Ebstein心脏畸形的外科治疗
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摘要
目的:Ebstein心脏畸形(Ebstein's anomaly),又称三尖瓣下移畸形(Downward Displacement of Tricuspid Valve,DDTV)该病是一种相对少见的先天性心脏畸形,其特征为三尖瓣隔瓣和后瓣下移导致右室流入道心房化及形成不同程度缩小但尚有功能的右心室。此畸形在先天性心脏病中占的比例小于1%,男女发病率无显著差别。多于80%的病例合并卵圆孔未闭或房间隔缺损。Ebstein畸形的外科矫治方法始于20世纪50年代,包括三尖瓣置换,Danielson、Carpentier和Quaegebeur等三尖瓣成形以及Glenn分流手术,Starnes手术等,并取得明显效果。对于治疗Ebstein的畸形,虽然目前采用三尖瓣成形术的人越来越多,并主张替代三尖瓣置换术,但是迄今为止,对其外科治疗方法的选择仍无一个统一的标准。尤其对于Ebstein畸形的各种亚型,采用具体的何种手术方法进行矫治尚需进一步探讨。因此,如何更好,更恰当的选择手术方法,以便改善患者心脏功能和提高患者生活质量,对临床治疗Ebstein心脏畸形有重要意义
结论.:Ebstein心脏畸形是一种复杂的先天性心脏病,其治疗的手术方案很多,具体方案应依据病理解剖学特点而定,三尖瓣成形术对多数Ebstein畸形处理均能产生较好效果。对三尖瓣置换术要严格掌握手术适应证。三尖瓣置换术与三尖瓣成形术相比较,存在围术期处理相对烦琐,术后并发症多,手术后需要长期抗凝治疗。在儿童患者中实施存在一定的困难,部分患者尚需二次换瓣手术等缺点。近年来,国外有些学者尝试应用三尖瓣成形术治疗成年及儿童Ebstein畸形,取得良好的疗效。国内亦有部分学者应用三尖瓣成形术矫正这一心脏畸形,取得一定疗效。因此对于所有Ebstein畸形患者都应首先考虑采用三尖瓣成形术,不能成形者方考虑行三尖瓣置换术和姑息性手术,抑或心脏移植。
Objective:Ebstein's anomaly(Downword Displacement of Tricuspid Valve,DDTV)is a relatively rare congenital malformation which is characterized by apical displacement of the septal or posterior leaflet of the tricuspid valve,resulting in 'atrialization' of the inflow tract of the right ventricle and consequently a variably small,but functional right ventricle,accounting for less than 1%of all congenital heart defects,The prevalence is equal in women and men.Up to 80%of patients with Ebstein's anomaly have a concomitant patent foramen ovale or secundum atrial septal defect Surgical Treatment of Ebstein Anomaly begins in 1950s,including Tricuspid Valve replacement, Danielson's procesure,Hardy's procesure,Carpentier's procesure,Glenn shunt and starnes operation,et which possess obvious outcomes.At present,more and more people advocate tricuspid valve annuloplasty instead of tricuspid valve replacement,but there is still not a standard procedure.especially in the treatment of subtype of Ebstein's anomaly.More discussions are needed in choosing the operation.Therefore,how to choose better and more fitable procedure to improve the patient's heart function and the quality of life is meaningful in the Surgical Treatment of Ebstein's Anomaly
Conclusion:Ebstein's anomaly is a complex form of congenital heart disease,which has many treatments.How to choose the treatment depends on the pathological Anatomy.Tricuspid valve annuloplasty can possess good outcomes.The indication for tricuspid valve replacement should be strictly selected.Comparing with tricuspid valve annuloplasty,tricuspid valve replacement is more complicated during the perioperative period,with more postoperative complications,and need long-term anticoagulatory therapy.The procedure is difficult to operate on children patients,part of which need a second operation.Recently some foreign researchers have tried to treat adult and young Ebstein anormaly with tricuspid valve annuloplasty,and achieved good outcomes.Some domestic researchers also treat this anormaly with tricuspid valve annuloplasty,achieving moderate efficacy.Therefore tricuspid valve annuloplasty should be considered as the first choice for all the Ebsteins.If tricuspid valve annuloplasty is not feasible,tricuspid valve replacement and conservative procedures,or heart transplantation should be considered.
