肺或心肺联合移植治疗艾森曼格综合征的初探

Lung and heart-lung transplantation for Eisenmenger syndrome:report of 5 cases

目的探讨肺移植及心肺联合移植治疗艾森曼格综合征的手术适应证和围手术期处理。方法 2002年9月至2006年3月共收治5例先天性心脏畸形伴艾森曼格综合征(Eisenmengersyndrome,ES)患者,其中先天性心脏畸形房间隔缺损2例,室间隔缺损2例,均进行了心脏畸形修补及同期右肺移植;另有1例为先天性心脏病房间隔缺损,采用介入房缺封堵后发生全心功能衰竭,紧急行心肺联合移植。结果 4例心脏畸形修补同期单肺移植中有1例术后20 d 死于原发性移植物失功;1例心肺联合移植术后23 d 因原发性移植物失功死亡。3例患者康复出院,现已分别存活5、8、22个月,生活质量良好。患者术后肺动脉压明显下降、血气分析结果改善明显。结论心脏畸形修补同期单肺移植能有效治疗先天性心脏畸形伴艾森曼格综合征,匹配的供肺、良好的供肺切取和保护、完善的术后处理是患者术后存活的关键。

Objective To discuss the operative indications and peri-operational management of single lung transplantation （SLT） and heart-lung transplantation （HLT） for patients suffering from Eisenmenger＇s syndrome （ES）. Methods From September. 2002 to March. 2006, four ES patients, 1 males and 3 females, aged 16 ~ 19, 2 with atrial septal defect and 2 with ventricular septal defect, underwent single right lung transplantation, and one patients, male, aged 43, who suffered heart failure secondary to an interventional occlusion for atrial septal defect underwent cardiac repair and HLT. Results Two patients, including the patient who received HLT and one, female, aged 15, who received SHT died of he died primary graft dysfunction on the 23 rd day and 20^th day post-operationally respectively. While the other 3 recipients kept a normal life for 5, 8, and 22 months respectively after operation with apparently decreased pulmonary artery pressure and improved cardiac-pulmonary functions. Conclusion Lung transplantation companied with cardiac repair is effective in the therapy of ES. Donor-recipient matching in size, proper removal and protection of the donor lung, and appropriate postoperational management are the key procedures for those patients to survive.