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Prenatal two‐ and three‐dimensional sonographic diagnosis of total anomalous pulmonary venous connection

Ultrasound in Obstetrics and Gynecology2007Vol. 30(5), pp. 788–789

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K. M. Law, Kwok Leung, Mónica Tang, Adolphus Kai-tung Chau

Abstract

We report the prenatal diagnosis of two cases of isolated total anomalous pulmonary venous connection (TAPVC) using two-dimensional (2D) echocardiography. In Case 1, a routine morphology ultrasound scan was performed on a 35-year-old woman, gravida 2, para 1, at 19 weeks' gestation. It showed dilatation of the right fetal heart, a ‘smooth’ appearance of the posterior wall of the left atrium in the four-chamber view, absence of a direct pulmonary venous connection to the left atrium, presence of a pulmonary venous confluence behind the atrium together with a wide separation between it and the descending aorta, and visualization of an additional vein on the left side of the pulmonary trunk in the three-vessel view. Color Doppler ultrasound examination showed turbulent flow in the drainage of this additional ascending vein into the left innominate vein. A diagnosis of TAPVC was made and termination of the pregnancy was performed at 20 weeks' gestation. Postmortem examination of the fetus confirmed the antenatal sonographic findings. In Case 2, an anomaly ultrasound scan was performed on a 35-year-old woman, gravida 2, para 1, because of a fetal nuchal translucency of 4 mm measured at 11 weeks' gestation, despite a normal karyotype. Due to the normal appearance of the fetal heart on echocardiographic ultrasound examination at 20 weeks' gestation, no diagnosis of TAPVC was made. Follow-up ultrasound examination at 27 weeks' gestation showed abnormal findings similar to those of Case 1 (Figure 1a). The three-vessel view showed the presence of an additional vein to the right of the prominent superior vena cava (Figure 1b). At a higher level, this additional vein became posterior to the superior vena cava and eventually coursed anteriorly to drain into it near its junction with the right innominate vein. Color Doppler ultrasound examination showed a continuous velocity with loss of biphasic pattern. A further follow-up scan at 32 weeks' gestation revealed two balloon-like chambers behind the left atrium (Figure 1c). The baby was delivered at 35 weeks' gestation, and underwent emergency surgical repair. The antenatal echocardiographic findings were confirmed. Unfortunately, the baby did not survive. Two-dimensional echocardiographic images of total anomalous pulmonary venous connection to the superior vena cava (Case 2). (a) Oblique four-chamber view showing the pulmonary venous confluence (pvc) behind, but not connecting to, the left atrium (la). (b) Three-vessel view showing an ascending vertical vein (avv) to the right of the superior vena cava (svc). (c) Four-chamber view showing the ‘double balloon’ sign, which is formed by the pulmonary venous confluence and the aorta. ao, aorta; pa, pulmonary artery; ra, right atrium; rv, right ventricle. Three-dimensional (3D) sonography, including multiplanar (three orthogonal planes)1 and multi-slice (multiple sequential parallel planes)2 images, was useful in the demonstration of the complex abnormal anatomy associated with TAPVC (Figure 2), although 2D images seem to be just as useful as 3D images. Segmental evaluation of the cardiac structures is required to make a diagnosis of TAPVC3. In obstructed TAPVC, an ascending or descending venous channel may be small and difficult to visualize because of consequently diminished pulmonary blood flow4. The use of 3D images can facilitate the segmental evaluation. Three-dimensional ultrasound images of total anomalous pulmonary venous connection to the superior vena cava (svc) near its insertion into the innominate vein (Case 2). (a) Multi-slice images showing an absence of pulmonary veins entering into the left atrium (la) at a lower level, the presence of the pulmonary venous confluence (pvc) behind but not connecting to the left atrium, and insertion of the ascending vertical vein (avv) into the svc at a higher level. (b) Multiplanar image showing the insertion of the ascending vertical vein into the svc near its junction with the right innominate vein (riv) in cross-section in the A-plane and in longitudinal section in the B-plane. (c) Multiplanar image with color Doppler mapping showing the absence of high blood flow in the pvc. ao, aorta; lv, left ventricle; pa, pulmonary artery; ra, right atrium; rv, right ventricle. The 2D sonographic findings associated with TAPVC have been well documented3-5. In addition, dilatation of the right atrium is prominent6. The posterior border of the left atrium appears more ‘smooth’ than usual, probably due to the absence of a pulmonary venous connection. In the cases we report, the three-vessel view allowed us to visualize the anomalous vein and trace its connections. However, as in Case 2, not all cases of TAPVC will show abnormal findings in early pregnancy4. The ‘double balloon sign’7 was not present until the third trimester. K. M. Law*, K. Y. Leung , M. H. Y. Tang , A. K. T. Chau?, * Department of Obstetrics and Gynaecology, Queen Elizabeth Hospital, Hong Kong, Department of Obstetrics and Gynaecology, Queen Mary Hospital, University of Hong Kong, Hong Kong, Department of Prenatal Diagnostics, Tsan Yuk Hospital, Hong Kong, ? Department of Paediatric Cardiology, Grantham Hospital, Hong Kong

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