Surgery for Pulmonary Venous Obstruction After Repair of Total Anomalous Pulmonary Venous Return

The major complication and the main cause of reoperation following surgery for total anomalous pulmonary venous return (TAPVR) is the occurrence of pulmonary venous obstruction. Outcomes after surgical repair of TAPVR have greatly improved in the last 10 years; however, the complex forms of TAPVR, particularly when associated with single ventricle and heterotaxy, still carry a high risk of failure. The pathology of pulmonary venous obstruction following TAPVR surgery is a fibrous intimal hyperplasia associated with some medial hypertrophy. There is an increasing severity in the spectrum of lesions from anastomotic stricture to pulmonary vein ostial stenosis and diffuse pulmonary vein stenosis. For anastomotic lesions, revision of the TAPVR anastomosis by patch enlargement of the left atrial anastomosis provides good results. On the contrary, conventional techniques in cases of pulmonary vein ostial stenosis had very poor results. The sutureless repair technique introduced in 1996 provides better midterm results than any other technique, with freedom from mortality and recurrence improving from 65% to 90%. The sutureless technique is described in detail, with emphasis on the need for resection of the pulmonary vein scar tissue and on the different techniques needed respectively on the right and the left side. In our experience, using an atraumatic technique at the initial repair has greatly decreased the occurrence of late pulmonary venous obstruction.