RESUMO

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. RESULTS: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). CONCLUSION: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Assuntos

RESUMO

INTRODUCTION: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. METHODS: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. RESULTS: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). CONCLUSION: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

Assuntos

RESUMO

ABSTRACT Introduction: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. Methods: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. Results: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). Conclusion: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

RESUMO

ABSTRACT Introduction: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. Methods: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. Results: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). Conclusion: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Assuntos

RESUMO

Abstract Clinical data: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. Diagnosis: Association of aortic arch interruption type A is uncommon and should be considered. Operation: Debanding of pulmonary arteries allowing for possible future complete repair.

RESUMO

CLINICAL DATA: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. DIAGNOSIS: Association of aortic arch interruption type A is uncommon and should be considered. OPERATION: Debanding of pulmonary arteries allowing for possible future complete repair.

Assuntos

RESUMO

Resumen Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Abstract Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

Assuntos

RESUMO

Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Assuntos

RESUMO

Las anomalías de las arterias coronarias son una condición clínica de muy baja incidencia y de estas el origen anómalo de la arteria coronaria derecha desde el tronco de la arteria pulmonar (ARCAPA) representa cerca de 0,002% en la población general. Se puede asociar a la presencia de otras anomalías cardíacas congénitas. Presentamos el caso de una recién nacida, pretérmino de 33 semanas, gemelar, a la cual se le realiza el diagnóstico de un probable origen anómalo de coronaria derecha en un estudio ecocardiográfico de rutina realizado en la unidad de terapia intensiva. Se confirma el diagnóstico mediante cateterismo cardíaco y se realiza la corrección quirúrgica definitiva a los 6 meses de edad, estando la niña actualmente asintomática y con una calidad de vida normal. Destacamos la baja frecuencia de la ocurrencia de dicha patología, realizamos una revisión sobre los tópicos principales en el desarrollo del árbol vascular coronario y las principales anomalías del mismo. Jerarquizamos la importancia de realizar un estudio ecocardiográfico adecuado como valoración de pacientes internados en unidades de terapia intensiva neonatal.

Coronary artery anomalies are a clinical condition of very low incidence. Out of these, the anomalous origin of the right coronary artery from the trunk of the pulmonary artery (ARCAPA) is estimated to represent 0.002% of the general population. It may be associated with the presence of other congenital cardiac anomalies. The study presents the case of a 33 week preterm newborn twin who is diagnosed with an anomalous origin of the right coronary in a routine echocardiographic study performed in the Intensive Care Unit. The diagnosis is confirmed by cardiac catheterization and definitive surgical correction is performed at 6 months of age, the child being currently asymptomatic and enjoying a normal quality of life. We stand out the low frequency of the occurrence of this pathology. We performed a review of the main topics in the development of the coronary vascular tree and their main anomalies. We emphasize on the importance of performing an adequate Echocardiographic study as an assessment of patients admitted to Neonatal Intensive Care Units.

As anomalias das artérias coronárias representam uma condição clínica de incidência muito baixa; e a origem anômala da artéria coronária direita do tronco da artéria pulmonar (ARCAPA) representa somente uma estimativa de 0,002% na população geral. Pode estar associada à presença de outras anomalias cardíacas congênitas. Apresentamos o caso de um recém-nascido, prematuro de 33 semanas, gêmeo, diagnosticado com provável origem anômala de coronária direita em estudo ecocardiográfico de rotina realizado na Unidade de Terapia Intensiva. Confirmou-se o diagnóstico através de cateterismo cardíaco e realizou-se a correção cirúrgica definitiva aos 6 meses de idade; a doença atualmente é assintomática e a menina tem uma qualidade de vida normal. Ressaltamos a baixa frequência da ocorrência da referida patologia; realizamos uma revisão dos principais tópicos no desenvolvimento da árvore vascular coronariana e suas principais anomalias. Destacamos a importância de realizar um estudo ecocardiográfico adequado como estratégia de avaliação de pacientes internados em Unidades de Terapia Intensiva Neonatal.

Assuntos

RESUMO

BACKGROUND: Surgical repair of common arterial trunk (CAT) by means of a homograft conduit has become a standard practice. We report our experience in the correction of this heart disease with a handmade bovine pericardial-valved woven Dacron conduit as an alternative procedure to homografts, with a focus on early, mid-term, and long-term results. METHODS: We designed a retrospective study that included 15 patients with a mean age of 1.5 years (range: three months to eight years), who underwent primary repair of simple CAT. Right ventricular outflow tract was reconstructed in all the cases with this handmade graft that was explanted at the time of its biological stenotic degeneration. A peeling procedure was performed at this time, in order to reconstruct the right ventricle-to-pulmonary artery continuity. RESULTS: Overall mortality was 13.3% (one death at the early postoperative primary repair and the other at the mid-term postoperative peeling reoperation). Actuarial survival rate was 93.3%, 86.7%, and 86.7% at 5, 10, and 15 years, respectively. All of the 14 survivors developed stenosis of the handmade conduit at the mid-term period (8 ± 3 years), but after the peeling procedure, 13 survivors remain asymptomatic to date. CONCLUSIONS: Primary repair of common arterial trunk using a handmade conduit can be performed with very low perioperative mortality and satisfactory mid-term and long-term results, which can be favorably compared with those reported with the use of homografts. When graft obstruction develops, peeling procedure is a good option because it does not affect the overall survival, although long-term outcomes warrant further follow-up.

