Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology

Background:

• Total anomalous pulmonary venous connection (TAPVC) is a complex congenital heart defect requiring surgical repair.
• Long-term outcomes of TAPVC repair, considering different surgical techniques and anatomical variations, are crucial for patient management.

Purpose of the Study:

• To evaluate the late outcomes of non-isomeric TAPVC repair.
• To compare the effectiveness of different surgical techniques and analyze predictors of mortality and reintervention.

Main Methods:

• Retrospective analysis of 89 patients undergoing TAPVC repair between 1983 and 2001.
• Categorization by anatomical subtype (supracardiac, cardiac, infracardiac, mixed) and surgical approach (double-patch, side-to-side anastomosis, coronary sinus unroofing).
• Long-term follow-up (727.16 patient-years) to assess survival, reintervention rates, and identify outcome predictors.

Main Results:

• Early mortality was 7.86%, with 11.2% requiring reintervention for pulmonary venous (PV) stenosis.
• Late survival was 87.3% at 18 years, with associated anomalies and reoperation for PV stenosis predicting mortality.
• Preoperative obstruction increased the risk of PV stenosis reintervention, while the double-patch technique was linked to higher late arrhythmia rates.

Conclusions:

• Side-to-side anastomosis demonstrates excellent results for TAPVC repair.
• Left atrial enlargement techniques may be associated with an increased risk of late arrhythmias.
• Aggressive reintervention for PV obstruction is necessary, but intrinsic PV stenosis remains a significant predictor of adverse outcomes.