Abstract

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7months (range 7-14months) and the mean body weight was 7.9 ± 2.6kg (range 4.1-10.5kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.

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