The clinical data of patients with total anomalous pulmonary venous connection who underwent repair in our centre in the past 13 years were reviewed. In this study, we systemically reviewed our experience in the optimal surgical strategy for patients with total anomalous pulmonary venous connection, aiming to provide evidence for clinical decision-making.

From January 1, 2009, to December 31, 2021, 122 patients undergoing surgical treatment for total anomalous pulmonary venous connection in our hospital were enrolled. Among them, 18 patients with single ventricle repair were excluded from the study. Multivariate analysis was used to determine the risk factors for early and late death and the risk factors for pulmonary vein obstruction.

There were 64 males and 40 females. The median age at surgery was 107 days (range, 25 days–788 days), the median weight at surgery was 4.8 kg (range, 3 kg–22 kg), and the median follow-up was 59 months (range, 0–150 months). Seven patients died early after surgery and six died late after discharge. Multivariable analysis indicated that prolonged cardiopulmonary bypass time was the only independent risk factor for early postoperative mortality. Multivariate analysis did not identify risk factors for late death. Emergency surgery, preoperative moderate and severe pulmonary hypertension, and prolonged cardiopulmonary bypass time were independent risk factors for postoperative pulmonary vein obstruction.

Early and long-term late outcomes of repair in patients with total anomalous pulmonary venous connection have been encouraging. Postoperative pulmonary vein obstruction remains a major problem for specialists worldwide. Pulmonary vein obstruction should be considered in children with preoperative emergency surgery, moderate to severe pulmonary hypertension and prolonged cardiopulmonary bypass time, and regular follow-up is necessary.

Management of total anomalous pulmonary venous connections has been extensively studied to further improve outcomes. Our institution previously reported factors associated with mortality, recurrent obstruction, and reintervention. The study purpose was to revisit the cohort of patients and evaluate factors associated with reintervention, and mortality in early and late follow-up.

A retrospective review at our institution identified 81 patients undergoing total anomalous pulmonary venous connection repair from January 2002 to January 2018. Demographic and operative variables were evaluated. Anastomotic reintervention (interventional or surgical) and/or mortality were primary endpoints.

Eighty-one patients met the study criteria. Follow-up ranged from 0 to 6,291 days (17.2 years), a mean of 1263 days (3.5 years). Surgical mortality was 16.1% and reintervention rates were 19.8%. In re-interventions performed, 80% occurred within 1.2 years, while 94% of mortalities were within 4.1 months. Increasing cardiopulmonary bypass times (p = 0.0001) and the presence of obstruction at the time of surgery (p = 0.025) were predictors of mortality, while intracardiac total anomalous pulmonary venous connection type (p = 0.033) was protective. Risk of reintervention was higher with increasing cardiopulmonary bypass times (p = 0.015), single ventricle anatomy (p = 0.02), and a post-repair gradient >2 mmHg on transesophageal echocardiogram (p = 0.009).

Evaluation of a larger cohort with longer follow-up demonstrated the relationship of anatomic complexity and symptoms at presentation to increased mortality risk after total anomalous pulmonary venous connection repair. The presence of a single ventricle or a post-operative confluence gradient >2 mmHg were risk factors for reintervention. These findings support those found in our initial study.

Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients.

A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed.

Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found.

We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.