CAJ | 학술논문

目的探讨婴幼儿主动脉弓中断(IAA)合并心内畸形胸骨正中切口一期矫治手术效果以及影响因素.方法 2005年8月至2012年1月,采用胸骨正中切口体外循环(CPB)下一期纠治IAA合并心内畸形患婴23例,其中男12例,女11例；年龄18天～3岁,体质量(6.61 ±3.26) kg.按Rastelli 分型,A型20例,B型3例.患儿均合并粗大动脉导管未闭及大型室间隔缺损,其他合并畸形包括主肺动脉窗、右肺动脉起源于主动脉、主动脉瓣两瓣畸形和主动脉瓣下狭窄等.患儿术前均行超声心动图检查,14例行64排CT检查,4例行造影检查.22例术前明确诊断,1例术中明确诊断.行主动脉直接吻合3例,补片扩大主动脉19例,人工管道经肺动脉内连接主动脉弓1例；同期矫治合并心内畸形.结果全组体外循环(129.76±38.98) min,主动脉阻断(74.47±24.30)min,住ICU院平均96 h.术后2例死亡,其中1例2月龄患婴死于肺动脉高压危象,1例7月龄患婴术中出血、术后死于严重低心排血量综合征.21例患儿生存,随访2个月～6年,超声检查示患儿术后心功能良好；5例术后6个月上、下肢平均压差＞30 mm Hg(1 mm Hg =0.133 kPa).无因再狭窄再次手术患儿.无神经系统并发症发生.结论 IAA采用胸骨正中一期外科治疗简化了手术过程,减少了手术死亡,提高了患儿的生活质量,早、中期预后良好,是一种值得推广的治疗方法.
목적 탐토영유인주동맥궁중단(IAA)합병심내기형흉골정중절구일기교치수술효과이급영향인소.방법 2005년8월지2012년1월,채용흉골정중절구체외순배(CPB)하일기규치IAA합병심내기형환영23례,기중남12례,녀11례；년령18천～3세,체질량(6.61 ±3.26) kg.안Rastelli 분형,A형20례,B형3례.환인균합병조대동맥도관미폐급대형실간격결손,기타합병기형포괄주폐동맥창、우폐동맥기원우주동맥、주동맥판량판기형화주동맥판하협착등.환인술전균행초성심동도검사,14례행64배CT검사,4례행조영검사.22례술전명학진단,1례술중명학진단.행주동맥직접문합3례,보편확대주동맥19례,인공관도경폐동맥내련접주동맥궁1례；동기교치합병심내기형.결과 전조체외순배(129.76±38.98) min,주동맥조단(74.47±24.30)min,주ICU원평균96 h.술후2례사망,기중1례2월령환영사우폐동맥고압위상,1례7월령환영술중출혈、술후사우엄중저심배혈량종합정.21례환인생존,수방2개월～6년,초성검사시환인술후심공능량호；5례술후6개월상、하지평균압차＞30 mm Hg(1 mm Hg =0.133 kPa).무인재협착재차수술환인.무신경계통병발증발생.결론 IAA채용흉골정중일기외과치료간화료수술과정,감소료수술사망,제고료환인적생활질량,조、중기예후량호,시일충치득추엄적치료방법.
Objective To sum up the clinical information and outcomes of one-stage surgical repairs for interrupted aortic arch (IAA) associated with cardiac defects through median stemotomy in infant.Methods From August 2005 to January 2012,23 IAA patients,aged 18 days to 3 years [mean age (8.61 ± 11.81) months],body weight 3.3-13.0 kg [mean (6.61 ± 3.26) kg] were reviewed underwent one-stage repair.There were 12 male and 11 female.The anatomic subtype ineluded type A 20 (87.0％),type B 3 (13.0％),and no type C in the records.All cases included ventricular septal defect and patent ductus artefious,some also with artirical-pulmonary window,aterical septal defect,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was determined in of the patients and suspected in by echocardiography.Also,64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 patients.All patients with cardiac anomalies underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch.Results In all 23 cases,21 were successful.There was 2 (8.69％) postoperative death:one was due to surgical hemorrhage and severe low cardiac output during perioperative stage and the other was 2-month old due to crisis of pulmonary hypertension.CPB time was ranged from 53-215 min [(129.76 ± 38.98) min],and aortic crossclamp time was 34-125 min[(74.47 ± 24.30) min].The length of stay in ICU postoperatively was 96h averagely.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 13,pneumonia in 7,and supraventricular tachycardia in 12.21 patients were followed up from 2 months to 6 years and were in good condition without recoactation.Conclusion The outcomes of early and medium term for one-stage repair of IAA and associated cardiac anomalies through median stemotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation.It is simplified the procedure and improved life quality of patients.