儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.

The diagnosis and surgical treatment of 11 cases of pediatric aortic origin of the right pulmonary artery

Objective To evaluate the therapeutic efficacy of pediatric aortic origin of the right pulmonary artery(AORPA)by surgical procedures,and to explore the diagnosis and differential diagnosis of AORPA.Methods Between May 1993 and March 2007,the preoperative diagnosis,perioperative therapeutic effects and results of mid or long term follow-up of 11 cases with AORPA who were diagnosed in Anzhen Hospital were retrospectively analyzed.Eleven patients underwent 4 different surgical procedures as follows:direct anastomosis of right pulmonary artery(RPA)and main pulmonary artery(MPA)under cardiopulmonary bypass(3 cases),double flap technique of anastomosis of RPA and MPA under cardiopulmonary bypass(2 cases),conduit of MPA to RPA by vascular prosthesis or autologous aorta under cardiopulmonary bypass(2 cases respectively),and conduit of MPA to RPA by vascular prosthesis not under cardiopulmonary bypass(2 cases).Results Ten of the 11 cases were diagnosed preoperatively,and one was diagnosed during operation.Five patients accepted the examination of right ventriculography,aortic angiography or multi-sliced CT or MRI,by which definite diagnosis was made.For those who only accepted echocardiography,5 got diagnosis while 1 was misdiagnosed.Two died during the perioperative period due to low output syndrome or pulmonary hypertension crisis.During the follow-up period of(5.4±4.6 yrs),one case died from right cardiac failure 4 yrs after the surgical procedure.Of the 8 cases surviving from AORPA,5 were in NYHA class I,and 3 in class II.Conclusions It is necessary to adopt right ventriculography and aortic angiographies or multi-sliced CT or MRI to avoid misdiagnosis.The perioperative and mid or long term efficacies of the surgical treatment of AORPA were good,but it was imperative to undergo surgical procedure as early as possible to promote the curative efficacy.Lengthening and widening RPA with autologous tissue could lessen the incidence of stenosis at the anastomotic site.