of the great vessels with ventricular septal defect and pulmonary stenosis, who due to their morphology are inadequate candidates for a Rastelli procedure. In the mid-term follow-up, the left ventricular outflow tract remained without obstruction and there was no significant aortic valve regurgitation.

Resultados inmediatos y alejados del switch arterial en pacientes condextrotransposición de grandes arterias: experiencia de 20 años / Early and late results of the switch operation for d-transposition of the great vessels. clinical experience in 108 patients operated on between 1992 and 2012

Objetivos: Comunicar los resultados de la operación de switch arterial en pacientes portadores de dextro Transposición de Grandes Arterias (D-TGA) y evaluar su evolución en el tiempo. Pacientes y Método: Estudio retrospectivo de pacientes sometidos a switch arterial entre mayo de 1992 y noviembre de 2012. Se comparó período 1 (1992 a 2002) con período 2 (2003 a 2012). Se definió D-TGA simple aquella sin lesiones asociadas y D-TGA compleja aquella con asociación de comunicación interventricular o coartación aórtica. Resultados: Un total de 108 pacientes componen la serie, 44 en el período 1 y 64 en el período 2, sin diferencias demográficas y anatómicas entre ambos períodos. Setenta tenían D-TGA simple y 38 D-TGA compleja. La mortalidad operatoria en el período 1 fue 33% versus 8,4 por ciento en el período 2 (p< 0.025); hubo tendencia a mayor mortalidad en pacientes con anatomía coronaria compleja. Se observó una disminución del riesgo relativo de mortalidad de 68,7 por ciento en el período 2. El 28,7 por ciento presentaron complicaciones postoperatorias, sin diferencias entre ambos períodos. Un paciente falleció en forma alejada. La mediana de seguimiento fue de 60 meses y la supervivencia de 84,25 por ciento a 10 y 20 años. Se realizaron 16 re intervenciones, principalmente plastías percutáneas de ramas pulmonares. La mayoría de los pacientes se mantenían asintomáticos. La mortalidad operatoria de los últimos 5 años fue 2,6 por ciento. Conclusiones: La mortalidad operatoria ha disminuido significativamente a lo largo de 20 años; persiste un mayor riesgo en ciertos patrones de anatomía coronaria. La supervivencia alejada y libre de reintervenciones es muy favorable.

Aim: to report the results of the arterial Switch operation in patients with D-transposition of the great vessels (D-TGA) and to evaluate their late course. Patients and Methods: A retrospective review of the clinical data on patients consecutively operated on for D-TGA using the switch procedure. Results obtained in patients operated on between 1992 and 2002 (Period 1) were compared to those obtained in patients undergoing their operation between 2013 and 2012. Patients with D-TGA and no complications were compared to those who had interventricular septal defect or aortic coarctation associated their TGA. Results: 44 patients belonged in Period 1 and 64 in Period 2 (total 108). Demographic and anatomical characteristics were similar in both periods. Simple D-TGA was present in 70 patients and complex D-TGA in 38. Operative mortality was higher in Period 1 compared to Period 2 (33 per cent vs. 8.4 percent, p<0.025). A higher, albeit not statiscally significant mortality was observed in patients with complex as opposed to simple D-TGA. A 68.7 per cent relative reduction mortality risk was observed in Period 2. Complications developed in 28.7 per cent of patients, with no difference between periods. Only 1 patient died during late follow up. Median follow up was 60 months and survival rate was 84.3 percent at 10 y 20 years. Sixteen patients required re-intervention, mainly to perform percutaneous plastic procedures on pulmonary artery branches. Most patients had an asymptomatic course during follow up. Surgical mortality for the last 5 years was 2.6 percent. Conclusion: surgical mortality for the arterial switch operation in patients with D-TGA has significantly decreased along a 20 year period. An increased surgical risk persists for patients with certain forms of coronary artery anomalies. Late survival free of re-intervention was the rule in these patients.

