Diagnosis and Management of the Unligated Vertical Vein in Repaired Total Anomalous Pulmonary Venous Connection

Abstract

During initial repair of supracardiac total anomalous pulmonary venous connection (TAPVC), the vertical vein (VV) is sometimes left patent (not ligated or divided) in the hope that this strategy may reduce the likelihood or severity of postoperative pulmonary hypertensive crises. We report a case of a 35-year-old pregnant patient with previously repaired supracardiac TAPVC who presented with atrial arrhythmia and right heart dilation. A cardiac magnetic resonance imaging study confirmed the diagnosis of patency of the vertical vein and right heart dilation. The VV was occluded with a catheter-delivered vascular occlusion device through a percutaneous approach, resulting in resolution of right heart dilation and arrhythmia. This case highlights the role of cross-sectional imaging as an adjunct to echocardiography in adults with repaired congenital heart disease.

Keywords

adult congenital heart disease total anomalous pulmonary venous connection unligated vertical vein cardiac MRI

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