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

Guido Michielon, Roberto M. Di Donato, Luciano Pasquini, Salvatore Giannico, Gianluca Brancaccio, Ennio Mazzera, Cosimo Squitieri, Glauco Catena

Research output: Contribution to journalArticlepeer-review


Objectives: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. Methods: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). Results: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3±0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7±0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). Conclusions: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.

Original languageEnglish
Pages (from-to)184-191
Number of pages8
JournalEuropean Journal of Cardio-thoracic Surgery
Issue number2
Publication statusPublished - 2002


  • Pulmonary vein stenosis
  • Total anomalous pulmonary venous connection

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery


Dive into the research topics of 'Total anomalous pulmonary venous connection: Long-term appraisal with evolving technical solutions'. Together they form a unique fingerprint.

Cite this