CAJ | 학술논문

目的：总结双向 Glenn 术治疗复杂先天性心脏病的临床经验。方法2008年5月至2012年12月,我科对103例具有 Fontan 术高危因素的复杂紫绀型先心病患儿行双向 Glenn 术。术前诊断：功能性单心室61例,大动脉转位12例；矫正型大动脉转位15例,右心室双出口5例,Ebstein畸形4例,肺脉闭锁合并室间隔缺损2例,右室发育不良4例。50例在体外循环下手术,53例在非体外循环下手术。手术方式包括：单侧双向 Glenn 术83例,双侧双向 Glenn 术20例,Glenn 同期行肺动脉成形术18例,房间隔切除术15例,肺动脉环缩术12例,完全性肺静脉异位引流矫治术6例,房室瓣整形术6例、房室瓣置换术2例。结果手术死亡2例,病死率1.9%。术毕平均肺动脉压力(mPAP)(16.74±2.95)mmHg。出院日不吸氧静息氧饱和度由术前(68±13)%升高至(84±6)%。术后随访6个月至3年,1例死亡；2例因房室瓣反流加重行“房室瓣置换术”；1例因顽固性上腔静脉综合征行“双向 Glenn 拆除+B-T 分流术”；26例完成 Fontan 术；余患儿生长发育基本正常,心功能(NYHA)I-II 级,静息 SpO2(80±11)%,无吻合口狭窄。结论双向 Glenn 术可有效改善复杂紫绀先心病患儿氧饱和度,降低心室容量负荷,近期及中期效果满意。积极处理合并的危险因素,可显著提高双向 Glenn 手术效果。
목적：총결쌍향 Glenn 술치료복잡선천성심장병적림상경험。방법2008년5월지2012년12월,아과대103례구유 Fontan 술고위인소적복잡자감형선심병환인행쌍향 Glenn 술。술전진단：공능성단심실61례,대동맥전위12례；교정형대동맥전위15례,우심실쌍출구5례,Ebstein기형4례,폐맥폐쇄합병실간격결손2례,우실발육불량4례。50례재체외순배하수술,53례재비체외순배하수술。수술방식포괄：단측쌍향 Glenn 술83례,쌍측쌍향 Glenn 술20례,Glenn 동기행폐동맥성형술18례,방간격절제술15례,폐동맥배축술12례,완전성폐정맥이위인류교치술6례,방실판정형술6례、방실판치환술2례。결과수술사망2례,병사솔1.9%。술필평균폐동맥압력(mPAP)(16.74±2.95)mmHg。출원일불흡양정식양포화도유술전(68±13)%승고지(84±6)%。술후수방6개월지3년,1례사망；2례인방실판반류가중행“방실판치환술”；1례인완고성상강정맥종합정행“쌍향 Glenn 탁제+B-T 분류술”；26례완성 Fontan 술；여환인생장발육기본정상,심공능(NYHA)I-II 급,정식 SpO2(80±11)%,무문합구협착。결론쌍향 Glenn 술가유효개선복잡자감선심병환인양포화도,강저심실용량부하,근기급중기효과만의。적겁처리합병적위험인소,가현저제고쌍향 Glenn 수술효과。
Objective To review the current outcomes of bidirectional Glenn (BDG)shunt in the treatment of children with complicated cyanotic congenital heart disease.Methods A total of 103 consecutive patients in high-risk Fontan procedure underwent Glenn shunt at our center from May 2008 to December 2012.The diagnoses included single ventricle (n = 61 ),transposition of great arteries (TGA,n= 12),corrected transposition of great arteries (c-TGA,n = 1 5 ),double outlet of right ventricle (DORV,n = 5 ),Ebstein anomaly (n =4),pulmonary atresia with intact ventricular septum (PA/VSD,n = 2 )and right ventricular hypoplasia (n = 4 ).There were 1 7 patients with heterotaxy syndrome. A total of 83 patients underwent unilateral operations and the remainder bilaterally.Among them,the procedure was performed with cardiopulmonary bypass (CPB)(n=50) and without (n = 56).Concomitant procedures included pulmonary artery reconstruction (n = 1 8 ), atrial septectomy (n = 1 5 ), pulmonary artery banding (n = 12 ), correction of total anomalous pulmonary venous connection (TAPVC, n = 6 ), atrioventricular valve repair (n = 6 ) and atrioventricular valve replacement (n = 2).Results Two early operative mortalities occurred with a rate of 1 .9%.The mean pulmonary artery pressure was 16.74 ± 2.95 mmHg. And the mean systematic oxygen saturation increased from 68 ± 13% to 84±6% at discharge.The mean follow-up period was 1 .5±1 .2 (0.5-3)years.During the observational period,1 patient died.The procedures included Glenn takedown plus B-T shunt (n = 1 )and atrioventricular valve replacement (n = 2 ). Twenty-six patients,considered optimal candidates,underwent Fontan operation.The remainders were well-palliated with an arterial oxygen saturation of 80±1 1 % at rest,cardiac function (NYHA) I-II class and no anastomotic stenosis.Conclusions Glenn shunt is an excellent temporary palliation prior to a Fontan operation for various cyanotic CHDs leading eventually to a single ventricle repair.It is of great importance for preventing an elevation of pulmonary arterial pressure and improving patient outcomes.