Cuidados en el tratamiento odontológico de pacientes pediátricos cardiópatas con transposición de grandes vasos: reporte de un caso / Care in the dental treatment of pediatric heart patients with transposition of the creat vessels: a case report

En este artículo se reporta el caso de un paciente de 12 años de edad, con una cardiopatía congénita conocida como transposición de grandes vasos, quien se presenta a consulta en la Clínica de Odontopediatría de la Facultad de Odontología de la Universidad de Baja California en Mexicali, México, para quien se estableció un protocolo de atencióncon interconsulta con el médico cardiólogo que lo atendió, así como con especialistas en periodoncia y odontopediatría. Para prevenir la endocarditis bacteriana se recomendó la profilaxis antibiótica en cada cita, administrándole clindamicina (600 mg) dos horas antes del procedimiento dental y posteriormente una tableta cada ocho horas durante siete días.Localmente, en cada cita se le administró mepivacaína al 1% como anestésico local, para su tratamiento periodontal y dental. Normalmente, los pacientes cardiópatas con antecedenteshospitalarios requieren una atención especial del odontopediatra en el control de la conducta en cualquier tipo de tratamiento dental...

This article reports the case of a 12-year-old patient who suffered from a congenital heart disease known as transposition of the great vessels, and attended the pediatric dental careservice at the Baja California University Dental School in Mexicali, Mexico. Treatment was planned based on consultation with the cardiologist, the periodontist, and the pediatric dentist. To prevent the risk of bacterial endocarditis, 600 mg clindamycin 2 hours before the dental procedures and 1 tablet every 8 hours for seven days afterwards was prescribed.1% mepivacaine was used as local anesthetic to perform periodontal and dental therapy. Usually, heart patients with history of hospitalization require special behavior management by the pediatric dentist for any kind of dental treatment...

Análise do processo de adaptação ventricular após bandagem do tronco pulmonar em animais adultos / Study of right ventricles adaptation process following pulmonary artery banding in adult animals

Pacientes portadores de transposição das grandes artérias submetidos previamente. Correção cirúrgica no plano atrial ou transposição corrigida das grandes artérias frequentemente evoluem com disfunção do ventrículo direito morfológico. Para estes casos, tem sido proposta a correção anatômica, chamada cirurgia de Double Switch ou dupla inversão, invariavelmente realizada na adolescência ou na idade adulta. Com isso, grande parte destes pacientes necessita preparar o ventrículo esquerdo, através da bandagem do tronco pulmonar. Várias são as dificuldades no preparo do miocárdio adulto e resultados desapontadores são relatados nestes indivíduos. O objetivo deste trabalho foi promover e analisar o processo de adaptação do ventrículo direito em modelo animal adulto, comparando o método de bandagem fixa, utilizado tradicionalmente na prática clínica, com um novo método de bandagem intermitente, por um período de quatro semanas. Foram utilizadas 18 cabras adultas, divididas Em três grupos: Chama (n = 6, peso = 26,42. 2,63 Kg, bandagem frouxa, sem sobrecarga sistólica), Fixo (n = 6, peso = 26,33 . 2,32 kg, bandagem convencional com sobrecarga sistólica contínua do ventrículo direito), Intermitente (n = 6, peso = 25,17. 2,48 kg, bandagem com dispositivo ajustável e 12 horas diárias de sobrecarga sistólica do ventrículo direito). No grupo fixo, os animais foram submetidos bandagem do tronco pulmonar com fita umbilical, tentando alcançar uma relação pressórica entre ventrículo direito e aorta de 0,7 no momento da cirurgia. Nos animais do grupo Intermitente, a sobrecarga sistólica foi promovida por um dispositivo ajustável calibrado percutaneamente. A mesma relação pressórica foi almejada diariamente através da insuflação do dispositivo por 12 horas, alternada com 12 horas de desinsuflação do dispositivo. Aferições hemodinâmicas foram feitas três vezes por semana no grupo Intermitente e duas vezes por semana nos grupos Fixo e Sham...

