Comparison of intracardiac and upturned approach for total anomalous pulmonary venous connection / 中华胸心血管外科杂志

Objective:Analyze the effect of intracardiac method and upturning method in the treatment of infracardiac total anomalous pulmonary venous connection(TAPVC), to explore the surgical method of infracardiac TAPVC.Methods:From July 2011 to August 2019, 20 patients with infracardiac TAPVC were treated, including 12 cases with upturning method and 8 cases with intracardiac method. The cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time, mechanical ventilation time, postoperative days and anastomotic flow rate were compared between the two groups.Results:There was no significant difference in cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time and mechanical ventilation time between the two groups. The postoperative hospital stay in upturning group was significantly lower than that in intracardiac group [(14.7±2.9)days vs.(16.1±6.2)days, P<0.05], and the postoperative anastomotic velocity > 120 cm/s in intracardiac group was significantly less than that in upturning group(1 case vs. 7 cases, P<0.05). Two patients died in upturning group, but there was no significant difference compared with the intracardiac group. Conclusion:There is no significant difference between the two methods in the treatment of subcardiac TAPVC. The authors think that the exposure of the upturning methods is difficult, and the distortion of the anastomosis may be hidden trouble. The in situ anastomosis of the intracardiac method is not easy to make mistakes.

Echocardiographic diagnosis of infracardiac total anomalous pulmonary venous connection / 北京大学学报(医学版)

Objective:To investigate the clinical usefulness of echocardiography in the diagnosis of infracardiac total anomalous pulmonary venous connection (ITAPVC) in neonates and infants.Methods:Retrospective analysis on 8 patients with ITAPVC was performed using echocardiography between April 2006 and December 2016.There were 4 boys and 4 girls with a mean age of 79.8 days (ranging from 15 to 195 days).A combined scanning via parasternal,subcostal and apical acoustic windows had been employed to diagnose ITAPVC and to trace the course and site of the anomalous pulmonary venous drainage,and to confirm the direction of the inter-atrial shunt and enlargement of right atrium and right ventricle.Results:Of the 8 patients who received echocardiography,ITAPVC was diagnosed in 7 patients.Misdiagnosis by echocardiography was encountered in one patient.The diagnosis by echocardiography was compatible with the operative findings in 5 patients receiving surgery and with the results of multislice computed tomography in 6 patients.The diagnostic accuracy rate of ITAPVC was 87.5％.The indirect signs obtained from echocardiogram was coexistence of a small malformed,triangle-shaped left atrium and right to left shunting at atrial level with dilatation and tortuousness of portal vein or hepatic vein and abundant blood flow in liver.The direct signs was total pulmonary veins unconnected with left atrium,whose confluence joining into vertical vein drained right-inferiorly to portal vein or hepatic vein through diaphragm.Three parallel vessels including vertical vein,abdominal aorta and inferior vena cava arranged anteriorly,left-posteriorly and right-posteriorly with the opposite flow directions of inferior vena cava and the other two were found on sub-costal view.Sites of the drainage to the infra-diaphragm veins located portal vein in 8 patients.Stenosis of site of vertical vein connecting to portal vein or hepatic veins occurred in 3 patients.Conclusion:Echocardiography has significant value in the diagnosis of pediatric ITAPVC and is capable of providing important structural and hemodynamic information for preoperative assessment of surgery.With multiple windows and multiple sections,ITAPVC could be diagnosed accurately by echocardiography.However,it is necessary to differentiate ITAPVC with intrahepatic portosystemic venous shunts or hepatic arteriovenous fistula.

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.

Staged Repair for a Patient with Infracardiac Total Anomalous Pulmonary Venous Connection Complicated by Hypoplastic Left Heart Complex / 日本心臓血管外科学会雑誌

Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior <i>vena cava</i>, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.

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.

A modification of surgical procedure for infracardiac total anomalous pulmonary venous connection / 中华胸心血管外科杂志

Objective Infracardiac total anomalous pulmonary venous connection, a rare congenital cardiac defect, is associated with high mortality. A modification was designed for the procedure to reduce the post-operative obstruction in the pulmonary venous. Methods From September 2005 to December 2007, seven patients with infracardiac total anomalous pulmonary venous connection were treated with repair surgery through right side approach. The patients' age at operation was (70.57 ±44.67) days , the weight was (4.07 ±0.87) kg. Three patients had pulmonary venous obstruction, and 2 with small PFO. A modified right - side approach for repairing this defect was used. Results No death occurred after the operation. The postoperative complications included low cardiac output in 5 patients (71.43%), pulmonary hypertension crisis in 3patients ( 42.86% ) , mechanical ventilation for more than 7 days, which happened in 2 patients (28.58%) and pulmonary infection. All of the patients received follow-up. No residual shunt and pulmonary venous return obstruction were identified on echocardiogram(with a velocity from 1.2 m/s to 1.47 m/s). The heart function of patients was within the normal range(EF 0.70 -0.79, FS 0.32 -0.44). Conclusion The modified surgical procedure for the correction of infracardiac total anomalous pulmonary venous connection by right side approach was associated with favorite clinical outcomes, The post-operative outcomes depended on the size of anastomosis between the common vein and left atrium and the patency of the pulmonary venous return. Adequate size of anastomosis and maintenance of the spatial structures in adjacent regions were helpful in decreasing the adverse effect of postoperative obstruction.

Infracardiac total anomalous pulmonary venous drainage with unusual presentation.

Total anomalous pulmonary venous drainage (TAPVD) is a rare entity which forms approximately 0.4 to 2% of all congenital heart disease. The infracardiac type usually involve obstructions on pulmonary venous connections and comprising a quarter of all TAPVD cases. The clinical findings in patients with obstructed infracardiac TAPVD could mimic respiratuary distress of several different etiologies during first hours of life. In this article,we present a case of a neonate with infracardiac type of TAPVD presented with only distinct subcutaneous veins of abdominal and thoracic wall.