The ‘starfish’ sign: a novel sonographic finding with B‐flow imaging and spatiotemporal image correlation in a fetus with total anomalous pulmonary venous return

Abstract

Total anomalous pulmonary venous return (TAPVR) is a serious congenital anomaly that can lead to delivery of a critically ill newborn if not recognized during fetal life.1,2 Volpe et al.3 have described the inclusion of B-flow imaging using spatiotemporal image correlation (STIC) for fetuses with total anomalous pulmonary venous connection. We now wish to report another affected fetus that extends these original observations and further demonstrates the value of this methodology for detailed anatomic evaluation of complex congenital heart disease. A 19-year-old woman, gravida 1 para 0, was referred to our unit at 21 weeks’ gestation for an abnormal cardiac screening examination that included left cardiac axis deviation, ventricular septal defect (VSD), and a single overriding arterial outflow tract. In addition to her clinical examination, she agreed to participate in an imaging research protocol that was approved by the institutional review boards of William Beaumont Hospital and the Eunice Kennedy Shriver National Institute of Child Health and Human Development. Volume ultrasonography (Voluson E8, GE Healthcare, Milwaukee, WI, USA) demonstrated the following: (1) right-sided aortic arch; (2) large perimembranous VSD with overriding aorta; (3) pulmonary atresia; (4) large aortopulmonary collateral artery arising anteriorly from the descending aorta; and (5) poorly visualized pulmonary veins into the left atrium. Our differential diagnosis included tetralogy of Fallot with pulmonary atresia and truncus arteriosus. Anomalous pulmonary venous return was also considered because of the poorly visualized pulmonary veins into the left atrium. Four-dimensional ultrasonography with B-flow and STIC imaging were later used to demonstrate a confluence of pulmonary veins that drained into a vertical vein behind the left atrium, resembling a starfish in shape (Figure 1, Videoclip S1). Figure 1 4D ultrasonography, using B-flow imaging and spatiotemporal image correlation, demonstrating tetralogy of Fallot, pulmonary atresia and total anomalous venous return, from a left oblique view of the posterior heart. A confluence of pulmonary veins drains ... Amniocentesis results revealed an apparently unrelated partial duplication of the short arm of chromosome 16 (16p11.2 to 16p12), but not a 22q minus chromosomal anomaly. A male infant was delivered at term gestation. Neonatal echocardiography confirmed tetralogy of Fallot with pulmonary valve atresia, levocardia, and a right aortic arch. Situs solitus was noted without evidence of isomerism. An aortopulmonary collateral vessel ultimately supplied both pulmonary arteries and the ductus arteriosus was absent. Four pulmonary veins drained into a vertical vein that emptied into a left innominate vein. This vessel subsequently coursed beneath the aortic arch into a right superior vena cava. Postnatal surgery on day 3 included placement of a conduit that connected the right ventricle to the pulmonary artery, perimembranous VSD closure, and correction to total anomalous venous return. The infant had failure to thrive with persistent chylous pleural drainage and eventually expired at 5 months of age. B-flow technology digitally enhances signals from weak blood reflectors from vessels and, at the same time, suppresses strong signals from surrounding tissues.4 The method does not rely on Doppler ultrasonography to display blood flow, is angle-independent, and permits relatively high frame rates with excellent spatial resolution. This approach may be advantageous over color or power Doppler imaging when used in conjunction with STIC for the evaluation of fetal vasculature. Our findings extend those in the original case series of Volpe et al. of fetuses with TAPVR using B-flow imaging and STIC.3 This image demonstrates an unusual degree of anatomic detail for this complicated anomaly and now documents a starfish-like appearance of confluent pulmonary veins as a supracardiac form of TAPVR. In our experience, most prenatal cases of congenital heart disease can be adequately visualized using conventional 2D ultrasonography. Some complex heart anomalies, however, can be further characterized from the additional use of volume sonography with B-flow imaging.