EP10.26: A case of fetal isolated total anomalous pulmonary venous connection with severe pulmonary vein obstruction

Objectives. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease. This study aimed to evaluate the outcomes of TAPVC repair in neonates, controlling for anatomic subtypes and surgical techniques.Methods. Between 1997 and 2013, 88 patients (median age: 16 days) underwent repair for supracardiac (31), cardiac (18), infracardiac (36), or mixed (3) TAPVC. All the patients underwent emergency operation due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included a side-to-side anastomosis between the pulmonary venous confluence and left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair.Results. The early mortality rate was 2.3% (2/88 patients). The echocardiogram showed no obstruction in the pulmonary vein anastomosis, and flow rate was 1.1–1.42 m/s in the 3-year follow-up period.Conclusions. The accurate preoperative diagnosis, improved protection of heart function, use of pulmonary vein tissue to anastomose and avoid damage of the pulmonary vein, and delayed sternum closure can reduce the risk of mortality. The preoperative severity of pulmonary vein obstruction, the timing of the emergency operation, and infracardiac or mixed-type TAPVC can affect prognosis. Using our surgical technique, the TAPVC mortality among our patients was gradually reduced with remarkable results. However, careful monitoring of the patient with pulmonary vein restenosis and the timing and method of reoperation should also be given importance.

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Serum Levels After Everolimus-Stent Implantation and Paclitaxel-Balloon Angioplasty in an Infant with Recurrent Pulmonary Vein Obstruction After Repaired Total Anomalous Pulmonary Venous Connection

Pediatric Cardiology ◽

10.1007/s00246-011-0054-1 ◽

2011 ◽

Vol 32 (7) ◽

pp. 1036-1039 ◽

Cited By ~ 12

Author(s):

Matthias J. Müller ◽

Ulrich Krause ◽

Thomas Paul ◽

Heike E. Schneider

Keyword(s):

Pulmonary Vein ◽

Balloon Angioplasty ◽

Stent Implantation ◽

Serum Levels ◽

Anomalous Pulmonary Venous Connection ◽

Pulmonary Vein Obstruction

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Emergency surgical treatment of total anomalous pulmonary venous connection

10.22541/au.163250080.03113880/v1 ◽

2021 ◽

Author(s):

Wu Yongtao ◽

Xiangming Fan ◽

Chen Li ◽

Dong Wang ◽

Junwu Su ◽

...

Keyword(s):

Risk Factors ◽

Surgical Treatment ◽

Pulmonary Vein ◽

Perioperative Period ◽

Post Discharge ◽

Aortic Cross Clamp Time ◽

Late Mortality ◽

Anomalous Pulmonary Venous Connection ◽

Pulmonary Vein Obstruction

Background: This study explores the strategy and effect of emergency surgical treatment for total anomalous pulmonary venous connection (TAPVC). Methods: From March 2009 to February 2020, 78 patients with TAPVC underwent emergency surgical correction. There were 51 males and 27 females. The median age was 39.5 days, and the median weight was 4.0 kg. The preoperative percutaneous oxygen saturation was 80.8±4.5%. Results: Of the cases investigated, seven died during the perioperative period, 16 had delayed chest closure, 19 had early pulmonary vein obstruction, two had secondary tracheal intubation, one had a brain complication, and one had third-degree atrioventricular block. Low weight, younger age, cardiopulmonary bypass time, and aortic cross-clamp time were identified as risk factors for early mortality. During the follow-up from four to 137 months, 12 cases did not respond to follow up. Ten patients died within one to six months after discharge. One patient underwent reoperation due to pulmonary vein obstruction. The longer hospital stays after operation and intensive care unit time were identified as risk factors for late mortality. Conclusions: Emergency surgery for severe TAPVC patients after admission had achieved good results in the near future. Prenatal diagnosis should be strengthened to save more patients. The higher late mortality rate indicates that such patients should strengthen post-discharge management to reduce the occurrence of post-discharge deaths.

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