The newborn can experience two types of differential cyanosis (DC). The common type of DC occurs when oxygen saturation in the right hand is greater than in the foot. The second type of DC, reversed differential cyanosis (RDC), occurs when oxygen saturation is lower in the right hand than in the foot. This phenomenon is observed in transposition of the great arteries (TGA) with patent ductus arteriosis (PDA) and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation. This report describes a case of RDC not previously described involving an infant with supracardiac total anomalous pulmonary venous connection (TAPVC). In supracardiac TAPVC, RDC results from streaming of highly saturated superior vena cava (SVC) blood into the right ventricle, out the main pulmonary artery, through a PDA, and to the descending aorta, with streaming of more desaturated blood from the inferior vena cava (IVC) into the left atrium across the atrial septal defect (ASD)/foramen ovale. Therefore, as part of a neonatal examination to rule out congenital heart disease (CHD), simultaneous pre- and postductal oxygen saturations should be documented. The presence of RDC should initiate immediate full cardiac evaluation for CHD. Supracardiac TAPVC should be included in the differential diagnosis if RDC is observed.
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Electronic Supplementary Material
MOESM1 [Subcostal sagittal echocardiographic sweep at the bicaval level demonstrating superior vena caval flow (red) toward the right atrium and directed to the left of the screen toward the tricuspid valve and right ventricle. The inferior vena cava is shown at the lower right of the screen. Color mapping demonstrates flow (blue) from the inferior vena cava directed toward the right atrium posterior and to the left of the flow from the superior vena cava (red). The blue-colored Doppler flow is observed to cross the atrial septal defect superiorly and rightward toward the left atrium] (MOV 2101 kb)
MOESM2 [Suprasternal frontal echocardiographic view presented in a split-screen format. The right of the screen shows color Doppler flow (red) arising from the left pulmonary veins and directed superiorly through an ascending vertical vein that connects with the left innominate vein. The latter drains into the superior vena cava at the right side (not shown)] (MOV 1574 kb)
MOESM3 [Right parasternal long-axis view with the transducer positioned at the right chest demonstrating flow from the right pulmonary veins (red Doppler flow signal) toward the superior vena cava. This part of the superior vena cava is at the junction with the right atrium (left lower part of the screen) such that flow (red) also is observed directed into the right atrium. The tricuspid valve is to the left of the right atrium. Part of the ascending aorta is seen above the superior vena cava] (MOV 1395 kb)
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Yap, S.H., Anania, N., Alboliras, E.T. et al. Reversed Differential Cyanosis in the Newborn: A Clinical Finding in the Supracardiac Total Anomalous Pulmonary Venous Connection. Pediatr Cardiol 30, 359–362 (2009) doi:10.1007/s00246-008-9314-0
- Reversed differential cyanosis
- Total anomalous pulmonary venous connection