Treatment of supracardiac total anomalous pulmonary venous connection in a single center / 中国胸心血管外科临床杂志

@#Objective    To analyze the surgical results of patients with supracardiac total anomalous pulmonary venous connection (TAPVC) in a single pediatric cardiac center. Methods    A retrospective study was conducted on 98 pediatric patients with supracardiac TAPVC receiving surgical repair from 2014 to 2019 in our center. There were 64 males and 34 females with a median surgical age of 3.0 (1.5, 7.0) months and a median weight of 5.0 (4.0, 6.0) kg. Twenty-three (23.5%) patients had preoperative pulmonary vein obstruction. Ninety-two (93.9%) patients received conventional surgical repair, while six (6.1%) patients were treated with the sutureless technique. The Cox regression model was used to analyze the data. Results    The median follow-up time was 26.50 (5.75, 44.25) months. There were 9 (9.2%) deaths. Lower weight at the time of repair (P=0.013) and prolonged cardiopulmonary bypass time (P=0.007) were associated with mortality. Postoperative pulmonary vein obstruction was observed in 8 (8.2%) patients. Associated risk factors for postoperative pulmonary vein obstruction included lower weight at the time of repair (P=0.042) and prolonged cardiopulmonary bypass time (P=0.002). Conclusion    Surgical repair of supracardiac TAPVC has achieved satisfactory results in our center. Risk factors such as lower weight at the time of repair and prolonged cardiopulmonary bypass time are associated with a poor prognosis.

Bailout procedure in obstructed supracardiac total anomalous pulmonary vein drainage / Procedimiento de rescate en drenaje venoso pulmonar anómalo total supracardíaco obstructivo

Abstract Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the inter ventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

Resumen El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

Drenaje pulmonar anómalo total con drenaje de pulmonares a vena porta / Total anomalous pulmonary drainage with drainage

Las anomalías de drenajes de las venas pulmonares se deben a una alteración precoz en el desarrollo embrionario de las venas pulmonares (derechas o izquierdas) mientras están presentes aún conexiones con venas sistémicas (cardinal, umbilicovitelinas). La conexión anómala total de las venas pulmonares constituye entre 0.4 y 2.0% de las cardiopatías congénitas: ocurre en 6.8 de cada 100,000 individuos. Se diagnostica en 68% de los pacientes en etapa neonatal, lo que refleja la gravedad de esta cardiopatía. La variedad de conexión anómala total de las venas pulmonares infracardiaca representa entre 15 y 26% de todas sus variedades.

The anomalies of pulmonary venous drainage due to alteration in early embryonic development of the pulmonary veins (right or left) while still connections are present with systemic veins (cardinal, umbilicovitelins). These malformations represent 0.4 to 2.0 % of all congenital heart diseases, with an incidence of 6.8 patients in every 100,000 births. Sixty eight percent of the cases are diagnosed in newborns, which indicates that most patients are symptomatic early in life. The infracardiac TAPVC represent 15 to 25% of all TAPVC.

Clinical profile of patients with Total Anomalous Pulmonary Venous Return and their short term outcome in pediatric cardiac centre at Dhaka Shishu Hospital.

A5Background: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis without proper intervention. It has been detected more frequently in recent year due to the advent of echocardiography. The aim of this study is to evaluate the clinical manifestations, age at diagnosis and short term outcomes in TAPVR patients. Methods: From 1st January 2013 to 31st December 2013, a total of 34 cases with TAPVR were admitted in pediatric cardiac centre at Dhaka Shishu Hospital, Dhaka, Bangladesh. All of them were evaluated with 2-dimensional (2-D) and color Doppler echocardiography examination. CXR and ECG were also done. Patient’s sex, age at diagnosis, types of TAPVR, clinical manifestations, radiological finding, ECG findings and outcomes were compiled and analyzed. Results: In 34 patients with TAPVR, 23 (67.6%) were male and 11 (32.4%) were female with male to female ratio of 2.09:1. Most of the patients were diagnosed between 0-6 months of age that is 13 (38.2%) cases were in 0-2 month’s age group, 14 (41.2%) cases were in more than 2 month’s to 6 month’s age group. Tachypnea and cyanosis were more common symptoms. The types of TAPVR was supra-cardiac 18 (52.9%), cardiac 11 (32.4%), infra-cardiac 3 (8.8%) and mixed in 2 (5.9%) cases. Pulmonary hypertension was present in 31 (91.2%) of 34 cases. Among them, 20 (58.8%) patients had severe pulmonary hypertension. The most common associated intra-cardiac lesions of TAPVR patients were ASD 13 (38.2%) and PFO 13 (38.2%). ECG findings of TAPVR, 18 (52.9%) patient had right axis deviation (RAD), right ventricular hypertrophy (RVH) and 14 (41.2%) had right axis deviation (RAD), right ventricular hypertrophy (RVH), right atrial enlargement (RAE). X-ray findings of TAPVR patients, 32 (94.1%) patients had Cardiomegaly and increased pulmonary vascularity. Among admitted patient, 3 (8.8%) patients died due to pneumonia and intractable heart failure, 31 (91.2%) patients referred to advanced cardiac centre for operative treatment. Conclusions: Tachypnea and Cyanosis were an obvious clinical symptom of TAPVR. 2-D and color Doppler echocardiography can provide quick and accurate diagnostic information of TAPVR. Death rate is high in TAPVR patient in spite of adequate medical management. So, early detection and definitive surgical treatment of TAPVR is much needed.

Improved treatment of total anomalous pulmonary venous drainage (supracardiac anastomosis) / 局解手术学杂志

Objective To summarize the effect of venous anastomisis from left atrium-common venous anastomisis ( supracardiac anasto-mosis) at the top of left atrium,and to find the best method to treat total anomalous pulmonary venous connection ( TAPVC) . Methods 52 cases,of which 35 male and 17 female with the age of 1 month to 41 years old and the weight of 3. 1~77 kg,hospitalized in West China hos-pital from January 2000 to April 2008,were treated by supracardiac anastomosis. Results One was dead and the other 51 cases were fully recovered and left hospital. After the operation,no anastomotic stenosis or arrhythmia was observed except the dead one. During follow-up peri-od which lasted from 3 months to 12 years,the heart function of 45 cases were normal. Conclusion supracardiac anastomosis can reduce the risk of anastomotic stenosis and arrhythmia,it is a promising method to treat supracardiac type TAPVC .