结论.:Ebstein心脏畸形是一种复杂的先天性心脏病,其治疗的手术方案很多,具体方案应依据病理解剖学特点而定,三尖瓣成形术对多数Ebstein畸形处理均能产生较好效果。对三尖瓣置换术要严格掌握手术适应证。三尖瓣置换术与三尖瓣成形术相比较,存在围术期处理相对烦琐,术后并发症多,手术后需要长期抗凝治疗。在儿童患者中实施存在一定的困难,部分患者尚需二次换瓣手术等缺点。近年来,国外有些学者尝试应用三尖瓣成形术治疗成年及儿童Ebstein畸形,取得良好的疗效。国内亦有部分学者应用三尖瓣成形术矫正这一心脏畸形,取得一定疗效。因此对于所有Ebstein畸形患者都应首先考虑采用三尖瓣成形术,不能成形者方考虑行三尖瓣置换术和姑息性手术,抑或心脏移植。
Objective:Ebstein's anomaly(Downword Displacement of Tricuspid Valve,DDTV)is a relatively rare congenital malformation which is characterized by apical displacement of the septal or posterior leaflet of the tricuspid valve,resulting in 'atrialization' of the inflow tract of the right ventricle and consequently a variably small,but functional right ventricle,accounting for less than 1%of all congenital heart defects,The prevalence is equal in women and men.Up to 80%of patients with Ebstein's anomaly have a concomitant patent foramen ovale or secundum atrial septal defect Surgical Treatment of Ebstein Anomaly begins in 1950s,including Tricuspid Valve replacement, Danielson's procesure,Hardy's procesure,Carpentier's procesure,Glenn shunt and starnes operation,et which possess obvious outcomes.At present,more and more people advocate tricuspid valve annuloplasty instead of tricuspid valve replacement,but there is still not a standard procedure.especially in the treatment of subtype of Ebstein's anomaly.More discussions are needed in choosing the operation.Therefore,how to choose better and more fitable procedure to improve the patient's heart function and the quality of life is meaningful in the Surgical Treatment of Ebstein's Anomaly
Conclusion:Ebstein's anomaly is a complex form of congenital heart disease,which has many treatments.How to choose the treatment depends on the pathological Anatomy.Tricuspid valve annuloplasty can possess good outcomes.The indication for tricuspid valve replacement should be strictly selected.Comparing with tricuspid valve annuloplasty,tricuspid valve replacement is more complicated during the perioperative period,with more postoperative complications,and need long-term anticoagulatory therapy.The procedure is difficult to operate on children patients,part of which need a second operation.Recently some foreign researchers have tried to treat adult and young Ebstein anormaly with tricuspid valve annuloplasty,and achieved good outcomes.Some domestic researchers also treat this anormaly with tricuspid valve annuloplasty,achieving moderate efficacy.Therefore tricuspid valve annuloplasty should be considered as the first choice for all the Ebsteins.If tricuspid valve annuloplasty is not feasible,tricuspid valve replacement and conservative procedures,or heart transplantation should be considered.
引文
1. Glulian ER,Fuster V,Brandenberg RO ,et al.Ebstein's anomaly :the clinical features and naturalhistory of Ebstein's anomaly of the tricuspid valve. Mayo Clin Proc ,1979 ,54:163-173.
2. Brickner ME,Hillis LD,Lange RA Congenital heart disease in adult;second of two parts.N Engl J Med 2000,342;334-342
3. Soizic Paranon,Philippe Acar.Ebstein's anomaly of the tricuspid valve from fetus to adult. Heart 2008;94;237-243
4. Attie F,Rosas M,Rijlaarsdam M,et al:The adult patient with Ebstein's anormaly;outcome in 72 unoperated patients.Medline 2000,79;27-36
5. Yetman AT, Freedom RM, McCrindle BW.Outcome in cyanotic neonates with Ebstein's anomaly.Am J Cardiol. 1998 Mar 15;81(6):749-54.
6. Zalzstein E, Koren G, Einarson T. Freedom RM. A case-control study on the association between first trimester exposure to lithium and Ebstein's anomaly. Am J Cardiol. 1990 Mar 15;65(11):817-8.
7. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications.J Am Coll Cardiol.1992 Apr;19(5):1049-53.