Assuntos

RESUMO

Background: Congenital heart defects (CHD) are the most prevalent group of structural abnormalities at birth and one of the main causes of infant morbidity and mortality. Studies have shown a contribution of the copy number variation in the genesis of cardiac malformations. Objectives: Investigate gene copy number variation (CNV) in children with conotruncal heart defect. Methods: Multiplex ligation-dependent probe amplification (MLPA) was performed in 39 patients with conotruncal heart defect. Clinical and laboratory assessments were conducted in all patients. The parents of the probands who presented abnormal findings were also investigated. Results: Gene copy number variation was detected in 7/39 patients: 22q11.2 deletion, 22q11.2 duplication, 15q11.2 duplication, 20p12.2 duplication, 19p deletion, 15q and 8p23.2 duplication with 10p12.31 duplication. The clinical characteristics were consistent with those reported in the literature associated with the encountered microdeletion/microduplication. None of these changes was inherited from the parents. Conclusions: Our results demonstrate that the technique of MLPA is useful in the investigation of microdeletions and microduplications in conotruncal congenital heart defects. Early diagnosis of the copy number variation in patients with congenital heart defect assists in the prevention of morbidity and decreased mortality in these patients. .

Fundamento: Os defeitos cardíacos congênitos são o grupo de anormalidades estruturais mais prevalentes ao nascimento e uma das principais causas de morbidade e mortalidade infantil. Estudos têm mostrado a contribuição da variação no número de cópias na gênese das malformações cardíacas. Objetivos: Investigar a variação no número de cópias gênicas em crianças com defeito cardíaco conotruncal. Métodos: Multiplex Ligation-dependent Probe Amplification (MLPA) foi realizado em 39 pacientes com defeito cardíaco conotruncal. Avaliação clínica e laboratorial foi realizada em todos os pacientes. Os pais dos probandos que apresentaram alterações também foram investigados. Resultados: Variação no número de cópias foi detectada em 7/39 pacientes: deleção 22q11.2, duplicação 22q11.2, duplicação 15q11.2, duplicação 20p12.2, deleção 19p, duplicação 15q e 8p23.2 com duplicação 10p12.31. As características clínicas foram compatíveis com o relatado na literatura associadas com microdeleção/microduplicação encontrada. Nenhuma dessas alterações foi herdada dos pais. Conclusões: Nossos resultados demonstram que a técnica de MLPA é útil na investigação de microdeleções e microduplicações em defeitos cardíacos congênitos conotruncais. O diagnóstico precoce das variações no número de cópias em pacientes com defeito cardíaco congênito auxilia na prevenção de morbidade e diminuição da mortalidade nesses pacientes. .

Assuntos

RESUMO

Persistent truncus arteriosus in the 5th decade is a rare entity as the only physical anomaly. Truncus arteriosus consists of a single great arterial trunk with the systemic, pulmonary, and coronary circulation, with no atretic aortic or pulmonary vessel. The complex cardiac anatomy can be delineated with transesophageal and transthoracic echocardiography, as is illustrated the case of our patient, who was diagnosed in infancy, at 5 months of age. This is the oldest documented case report of Type 3 truncus arteriosus with balanced biventricular anatomy, whose survival was secondary to an innate severe branch pulmonary artery stenosis. .

RESUMO

The median survival of patients with truncus arteriosus is five weeks of age with rare cases reaching adulthood. The longest reported survivor with balanced ventricles, as the index case has, is 38 years of age. The index case is a 46-year old male with Type 3 truncus arteriosus. His case has never been documented in the English Literature as far as the author is aware.

La mediana de la supervivencia de pacientes con tronco arterioso es cinco semanas de edad, siendo raros lo casos que alcanzan la edad adulta. El caso más viejo reportado con ventrículos balanceados, como en el caso índice, es un sobreviviente de 38 años de edad. El caso índice es un hombre de 46 años con tronco arterioso de tipo 3. Su caso nunca se ha documentado en la literatura en inglés, hasta donde el autor tiene noticias.

