Rapid Development of a Large Systemic-to-Pulmonary Vein Fistula After Bidirectional Glenn Shunt and Successful Closure With an Amplatzer Duct Occluder
A 7-month-old boy presented with significant cyanosis 17 days after bidirectional cavopulmonary shunting (Glenn procedure). He had previously undergone a Norwood operation at 7 days of age for a double-outlet right ventricle with hypoplastic left ventricle, hypoplastic aortic arch, and coarctation. Cardiac catheterization 10 days before the Glenn operation showed a pulmonary venous wedge pressure of 16 mm Hg and an estimated pulmonary vascular resistance of 3 U · m2. Angiography demonstrated moderate right pulmonary artery stenosis and a 0.6 mm vein draining from the left end of the innominate vein into the posterior mediastinum (Figure, a).
The patient underwent a bidirectional Glenn shunting with placement of a right pulmonary artery patch. He was discharged home on postoperative day 8, with a systemic saturation of 82% on room air. At follow-up on postoperative day 17, he was in mild respiratory distress with a saturation of 60% on room air. Chest x-ray showed clear lung fields bilaterally. Contrast echocardiography with agitated saline injected in a scalp vein demonstrated rapid contrast return to the left atrium. A pulmonary perfusion scan with tracer injected in a scalp vein showed a right-to-left shunt with 60% of tracer passing to the body and 40% to the lungs.
Cardiac catheterization on the following day showed a superior caval pressure of 20 mm Hg and a systemic oxygen saturation of 73% (ventilated with 50% oxygen). Angiography showed a large, 6.4-mm venous fistula from the left end of the innominate vein to the left upper pulmonary vein (Figure, b). Through a shortened 5F delivery sheath placed in the left internal jugular vein, an 8/6 mm Amplatzer duct occluder was deployed in the distal fistula. A subsequent angiogram demonstrated complete occlusion of the vessel (Figure, c). Systemic oxygen saturation immediately improved to 83%, without an increase in superior vena cava pressure.
This case illustrates the rapid development of a large decompressing venous fistula within 17 days of bidirectional Glenn shunt. Contributing factors likely included the presence of a substrate vessel (a tiny vestigial levoatriocardinal vein) and high superior vena cava pressure.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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