One of the late complications of patients with simple or congenitally corrected transposition of the great arteries includes right (systemic) ventricular failure. The policy of anatomic repair (double switch operation) has often been proposed for adolescents and adults. However, a period of reconditioning the left ventricle through pulmonary artery banding is often required. Although it appears to be capable of providing adequate left ventricular training when done at an early age, it is not always suitable for mature myocardium, with disappointing results in older patients. This study sought to assess two different methods of promoting right ventricles hypertrophy in adult animals for a four-week period, by means of fixed and intermittent adjustable pulmonary artery banding system. Eighteen healthy adult goats were distributed into three groups: Sham (n = 6, weight = 26.42 kg Ž 2.63 kg, loose pulmonary artery banding, no systolic pressure overload), Fixed (n = 6, weight = 26.33 kg Ž 2.32 kg, continuous systolic overload with fixed pulmonary artery band) and Intermittent (n = 6, weight = 25.17 kg Ž 2.48 kg, daily 12-hour systolic overload with adjustable pulmonary artery band). Sham group animals received a loose pulmonary band, while in the Fixed group, an umbilical tape was used to band the pulmonary trunk, adjusted during the implantation to achieve a 0.7 right ventricle / aorta pressure ratio. In the Intermittent group, an adjustable pulmonary artery banding device was implanted on the pulmonary trunk and adjusted according to the volume injected percutaneously. The banding system was inflated daily until the 0.7 pressure ratio was achieved. Systolic overload was maintained for 12 hours, alternated with a 12-hour resting period. All animals were submitted to echocardiographic studies on a weekly basis, while hemodynamic evaluations were performed three times a week in the Intermittent group and twice a week in the Fixed and Sham groups...

Transposição de grandes vasos corrigida congenitamente e gestação: relato de caso / Congenitally corrected transposition of great vessels and pregnancy: case report

Este é um relato de caso com discussão dos desfechos materno-fetais de gestantes com transposição de grandes vasos congenitamente corrigida (TGVCC). Gestante com transposição de grandes artérias congenitamente corrigida TGACC, internada com 35 semanas de gestação por dispneia e cianose central, evoluindo para parto vaginal com fórcipe e recebendo alta com recém-nascido clinicamente estável. A mesma paciente é internada novamente após 1 ano, gestante e com as mesmas queixas, apresentando dor precordial no trabalho de parto. Foi indicada cesárea por desproporção cefalopélvica. Teve alta clinicamente estável. Os recém-nascidos não apresentaram malformações cardíacas. Em conclusão, gestantes portadoras de TGACC devem receber atenção multidisciplinar diferenciada devido à gestação de alto risco, tendo indicação de parto normal assistido e monitorado, visando diminuir a morbimortalidade materna.

Case report and discussion of maternal-fetal outcomes in pregnant patients with congenitally corrected transposition of the great vessels (CCTGV). A pregnant woman with CCTGV was hospitalized at 35 weeks of gestation for dyspnea and central cyanosis. Her baby was born via forceps delivery and was clinically stable when discharged from hospital. The patient returned to the hospital 1 year later. She was pregnant and presented the same complaints, with precordial pain during labor. Cesarean delivery was indicated due to dystocia. She was clinically stable when discharged from hospital. Both newborns did not have cardiac malformations. Pregnant patients suffering from CCTGV should receive multidisciplinary attention due to the high-risk pregnancy, with recommendation of assisted and monitored vaginal labor to reduce maternal morbidity and mortality.

Modified Nikaidoh Procedure for Patient with TGA, Restrictive VSD, and PS / 대한흉부외과학회지

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

Bandagem ajustável do tronco pulmonar: comparação de dois métodos de hipertrofia aguda do ventrículo sub-pulmonar / Adjustable pulmonary trunk banding: comparison of two methods of acute subpulmonary ventricle hypertrophy