Assuntos

RESUMO

Descrevemos um caso de adulto de 28 anos com suspeita de cardiopatia congênita desde o nascimento, não tratada na infância por opção da família. Aos 27 anos, foi feito diagnóstico de atresia pulmonar com comunicação interventricular e colaterais sistêmico-pulmonares, sendo contraindicada a cirurgia. Uma nova reavaliação em nosso serviço demonstrou tratar-se de um truncus arteriosus atípico. O fato de um tronco arterial comum com shunt esquerda-direita ter sido visualizado ao ecocardiograma foi um dado crucial para a indicação de novo cateterismo, abrindo perspectiva de correção cirúrgica. No momento, o paciente encontra-se bem, com 7 anos de evolução pós-operatória.

This is the case of 28 year-old adult with suspected congenital heart disease since birth, not treated in childhood at the his family's choice. At 27 years old, he was diagnosed with pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals, where surgery was contraindicated. A new review in our department showed that it was an atypical form of truncus arteriosus. The fact that a common arterial trunk with left-right shunt was viewed by echocardiography was a crucial fact for the indication of new catheterization, opening the prospect of surgical correction. Currently, the patient is well, with 7 years of postoperative outcome.

Describimos un caso de adulto de 28 años con sospecha de cardiopatía congénita desde el nacimiento, no tratada en la niñez por opción de la familia. A los 27 años, se hizo diagnóstico de atresia pulmonar con comunicación interventricular y colaterales sistémico-pulmonares, y se contraindicó la cirugía. Una nueva reevaluación en nuestro servicio reveló tratarse de un truncus arteriosus atípico. El hecho de un tronco arterial común con shunt izquierdaderecha haber sido visualizado al ecocardiograma fue un dato crucial para la indicación de nuevo cateterismo, abriendo perspectiva de corrección quirúrgica. Al momento, el paciente se encuentra bien, con 7 años de evolución posoperatoria.

Assuntos

Assuntos

RESUMO

Paciente de 27 anos, portador de sífilis terciária, manifestando isquemia miocárdica, com angina instável, secundária à oclusão do tronco da coronária esquerda. O diagnóstico foi confirmado pelos achados da sorologia e da patologia do fragmento da aorta.

A 27-year-old patient with tertiary syphilis, manifested as myocardial ischemia, presenting unstable angina, secondary to left coronary trunk occlusion. The diagnosis was confirmed by the serological findings and the pathological assessment of the aorta fragment.

Paciente de 27 años, portador de sífilis terciaria, manifestando isquemia miocárdica, con angina inestable, secundaria a la oclusión del tronco de la coronaria izquierda. El diagnóstico fue confirmado por los resultados de la serología y de la patología del fragmento de la aorta.

Assuntos

Assuntos

RESUMO

Objetivo - Avaliar os resultados dos enxertos valvulados extracardíacos, empregados no estabelecimento da continuidade entre o ventrículo direito e as artérias pulmonares em pacientes portadores de tronco arterioso comum. Métodos - Quinze pacientes portadores de tronco arterioso comum foram submetidos a cirurgia corretiva com a utilização de enxerto valvulado extracardíaco no estabelecimento da continuidade entre o ventrículo direito e as artérias pulmonares no período de janeiro de 1981 a janeiro de 1991. A idade média foi de 2 anos e 9 meses e o peso médio de 9,6 kg. Oito pacientes eram do sexo feminino e sete do masculino. O diagnóstico foi estabelecido através de estudo hemodinâmico e/ou ecocardiográfico e a indicação cirúrgica baseada na presença de insuficiência cardíaca e na prevenção da progressão de doença vascular pulmonar obstrutiva. Os enxertos foram confeccionados com a inserção de válvula biológica de pericárdio bovino em prótese tubular de "woven dacron", com diâmetro variando de12a22mm. Resultados - A mortalidade hospitalar foi de 33% tendo como causa principal a síndrome de baixo débito. Complicações pós-operatórias, de gravidade variável, ocorreram em 80% dos pacientes, incluindo os óbitos hospitalares. Na evolução tardia, quatro pacientes foram submetidos a reintervenções por complicação estenótica do enxerto valvulado, em média 5 anos e 2 meses após o implante. A mortalidade tardia foi de 30%, correspondendo a uma sobrevivência atuarial de 66,6% no 1° ano, 53,3% no 3° ano e 46,2% em 5 e 10 anos. Conclusão - Embora os enxertos valvulados extracardíacos tenham melhorado substancialmente a sobrevida dos pacientes portadores de tronco arterioso comum. As complicações estenóticas tardias impedem que os mesmos constituam uma solução definitiva para o problema original, motivando o constante desenvolvimento de técnicas de anastomose direta ventrícularterial.

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-pulomonary 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 insuficience and prevention of pulmonary vasculardisease. 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 anasthomosis techniques

Assuntos