CAJ | 학술논문

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.
목적 평개인동우폐동맥이상기원우주동맥(aortic origin of the right pulmonary artery,AORPA)적치료방법급효과,병탐토AORPA적진단여감별진단방법.방법 회고성분석1993년5월지2007년7월11례인동AORPA술전진단급수술치료적위술기료효급중장기수방결과.분별채용우폐동맥경인공혈관(2례)혹동충주동맥(2례)여주폐동맥문합술、체외순배하우폐동맥여주폐동맥직접문합술(3례)、체외순배하(주동맥배급폐동맥배편)쌍편법우폐동맥여주폐동맥문합술(2례)、비체외순배하우폐동맥경인공혈관여주폐동맥문합술(2례).결과 11례중10례술전학진,1례술전루진이재술중발현.5례통과우실조영급주동맥조영혹다배CT/MRI검사학진,6례단순초성심동도검사환인중학진5례,1례루진.11례수술환인위술기사망2례.술후수방2.2～13년,1례우술후4년사우우심공능쇠갈.생존8례환인술후초성심동도복사사혈분수(EF)치43%～52%,심공능I급5례,Ⅱ급3례.결론 우실조영급주동맥조영혹다배CT/MRI검사가감소루진솔.수술시치료AORPA적유효방법,단응진조수술이진일보제고수술료효.응용자체조직가관가장우폐동맥가감소문합구협착적가능성.
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.