Abstract

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and prenatal detection of TAPVC malformation remains a challenging. TAPVC can be easily missed or misdiagnosed in prenatal examinations. This study was aimed to use the modified vascular corrosion casting technique to prepare fetal cardiovascular casts with TAPVC and investigate the utility of cardiovascular casting for the demonstration of fetal TAPVC. The retrospective study enrolled twenty fetuses (22 to 29 + 4 gestational weeks) with TAPVC diagnosed by prenatal echocardiography and casting technique from May 2015 to May 2020. Pre- and postnatal medical records, including results obtained by prenatal ultrasound, postpartum computed tomography angiography, as well as anatomic and cardiovascular casting findings were carefully reviewed and analyzed. In twenty cases, 80% (16/20) had intra- or extracardiac malformations. The TAPVC types were supracardiac (n = 8), cardiac (n = 6), infracardiac (n = 4), and mixed (n = 2). The diagnosis of 1 case each of supracardiac and cardiac TAPVC was modified to partial anomalous pulmonary venous connection; additionally, 4 malformations were missed and 2 were misdiagnosed, including an anomalous left brachiocephalic vein in supracardiac TAPVC, abnormal inflow of the hepatic vein and a double inferior vena cava in infracardiac TAPVC; and bilateral ductus arteriosus in infracardiac TAPVC; a tetralogy of Fallot in cardiac TAPVC that was corrected to right ventricular double outlet; and an absence of ductus arteriosus that was misdiagnosed as slim ductus arteriosus. Comparing with ultrasound, casting technique has its own superiority in exhibiting TAPVC abnormalities, especially in certain types such as course, origin and absence abnormalities of ductus. Postpartum cardiovascular casts can accurately depict the branch structure of the heart’s larger vessels, and may be used as a clinical assessment and teaching method in complex cardiac malformations.

Highlights

  • Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and prenatal detection of TAPVC malformation remains a challenging

  • We retrospectively analyzed the clinical data of 20 cases of fetal TAPVC diagnosed by prenatal ultrasound, and compared the results of antenatal echocardiography with postpartum echocardiography, postpartum computed tomography angiography (CTA), and anatomic findings to establish the characteristics of different types of TAPVC and their venous return

  • In the early stage of pregnancy, fetal blood mostly circulates through the ductus arteriosus and only a small portion of pulmonary blood flows into the left atrium via the pulmonary veins (PVs); as no hemodynamic changes are observed at this time, diagnosing TAPVC is difficult

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Summary

Introduction

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and prenatal detection of TAPVC malformation remains a challenging. Total anomalous pulmonary venous connection (TAPVC) is a rare condition that accounts for 0.7%–1.5% of cases of congenital heart ­disease[1], with a reported incidence of approximately 7–9 per 100,000 in live ­births[2,3]. We retrospectively analyzed the clinical data of 20 cases of fetal TAPVC diagnosed by prenatal ultrasound, and compared the results of antenatal echocardiography with postpartum echocardiography, postpartum computed tomography angiography (CTA), and anatomic findings to establish the characteristics of different types of TAPVC and their venous return. In order to clearly display the 3D structure of fetal vessels, modified cardiovascular casts of some specimens were used for postpartum diagnosis

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