Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage

Background. Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy. Methods. Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques. Results. Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p< 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p< 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years’ follow-up. Stenting was uniformly unsuccessful. Conclusions. In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.