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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 …

Rapid Development of a Large Systemic-to-Pulmonary Vein Fistula After Bidirectional Glenn Shunt and Successful Closure With an Amplatzer Duct Occluder