A novel percutaneously adjustable device for ligature of the vertical vein in the setting of obstructive totally anomalous pulmonary venous connection

Abstract

A patent vertical vein might be desirable in patients with obstructive totally anomalous pulmonary venous connection with pulmonary hypertension, in order to decrease perioperative pulmonary arterial pressure and avoid pulmonary hypertensive crises. A subset of patients with an unligated vertical vein requires interruption of the vein due to the development of significant left-to-right shunt and right heart failure. We describe here a new device, permitting adjustable ligation of the vertical vein, which permits us to avoid multiple reoperations. In five patients, aged 2, 4, 3, 4, and 3 months respectively, and undergoing rechannelling of totally anomalous pulmonary venous connection with an unligated vertical vein, were treated with a device permitting adjusted ligation of the vertical vein over the course of postoperative congestive cardiac failure. There was no early or late death. Postoperatively, all ligatures were tightened gradually over a period of 24 to 96 hours, maintaining stable haemodynamics. At a mean follow-up of 55.40 months, there was no evidence of congestive heart failure in any patient, the clinical risk score varying from zero to 2, and no requirement of anti-failure medications. Computed tomographic angiograms during follow-up revealed absence of flow through the vertical vein, and ruled out distortion of the left upper pulmonary and left brachiocephalic veins. Use of a percutaneously adjustable device to ligate the vertical vein allows gradual tightening or loosening of the ligature under optimal physiologic conditions, without re-opening the sternum, or having to resort to another thoracotomy once the reactive components of pulmonary hypertension disappear.