Abstract
Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following repair of total anomalous pulmonary venous connection (TAPVC). However, surgical repair of neonatal TAPVC remains associated with a high risk of postoperative mortality and pulmonary venous obstruction (PVO). We conducted this retrospective study to identify risk factors associated with surgical outcomes in the neonatal population.Methods: A retrospective review was conducted for all 127 neonates who underwent operations for isolated TAPVC from January 2009 to January 2019.Results: Preoperative PVO occurred in 33 (26.0%) of the 127 patients. Fifty patients (39.4%) required tracheal intubation before the operation. Twenty-three patients (18.1%) underwent emergency surgery. There were 11 (8.7%) early deaths. Significant risk factors were prolonged cardiopulmonary bypass (CPB) time (p = 0.013) and increased postoperative central venous pressure (CVP, p = 0.036). There were 5 (4.3%) late deaths within 1 year of repair. The risk factors for overall death were preoperative acidosis (p = 0.001), prolonged CPB time (p < 0.001) and increased postoperative CVP (p = 0.007). In particular, mortality was significantly higher (p = 0.007) with a postoperative CVP > 8 mmHg. With an increase in use of sutureless techniques (p = 0.001) and decrease in deep hypothermic circulatory arrest (p = 0.009) over the past 5 years, postoperative mortality greatly decreased (21.2%: 6.7%, p = 0.016). Postoperative PVO occurred in 15 patients (11.8%). Risk factors were mixed TAPVC (p = 0.037), preoperative acidosis (p = 0.001) and prolonged CPB time (p = 0.006).Conclusion: Although postoperative mortality of neonatal TAPVC has dropped to 6.7% over the past 5 years, it is still relatively high. Risk factors for postoperative death include preoperative acidosis, prolonged CPB time and increased postoperative CVP. Mortality was significantly higher for neonates with an average CVP > 8 mmHg 24 h after surgery.
Highlights
Total anomalous pulmonary venous connection (TAPVC) is a rare but critical cardiac anomaly, accounting for approximately 2% of congenital heart disease cases [1]
16 patients had vertical vein stenosis, 6 had stenosis at the common junction of pulmonary veins, 3 had stenosis caused by a restrictive atrial septal defect, 3 had stenosis where the vertical vein passed through diaphragm, 4 had stenosis caused by compression of the pulmonary veins by the aorta or pulmonary artery, and 1 had common pulmonary vein stenosis
Postoperative PVO occurred in 15 patients (11.8%), 8 of whom suffered from anastomotic obstruction, 4 from anastomotic and branch pulmonary vein obstruction, and the other 3 from branch pulmonary vein obstruction
Summary
Total anomalous pulmonary venous connection (TAPVC) is a rare but critical cardiac anomaly, accounting for approximately 2% of congenital heart disease cases [1]. As reported from various research centers, newborns have a significantly higher risk of postoperative mortality and pulmonary vein obstruction (PVO), and are one of the TAPVC subgroups with the worst prognosis [6,7,8]. This study was a retrospective analysis designed to identify risk factors associated with postoperative death and PVO in all neonatal patients who underwent TAPVC repair in our center in recent years. Recent developments in surgical techniques and hospital care have led to improved outcomes following repair of total anomalous pulmonary venous connection (TAPVC). Surgical repair of neonatal TAPVC remains associated with a high risk of postoperative mortality and pulmonary venous obstruction (PVO). We conducted this retrospective study to identify risk factors associated with surgical outcomes in the neonatal population
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