Supracardiac Total Anomalous Pulmonary Venous Return (TAPVR)

Clinical history: A term newborn infant developed profound respiratory distress shortly after birth. 

Image 1: CTA coronal oblique images through the pulmonary veins. All four pulmonary veins bypass the left atrium and drain into a common vein that comes to a focal stenosis (arrow) before coursing superiorly and joining the left brachiocephalic vein. There is a dual lumen ECMO cannula in the right atrium (arrow head). Note is made of a nasogastric tube, an umbilical venous catheter and bilateral hypoaeration atelectasis.

Image 2: CTA coronal oblique images through the pulmonary veins. All four pulmonary veins bypass the left atrium and drain into a common vein that comes to a focal stenosis (arrow) before coursing superiorly and joining the left brachiocephalic vein. There is a dual lumen ECMO cannula in the right atrium (arrow head). Note is made of a nasogastric tube, an umbilical venous catheter and bilateral hypoaeration atelectasis.

Image 3: CTA 3D reconstructed images of the heart and great vessels. The dual lumen ECMO cannula courses within the superior vena cava (arrow head), to the right atrium with the tip at the inferior cavo-atrial junction. The four pulmonary veins drain into a common vein that comes to a focal stenosis (arrow) before heading superiorly over the pulmonary artery and aorta, and draining into the left innominate vein, eventually emptying into the superior vena cava and right atrium. Note is made of a nasogastric tube.

Image 4: CTA 3D reconstructed images of the heart and great vessels. The dual lumen ECMO cannula courses within the superior vena cava (arrow head), to the right atrium with the tip at the inferior cavo-atrial junction. The four pulmonary veins drain into a common vein that comes to a focal stenosis (arrow) before heading superiorly over the pulmonary artery and aorta, and draining into the left innominate vein, eventually emptying into the superior vena cava and right atrium. Note is made of a nasogastric tube.

Cine Clip: 3D Cine CTA shows all four pulmonary veins draining into the common vein that comes to a focal stenosis before heading superiorly over the pulmonary artery and aorta, and draining into the left innominate vein, eventually emptying into the superior vena cava and right atrium.

Discussion: After a negative echocardiogram (images not shown), venovenous ECMO (arrowheads) was performed. A cardiac CTA was requested, but complicated by the fact that the patient was on ECMO. The cardiac CTA was performed under the careful coordination of the ICU team, respiratory therapist and ECMO perfusionist while briefly suspending ECMO therapy during the scan.

The CTA revealed all four pulmonary veins formed a common vein that tapered to a short segment obstructing stenosis before draining into the right superior vena cava (video, arrows) consistent with supracardiac total anomalous pulmonary venous return (TAPVR).

Diagnosis: Supracardiac total anomalous pulmonary venous return (TAPVR)

The patient underwent immediate surgical repair with subsequent recovery. This illustrates an unusual case of obstructing Type 1 TAPVR, whose imaging was complicated by ECMO therapy.

TAPVR is the embryologic failure of connection between the pulmonary veins and left atrium that results in abnormal connection of the pulmonary veins to the right atrium resulting in a left to right shunt. An atrial septal defect of varying size is present resulting in an admixture lesion.

The three types are:
• Type 1 Supracardiac TAPVR: Pulmonary veins come together to form a “vertical vein” which joins the left innominate vein before draining into the right atrium via the superior vena cava.
• Type 2 Cardiac TAPVR: Pulmonary veins drain directly into the right atrium or coronary sinus.
• Type 3 Infracardiac TAPVR: Pulmonary veins drain downward as a large inferior draining vein connecting to the portal venous system, draining through the vascular bed of the liver and entering the right atrium from the hepatic veins.

References:
1. Friedman BA, Schoepf UJ, Bastarrika GA, Hlavacek AM. Computed tomographic angiography of infants with congenital heart disease receiving extracorporeal membrane oxygenation. Pediatr Cardiol. 2009 Nov;30(8):1154-6. Epub 2009 Jul 28.
2. Kim TH et al: Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol. 175(5):1381-6, 2000

James Chang, MD and Dorothy Gilbertson, MD, University of Arizona, University Medical Center, Department of Radiology