Total anomalous pulmonary venous connection: preoperative anatomy, physiology, preoperative evaluation, surgical considerations and single centre clinical experiences

Abstract

Objectives: The aim of the present study was to document the anatomical subtypes of total anomalous pulmonary venous connection (TAPVC) since it is critical in the surgical and medical management of this cardiac anomaly. Methods: This retrospective study was conducted at a paediatric heart centre between February 2019 and December 2021. The study included 27 patients who underwent intracardiac repair of total anomalous pulmonary venous connection. Results: The mean age of the patients were found 159.37±411.29 (range: 7.00−2160) days, the mean weight of patients were 4756.67±2988.78 grams. Mean oxygen saturation at the arrival of the hospital was 84.89±7.98 (range: 60−96) %. Mortality was seen in 7 (25.9%) patients. 2 of 7 patients (28.6%) had supracardiac type, 2 of 7 patients had (28.6%) cardiac type, 2 of 7 (28.6%) patients had infracardiac type and 1 of 7 patients (14.3%) had mixt type anomaly. Only 3 (11.1%) patients needed ECMO. One of these 3 patients (33.3%) had supracardiac type, and the remaining 2 of 3 patients (66.7%) had infracardiac type anomaly. Conclusion: Total anomalous pulmonary venous connection is a rare cardiac anomaly seen in 7/100,000 live births. It constitutes approximately 1% of all congenital heart diseases. It can be found as an isolated cardiac defect or accompany other diseases; usually accompanying cardiac lesions are associated with the severity of the disease and poor prognosis. Understanding the anatomical subtypes of TAPVC is critical in the surgical and medical management of this disease.

Keywords

• cardiac anomaly
• pulmonary venous hypertension
• total anomalous pulmonary venous connection

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