Lung transplantation for pulmonary lymphangioleiomyomatosis:a report of 9 cases / 中华胸心血管外科杂志

ABSTRACT

Objective To investigate the selection of recipients,operative technique, and perioperative management of lung transplantation.Methods From June 2013 to September 2016, 9 patients with pulmonary lymphangioleiomyomatosis un-derwent lung transplantation at Affiliated Wuxi People's Hospital.There were 9 females aged from 23 to 52 years.Chest en-hancement of CT in 9 patients prior to transplantation suggests multiple different sized thin-walled cystic shadows in whole lung. Nine patients received bilateral sequence lung transplantation ( BSLT) .The pathological findings after the operation indicated that cells of the abnormal proliferation of smooth muscle diffused around the bronchi , vessels and lymphatics , and widened the alveolar septum, which was consistent with the pathological changes in the patients with PLAM .We analyzed the differences of oxygenation index, forcedexpiratory volume at 1 sec(FEV1), and DLCO％ pred.Using paired t test to compare these indica-tors, P<0.05 was considered statistically significant.Results The oxygenation index was increased from(210 ±55) mmHg (1 mmHg=0.133 kPa) to(400 ±80)mmHg after treatment, the difference was statistically significant(P<0.05).The FEV1 was increased from(1.0 ±0.2)L to(2.8 ±0.4)L, and the difference was statistically significant(P<0.05).The DLCO％pred was increased from(27.5 ±2.5)％ to(75.0 ±10.0)％, and the difference was statistically significant(P<0.05).Fi-nally, 8 patients were discharged from the hospital, and the pulmonary function was good.1 patients died of infection and acute rejection on the 13 day after operation.Conclusion Lung transplantation is effective in the treatment of chronic thromboem-bolic pulmonary hypertension , and long-term survival requires further investigations .Lung transplantation is the only effective means for the treatment of end-stage pulmonary lymphangioleiomyomatosis .Bilateral sequence lung transplantation is the first choice.