CAJ | 학술논문

目的探讨先天性右心室双出口(DORV)的新分型方法,及其对术式选择的指导作用.方法回顾性分析2003年5月至2014年9月在阜外心血管病医院治疗的521例DORV患者的临床资料.根据大动脉的位置关系[分为大动脉关系正常(NA)和大动脉关系异常(AA)]、室间隔缺损与大动脉是否相关[分为大动脉相关室间隔缺损(VSD-A)和远离大动脉的室间隔缺损(VSD-N)]和肺动脉口是否狭窄[分为合并狭窄(POTO)和不合并狭窄但合并肺动脉高压(PHTN)],将DORV分为8型:Ⅰ型(NA,VSD-A,PHTN)、Ⅱ型(NA,VSD-A,POTO)、Ⅲ型(NA,VSD-N,PHTN)、Ⅳ型(NA,VSD-N,POTO)、Ⅴ型(AA,VSD-A,PHTN)、Ⅵ型(AA,VSD-A,POTO)、Ⅶ型(AA,VSD-N,PHTN)和Ⅷ型(AA,VSD-N,POTO).通过超声心动图(部分患者结合心血管造影和CT检查结果)对患者的DORV进行分型,分析此分型方法的可行性,及其对外科术式选择的指导作用.结果 521例DORV患者的分型结果中,Ⅰ型90例(17.3％),Ⅱ型94例(18.0％),Ⅲ型33例(6.3％),Ⅳ型34例(6.5％),Ⅴ型64例(12.3％),Ⅵ型61例(11.7％),Ⅶ型33例(6.3％),Ⅷ型112例(21.5％),新分型方法涵盖了全部DORV病例.对照术中探查或未行手术者的心血管造影和CT检查结果,DORV新分型的诊断准确率为100％.在排除了双心室矫治的禁忌证后,不同分型的DORV对应选择不同的外科术式,有效指导了外科治疗方案的选择.结论新分型方法能够准确诊断DORV病变,对外科治疗方案及术式的选择具有指导作用.
목적 탐토선천성우심실쌍출구(DORV)적신분형방법,급기대술식선택적지도작용.방법 회고성분석2003년5월지2014년9월재부외심혈관병의원치료적521례DORV환자적림상자료.근거대동맥적위치관계[분위대동맥관계정상(NA)화대동맥관계이상(AA)]、실간격결손여대동맥시부상관[분위대동맥상관실간격결손(VSD-A)화원리대동맥적실간격결손(VSD-N)]화폐동맥구시부협착[분위합병협착(POTO)화불합병협착단합병폐동맥고압(PHTN)],장DORV분위8형:Ⅰ형(NA,VSD-A,PHTN)、Ⅱ형(NA,VSD-A,POTO)、Ⅲ형(NA,VSD-N,PHTN)、Ⅳ형(NA,VSD-N,POTO)、Ⅴ형(AA,VSD-A,PHTN)、Ⅵ형(AA,VSD-A,POTO)、Ⅶ형(AA,VSD-N,PHTN)화Ⅷ형(AA,VSD-N,POTO).통과초성심동도(부분환자결합심혈관조영화CT검사결과)대환자적DORV진행분형,분석차분형방법적가행성,급기대외과술식선택적지도작용.결과 521례DORV환자적분형결과중,Ⅰ형90례(17.3％),Ⅱ형94례(18.0％),Ⅲ형33례(6.3％),Ⅳ형34례(6.5％),Ⅴ형64례(12.3％),Ⅵ형61례(11.7％),Ⅶ형33례(6.3％),Ⅷ형112례(21.5％),신분형방법함개료전부DORV병례.대조술중탐사혹미행수술자적심혈관조영화CT검사결과,DORV신분형적진단준학솔위100％.재배제료쌍심실교치적금기증후,불동분형적DORV대응선택불동적외과술식,유효지도료외과치료방안적선택.결론 신분형방법능구준학진단DORV병변,대외과치료방안급술식적선택구유지도작용.
Objective To explore the feasibility and value of a new categorization of double outlet right ventricular (DORV) on guiding the optimal choices of surgical approaches.Methods Five hundred and twenty one DORV patients diagnosed by echocardiography,angiocardiography and CT at Fuwai Hospital from May 2003 to September 2014 were enrolled in this retrospective study.Congenital DORV was categorized according to three basic factors as follows:the positional relationships of great arteries (normal relation or abnormal relation),the relationships of the ventricular septal defect (VSD) to the great arteries (committed VSD or remote VSD),the presence or absence of pulmonary outflow tract obstruction (POTO).Eight types of DORV were established:type Ⅰ (normal relation,committed VSD,without POTO),type Ⅱ (normal relation,committed VSD,POTO),type Ⅲ (normal relation,remote VSD,without POTO),type Ⅳ (normal relation,remote VSD,POTO),type Ⅴ (abnormal relation,committed VSD,without POTO),type Ⅵ (abnormal relation,committed VSD,POTO),type Ⅶ (abnormal relation,remote VSD,without POTO),type Ⅷ (abnormal relation,remote VSD,POTO).Feasibility of this classification and the value of this classification on guiding the choice of surgical approaches were analyzed.Results Among the five hundred and twenty one patients,there were 90 patients (17.3％) with type Ⅰ DORV,94 patients (18.0％) with type Ⅱ,33 patients (6.3％) with type Ⅲ,34 patients (6.5％) with type Ⅳ,64 patients (12.3％) with type Ⅴ,61 patients (11.7％) with type Ⅵ,33 patients (6.3％) with type Ⅶ,112 patients (21.5％) with type Ⅷ.Thus,all patients could be typed by this classification method.The echocardiography diagnosis was consistent with the intra-operative and or cardiac catheterization/CT findings.Excluding the contraindications of bi-ventricular repair,different surgical approaches were performed in every subtype of DORV according the classification,which indicated that this novel categorization could accurately guide the clinic managements.Conclusion This novel DORV categorization can accurately diagnose DORV lesions,and guide the clinic therapy choice.