Mixed type total anomalous pulmonary venous connection: Early results and midterm outcomes of surgical correction

Abstract

Background and Aim of the Study The morphological heterogeneity of anomalous pulmonary venous drainage in mixed type total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical repair resulting in high mortality in these patients. Methods A retrospective review of 14 patients with mixed type TAPVC undergoing biventricular repair between January 2012 and December 2019 was conducted. A descriptive analysis was done, highlighting the anatomic variation, diagnostic and surgical approach, and surgical outcomes in these patients. Results The most common anatomic pattern was “3 by 1” (79%) followed by “2 by 2” (21%). The correct diagnosis by transthoracic echocardiography was made in 10 (71%) of the 14 patients. In contrast, preoperative computed tomographic (CT) angiography was performed in 10 patients and correct diagnosis was obtained in 8 (80%) of them. Pulmonary venous obstruction was seen in one patient before surgery. The in‐hospital mortality was 14% (2/14). Four patients had pulmonary hypertensive crisis in the postoperative period. The average follow‐up was 54 ± 27 months (range: 17–98 months) after surgical repair, and all surviving patients were asymptomatic. There was no late death. No clinically apparent sequelae were seen in six patients in whom isolated left superior pulmonary vein drainage was left uncorrected. Conclusion An accurate diagnosis of anatomic pattern in mixed type TAPVC can be difficult to establish in all the patients before surgery. Detailed intraoperative assessment, individualized surgical approach, and aggressive perioperative management may reduce surgical mortality. Operative survivors have good midterm outcome.

Case report: Thoughts on two cases of total anomalous pulmonary venous connection complicated with pulmonary artery hypertension

“…The two examples in this article are the mixed TAPVC “3 + 1” type. It is simple to overlook one of them because of the varied position of pulmonary venous drainage ( 12 ). The main causes are the lack of a comprehensive and standard scan sequence, which ignores the scan of alternative ectopic drainage routes like the scan of the suprasternal fossa with or without vertical veins, and the content with detecting the common pulmonary venous cavity drainage channel.…”

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis

“…Mixed TAPVC: Mixed type TAPVC, which is complicated by more than 1 level of PV connection defect, is considered to be the most challenging type of TAPVC and sometimes requires combinations of surgical methods [ 28 ]. Few studies have reported clear conclusions as a result of the low morbidity (5–10% of patients with TAPVC); therefore, the reoperation rates of this type of TAPVC have not been clarified by large cohort studies [ 28 ]. It is difficult to pool surgical data from the published literature.…”