8.Seshadri Balaji,Nicholas R Dennis,Barry R Keeton.Familial Ebstein's anomaly:a report of six cases in two generations associated with mild skeletal abnormalities.Br Heart J 1991;66:26-8
9.杜亮,郭兰敏.Ebstein畸形中三尖瓣的解剖特征及其临床意义.中国临床解剖学杂志2000年第18卷第2期
10.汪曾炜,刘维永,张宝仁.手术学全集·心血管外科卷.北京:人民卫生出版社,1995.51-60.
11.Kirklin JW,BarrattBoyes BG.Cardiac surgery ed.New York:John Wiley & Sons Inc.,1993.1105-1130.
12.褚衍林,吴英渝,李鲁等.三尖瓣下移畸形的诊断及外科治疗心肺血管病杂志2004年8月第23卷第3期
13.丛涛,王珂.超声心动图对三尖瓣下移畸形术前诊断及术后疗效评价的作用 Chinese Journal of Cardiovascular Medicine,October 2006,Vol 11.No 5
14.陆培明,黄新胜Ebstein畸形的超声心动图诊断 南方医科大学学报 2007;27(1)
15.谷颖,车正兰 超声心动图诊断三尖瓣下移畸形 中国现代医学杂志Jan.2006 Vol.16 No.1
16.Joseph J.Vettukattil,Tara Bharucha and Robert H.Anderson.Defining Ebstein's malformation using three-dimensional echocardiography.Interact CardioVasc Thorac Surg 2007;6:685-690
17.耿庆国 吕增城 王丽娣 二维及彩色多普勒超声心动图对Ebstein畸形的诊断 天津医药1999年1月第27卷第1期
18.Oechslin E,Buchholz S,Jenni R.Ebstein's anomaly in adults:Doppler-echocardiographic evaluation.Thorac Cardiovasc Surg.2000 Aug;48(4):209-13
19.Quaegebeur JM,Sreeram N,Fraser AG,et al.Surgery for Ebstein's anomaly:the clinical and echocardiographic evaluation of a new technique[J].J Am Coll Cardiol,1991,17(3):722-728.
20.Carpentier A,Chauvaud S,Mace L,et al.A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.J Thorac Cardiovasc Surg,1988,96:92-101.
21.汪曾炜,刘维永,张宝仁主编.心血管外科卷[M].北京:人民军医出版社,1995.4962503.
22.吴清玉,张怀军,许建屏.108例三尖瓣下移的矫治.中华外科杂志1999年11月第37卷第11期
23.Mazzera E,Corno A,Picardo S,Di Donato R,Marino B,Costa D,Marcelletti C.Bidirectional cavopulmonary shunts:clinical applications as staged or definitive palliation.Ann Thorac Surg.1989 Mar;47(3):415-20.
24.Lemes V,Samuel B.Ritcher,M.D.et al.Enhancement of Ventricular Mechanics Following Bidirectional Superior Cavopulmonary Anastomosis in Patients with Single Ventricle.J Card Surg,1995;10:119-124
25.Vaughn A.Starnes MD,Paul T.Pitlick MD,et al.Ebstein's anomaly appearing in the neonate:A new surgical approach.J Thorac Cardiovasc Surg 1991;101:1082-7
26.Hunter SW.Lillehei.Ebstein's malformation of the tricuspid valve.Study of a case together with suggestion of a new form of surgical therapy.Circulation,1958;33:297-304
27.Hardy KL,Roe BB Ebstein's anomaly.Further experience with definitive repair.Thorac cardiovasc surg,1969;58:533
28.Danielson GK,Maloney J D,Devloo RAE.Surgical repair of Ebstein's anomaly[J].Mayo Clin Proc,1979,54:185-192.
29.尤斌,陈英淳,梁宜武等.改良术式矫正Ebstein畸形.中华胸心血管外科杂志,2000,16:321-3231
30.Carpentier A,Chauvaud S,Mace L,et al.A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.J Thorac Cardiovasc Surg,1988,96:92-101
31.陈胜喜,郗二平,罗万俊等.改良Carpentier法治疗埃布斯坦综合征25例 中国胸心血管外科临床杂志.2005.12(2):114-115
32.DeVega NG.Selective,adjustable and permanent annuloplasty.An original technic for the treatment of tricuspid insufficiency.Rev Esp Cardiol,1972,25:555-556.
