Abstract
Detailed preoperative imaging of total anomalous pulmonary venous connection (TAPVC) is critical to ensuring adequate surgical planning and preoperative decision making. The purpose of this study was to describe the computed tomography findings of TAPVC and identify morphologic death risk factors. We conducted a retrospective study included 70 patients with TAPVC between May 2014 and June 2017 in Hunan Children’s Hospital. All available clinical data and computed tomography imaging were reviewed, and survival time was followed-up. Life Tables analysis was used to estimate survival rates. Patient survival was described with Kaplan–Meier curves. Cox Regression model was used to test the potential risk factors. TAPVC was subdivided into four types. Of 70 cases, 42 (60%) had supracardiac, 13 (18.6%) had cardiac, 8 (11.4%) had infracardiac, and 7 (10%) had mixed type. Pulmonary venous obstruction (PVO) was found in 30 (42.9%) of 70 patients in this group. Of all concurrent abnormalities, atrial septal defect (ASD) was the most common (98.6%), followed by patent ductus arteriosus (PDA; 31, 44.3%), and persistent left superior vena cava (PLSVC; 5, 7.1%). 1, 3, 6 and 12-month survival rates were 76, 61, 49, and 38% respectively. Risk factors for mortality in multivariable analysis comprised PVO, McGoon index (MGI), and mode of delivery. Various concurrent abnormalities and great morphological heterogeneity were observed in patients with TAPVC. Patients with TAPVC had a highest mortality in the neonatal period. PVO, smaller MGI and caesarean are important predictors for mortality.
Highlights
Total anomalous pulmonary venous connection (TAPVC) is a rare malformation of congenital heart disease in which all pulmonary veins connect directly to the systemic venous circulation, and not the left atrium [1]
TAPVC was subdivided into four types according to the drainage site of the pulmonary veins
In cardiac TAPVC, the confluent pulmonary vein (CPV) drained into the coronary sinus (CS) in ten patients
Summary
Total anomalous pulmonary venous connection (TAPVC) is a rare malformation of congenital heart disease in which all pulmonary veins connect directly to the systemic venous circulation, and not the left atrium [1]. Computed tomography angiography (CTA) is an accurate, noninvasive, economical diagnostic modality in preoperative evaluation of TAPVC [7, 8]. It helps to improve diagnostic accuracy and have significant advantages in accurately evaluating the courses of pulmonary venous connections [9, 10]. An understanding of complete anatomical details and physiological assessment are critical to medical and surgical management [5].The purpose of this study was to describe the CTA findings of TAPVC and identify morphologic death risk factors
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