Murat Çiçek1, Oktay Korun1, Okan Yurdakök1, Hüsnü Fırat Altın1, Mehmet Akif Önalan1, Mustafa Orhan Bulut2, Filiz İzgi Çoşkun3, Ahmet Şaşmazel1, Fatih Özdemir1, Numan Ali Aydemir1

1Department of Pediatric Heart Surgery, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey
2Department of Pediatric Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey
3Department of Anesthesiology and Reanimation, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey

Keywords: Congenital heart disease; mortality; outcome; pulmonary vein

Abstract

Introduction: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic.

Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records.

Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days-9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospi- tal stay was 12 (IQR: 7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p< 0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p< 0.00001). Three patients were reoperated due to postoperative pulmonary venous stenosis.

Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.