O preparo do ventrículo sub-pulmonar através da bandagem do tronco pulmonar (TP) pode ser aplicado nos pacientes portadores de transposição das grandes artérias (TGA) que perderam a chance da cirurgia no período neonatal ou naqueles já submetidos à correção no plano atrial (Senning ou Mustard) e ainda nos portadores de transposição corrigida das grandes artérias (TCGA), que evoluíram com disfunção do ventrículo direito (sistêmico). Nesses casos, a bandagem do TP poderá induzir a hipertrofia do ventrículo sub-pulmonar (ventrículo esquerdo) na TGA, habilitando-o para o manuseio da circulação sistêmica após a cirurgia de Jatene. Entretanto, durante o preparo do ventrículo esquerdo (VE), observa-se elevada morbimortalidade, provavelmente relacionada a uma sobrecarga aguda de pressão, não tolerada pelo ventrículo. Isto se deve à dificuldade em ajustar o diâmetro da bandagem do TP, realizada em condições não fisiológicas, com o paciente anestesiado e com o tórax aberto. O objetivo deste trabalho foi o de comparar a eficiência de dois protocolos experimentais de sobrecarga pressórica contínua e intermitente do ventrículo direito (VD) para induzir a hipertrofia rápida do ventrículo subpulmonar. Foram utilizados 21 cabritos jovens (30 a 60 dias de idade), divididos em três grupos: controle (n = 7, peso = 7,5 ± 1,9 kg), contínuo (n = 7, peso = 9,3 ± 1,4 kg, sobrecarga sistólica contínua do VD), intermitente (n = 7, peso = 8,1 ± 0,8 kg, 12 horas/dia de sobrecarga sistólica do VD). A sobrecarga sistólica foi obtida através de um manguito hidráulico de silicone implantado no tronco pulmonar. O dispositivo foi insuflado percutaneamente, através de um botão auto selante de silicone, com o objetivo de se atingir uma relação de pressões entre VD e VE de 70%. Avaliações ecocardiográficas e hemodinâmicas foram feitas diariamente. A sobrecarga sistólica do VD foi mantida por 96 horas no grupo contínuo e por quatro períodos de 12 horas, alternados com 12 horas de descanso...

Preparation of the subpulmonary ventricle with pulmonary trunk (PT) banding may be applied not only in patients with transposition of the great arteries (TGA) beyond the neonatal period, but also in those who present with systemic ventricular failure in corrected TGA or after failed atrial baffle operations. PT banding can induce subpulmonary ventricle hypertrophy that must be able to assume the increased work load and support the systemic circulation. However, subpulmonary ventricle training carries a high morbidity and mortality rates, probably related to acute pressure overload, not tolerated by the ventricle. Adjustment of PT banding under non-physiological conditions is hard to achieve. We sought to assess two different programs of systolic overload (continuous and intermittent) on the pulmonary ventricle (RV) of young goats with the aim of inducing rapid ventricular hypertrophy. Twenty one healthy 60-days-old goats were divided in three groups: control (n = 7, wt = 7.5±1.9 kg, no surgical procedure), continuous stimulation (n = 7, wt = 9.3±1.4 kg, continuous RV systolic overload), and intermittent stimulation (n = 7, wt = 8.1 ± 0.8 kg, 12 hours a day RV systolic overload). Pressure load was achieved by an adjustable PT banding device. The device was implanted around the PT and inflated percutaneously so that a 0.7 RV/LV pressure ratio was achieved. Echocardiographic and hemodynamic evaluations were performed every day. Systolic overload was maintained for 96 hours in the continuous group, while the intermittent group had four 12-hour periods of systolic overload, alternated with a resting period of 12 hours. The animals were then killed for water content evaluation. The inttermitent group achieved a significant increase (p<0,05) of RV mass (1,7 g/kg ± 0,5 g/kg) and septum (1,4 g/kg ± 0,3 g/kg), as compared to the ones from control group (RV: 0,9 g/kg ± 0,2 g/kg; septum: 1,0 g/kg ± 0,2 g/kg), while the continuous group showed only an RV mass increase...

Successful Conversion Arterial Switch Operation after Palliative Senning Operation / 대한흉부외과학회지

Six year-old female having TGA, VSD with severe PHT which was considered inoperable for anatomical correction, received palliative Senning procedure. During follow-up, she was given prostacyclin and at the age of 21, she received Senning takedown, arterial switch and VSD closure after a reevaluation of the hemodynamic status. Significant reduction in PHT was found and she is doing well without complication 3 months after the operation.

Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up

PURPOSE: The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. METHODS: Medical records of 94 patients(males 58, females 36; mean age at last follow-up, 12+/-9 years; mean follow-up duration, 9+/-6.4 years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. RESULTS: Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P= 0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. CONCLUSION: TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

Operative Treatment of Congenitally Corrected Transposition of the Great Arteries ( CCTGA ) / 대한흉부외과학회지

BACKGROUND: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. MATERIAL AND METHOD: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. RESULT: Mean age was 5.5+/-4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3+/-1.4 to 2.2+/-1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54+/-49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0+/-5.6%, 75.0+/-5.6%, and 69.2+/-7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). CONCLUSION: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.