Lung transplant is a viable treatment option for patients with congenital and acquired pulmonary vein stenosis.

Congenital pulmonary vein stenosis (PVS) is associated with high mortality because surgical repair is usually not feasible or is ineffective. In addition, acquired PVS after repair of congenital heart disease is a potential complication that occurs in 5% to 10% of patients and carries a poor prognosis. Lung transplantation has been proposed as a viable option. However, long-term outcomes after lung transplant in these patients remain unknown. This was a retrospective review of prospectively maintained database. Between 1990 and 2010, 20 patients (12 girls, 8 boys) with PVS underwent transplantation. Of these, 8 had acquired stenosis from prior repair for total anomalous pulmonary venous return and 1 from atrioventricular canal repair. The median waiting time was 26 days. The mean age at transplant was 1.1 ± 0.89 years, and 16 of the 20 patients were white. All patients received bilateral lung transplants on cardiopulmonary bypass. Four patients (20%) were receiving extracorporeal membrane oxygenation (ECMO) support before transplant, and 3 (15%) required ECMO after transplant due to graft dysfunction. The mean intensive care unit stay was 33.5 ± 29.1 days, and the mean hospital stay was 58.7 ± 43.5 days. The 30-day mortality was 10%. ECMO support in the peri-operative period was the main predictor of 30-day and 1-year mortality (hazard ratio, 3.6; p = 0.01). The overall 5-year survival of the entire cohort was 59.8% (67.3% congenital vs 50.7% acquired). The predominant cause of long-term mortality was bronchiolitis obliterans. The 5-year bronchiolitis obliterans-free survival was 48% (57.2% congenital vs 41% acquired). Lung transplant is a viable treatment option for PVS, particularly for patients with diffuse disease or failed surgical correction.

Bharat A ，Epstein DJ ，Grady M ，Faro A ，Michelson P ，Sweet SC ，Huddleston CB ... -  《-》

Dismal lung transplant outcomes in children with tetralogy of Fallot with pulmonary atresia compared to Eisenmenger syndrome or pulmonary vein stenosis.

Grady RM ，Gandhi S ，Sweet SC ，Mao J ，Huddleston CB ... -  《-》

Extracorporeal membrane oxygenation as a bridge to lung transplant: midterm outcomes.

Extracorporeal membrane oxygenation (ECMO) is used occasionally as a bridge to lung transplantation. The impact on mid-term survival is unknown. We analyzed outcomes after lung transplant over a 19-year period in patients who received ECMO support. From March 1991 to October 2010, 1,305 lung transplants were performed at our institution. Seventeen patients (1.3%) were supported with ECMO before lung transplant. Diagnoses included retransplantation (n = 6), pulmonary fibrosis (n = 6), cystic fibrosis (n = 4), and chronic obstructive pulmonary disease (n = 1). Fifteen patients underwent double lung transplant, one patient had single left lung transplant and one patient had a heart-lung transplant. Venovenous and venoarterial ECMO were implanted in eight and nine cases, respectively. Median duration of support was 3.2 days (range, 1 to 49 days). Mean patient follow-up was 2.3 years. Thirty-day, 1-year, and 3-year survivals were 81%, 74%, and 65%, respectively, for the supported patients and 93%, 78%, and 62% in the control group (p = 0.56). Two-year survival was not affected by ECMO type, with survival of five out of nine patients supported by venoarterial ECMO vs seven out of eight patients supported by venovenous ECMO (p = 0.17). At 1- year follow-up, allograft function for the ECMO-supported patients did not differ from the control group (forced expiratory volume in one second, 2.35 L vs 2.09 L, p = 0.39) (forced vital capacity, 3.06 L vs 2.71 L, p = 0.34). Extracorporeal membrane oxygenation as a bridge to lung transplantation is associated with higher perioperative mortality but acceptable mid-term survival in carefully selected patients. Late allograft function did not differ in patients who received ECMO support before lung transplant from those who did not receive ECMO.

Bermudez CA ，Rocha RV ，Zaldonis D ，Bhama JK ，Crespo MM ，Shigemura N ，Pilewski JM ，Sappington PL ，Boujoukos AJ ，Toyoda Y ... -  《-》

Surgical repair of pulmonary venous stenosis: a word of caution.

Pulmonary vein stenosis (PVS), both congenital and acquired, is challenging to treat surgically with uncertain long-term results. We reviewed an 11-year surgical experience in 52 children. From 2002 to 2012, 52 children age 0 days to 13 years (mean 1.9 years, median 11.7 months) weighing 2.2 to 32.5 kg (mean 9.3 kg, median 7.6 kg) had surgical relief of PVS. Based on clinical characteristics or complexity, 33 (63%) had a sutureless pericardial well repair and 19 (37%) had a more standard patch repair. There were no significant differences in clinical characteristics between the 2 techniques. Twenty children (38%) had prior anomalous pulmonary vein repair and 8 had primary pulmonary vein stenosis; 26 (50%) had other operations at the time of PVS relief. There were 2 hospital deaths (10.5%) in the "standard" group and 5 (15.2%) in the sutureless group (p>0.99). Despite postoperative evidence of PVS relief by echocardiogram or cardiac cath in all patients, at 5 years, actuarial freedom from PVS recurrence or death in the hospital survivors was 67% in the standard group and 58% in the sutureless group. Most recurrences or deaths occurred within 6 months of operation. Heterotaxy, single ventricle anatomy, bilateral disease, and previous anomalous pulmonary vein repair were not predictors of failure. Surgical treatment of pulmonary vein stenosis remains a challenging problem with nontrivial early mortality and ongoing risk for recurrence or death regardless of surgical technique employed. Clearly, development of methods for earlier intervention or detection and improved surgical techniques are warranted.

Kanter KR ，Kirshbom PM ，Kogon BE 《-》

Replacing cardiopulmonary bypass with extracorporeal membrane oxygenation in lung transplantation operations.

Bittner HB ，Binner C ，Lehmann S ，Kuntze T ，Rastan A ，Mohr FW ... -  《EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY》