Multiple Color Doppler Flow Jets Into The Atria in a Child With Heterotaxy Syndrome

Abstract

A nine-month-old girl (weight: 7.92 kg; height: 70 cm) was taken to the operating room for the creation of a bilateral bidirectional Glenn shunt and atrial septectomy. Patent ductus arteriosus stenting had been done when the child was four days old. Transthoracic echocardiography showed heterotaxy (left atrial isomerism) with situs inversus, discordant atrioventricular and concordant ventriculoarterial connections (ventricular ‘noninversion’), a large ventricular septal defect, membranous pulmonary atresia with a hypoplastic main pulmonary artery, confluent branch pulmonary arteries, aorta anterior and to the right of the pulmonary artery, and bilateral superior vena cava with no bridging vein. The left superior vena cava was draining into the left-sided atrium (morphologic right atrium), while the right superior vena cava was draining into the same atrium via a massively dilated coronary sinus. The inferior vena cava was interrupted, with hemzygous continuation to the left superior vena cava. A computerized tomography scan revealed a right-sided stomach with a midline liver, and demonstrated the complex vascular communications (Fig 1, A and B). Transesophageal echocardiography confirmed the preoperative diagnosis (Fig. 2, Fig. 3; Videos 1 and 2). Color- flow Doppler mapping in the mid-esophageal view demonstrated multiple jets into both the atria and the right-sided ventricle (Fig 4, A and B; Videos 3 and 4). Pulse-wave Doppler of a dominant jet revealed a biphasic flow (Fig 5; Video 5). The intramyocardial origin of the jets was traced toward the aorta (Fig 6; Video 6). Fig. 2The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map showing demonstrating the anatomic abnormality in the child. View Large Image Figure Viewer Download Hi-res image Fig. 3The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map demonstrating the left superior vena cava. Left SVC, left superior vena cava. View Large Image Figure Viewer Download Hi-res image Fig. 4(A) The midesophageal transesophageal echocardiography views with color-flow Doppler map demonstrating jets (white arrows) into the left atrium. (B) The right atrium and the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 5The midesophageal pulse-wave Doppler signal interrogation of the jet by transesophageal echocardiography. View Large Image Figure Viewer Download Hi-res image Fig. 6The midesophageal transesophageal echocardiography view displaying the color-flow Doppler mapping delineating the origin of the jet (white arrows). View Large Image Figure Viewer Download Hi-res image