33.Wei J,Chang CY,Lee FY,et al.De Vega's semicircular annuloplasty for tricuspid valve regurgitation.Ann Thorac surg,1993,55:482-485.
34.于存涛 胡晓鹏 常谦 改良DeVega三尖瓣成形术的临床应用 中华胸心血管外科杂志Chinese Journal of Thoracic and Cardiovascular Surgery 2006年,第04期
35.肖学钧,张镜芳,吴若彬,等.左心瓣膜置换术后三尖瓣关闭不全的外科处理[J].中华胸心血管外科杂志,2003,19(5):2602-262.
36.Roland Hetzer MD.phD,Nicole Nagdyman MD,et al.A modified repair technique for tricuspid incompetence in Ebstein's anomaly.The Jounral of Thoracic and Cardiovascular Surgery 1998;15(4):857-868
37.Norbert Augustin MD,Peter Schmidt-Habelmann MD,et al.Results after surgical repair of Ebstein's anomaly.Ann-Thorac-Surg 1997;63:1650-6
38.Vargas FJ,Mengo G,etal.Tricuspid annuloplasty and ventricular plication for Ebstein's malformation.Ann-Thorac-Surg.1998 Jun;65(6):1755
39.朱晓东,主译.(deLevals.主编)先天性心脏病外科学.北京:人民卫生出版社,1996.543-549
40.Singh AK,Christian FD,Williams DO,et al.Follow-up assessment of the St.jude medical prosthetic valve in the tricuspid position:clinical and hemodynamic results.Ann Thorac Surg,1984,37:324-327.
41.Scully HE,Armstrong CS.Tricuspid valae replacement.Fifteen years of experience with mechanical prostheses and bioprostheses.J Thorac Cardiovasc Surg,1995,109:1035-1041.
42.Glower DD,White WD,Young WG,et al.In hospital and long-term outcome after porcine tricuspid valve replacement.J Thorac Cardiovasc Surg,1995,109:877-884
43.Nakano K,Eishi K,Kosakai Y,et al.Ten-year experience with the carpentier-edwards pericardial xenograft in the tricuspid position.J Thorac Cardiovasc Surg,1996,111:605-612.
44.Gersh BJ,Schaff HV,Vatterott PT,et al.Results of triple valve replacement in 91 patients.Perioperative mortality and long-term follow up.Circulation,1985,72:130-137.
45.Van Nooten GJ,Caes F,Taeymans Y,et al.Tricuspid valve replacement:postoperative and long-term results.J Thorac Cardiovasc Surg,1995,110:672-679.
46.邹良建,汪曾炜,张宝仁等.Ebscein心脏畸形的外科治疗.第二军医大学学报.1999,20(12):1012-1014
47.Kumar N,Gallo R Al-Halees,etal.Unstented semilunarhomograft replacement of Tricuspid Valvein Ebstein malformation[J]Ann Thorac Surg,1995,59(2):320-322
48.Kaulitz R,Ziemer G.Modified Fontan Procedure for Ebstein's anomaly of the tricuspid valve an alternative surgical approach preserving Ebstein's anomaly.Thorac Cardiovasc Surg,1995,43:275-279.
49.Marianeschi S M,McElhinney D B,Reddy V M,et al.Alternative approach to the repair of Ebstein's malformation:intracardiac repair with ventricular unloading[J].Ann Thorac Surg,1998,66(5):1546-1550
50.Stellin G,Vida VL,Milanesi O,et al.Surgical treatment of complex cardiac anomalies:the 'one and one half ventricle repair'[J].Eur J Cardiothorac Surg,2002,22(3):431-436
51.Antonio F.Corno,Pierre-Guy Chassot,Maurice Payot,Nicole Sekarski,Piergiorgio Tozzi.Ludwig K.yon Segesser Ebstein's anomaly:one and a half ventricular repair SWISS MED WKLY 2002;132:485-488
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53. Lower RR,Shumway NE,srudies on orthotopic transplantation of the canine heart. Surg Forum,1960,11:18
2. Brickner ME,Hillis LD,Lange RA Congenital heart disease in adult;second of two parts.N Engl J Med 2000,342;334-342
3. Soizic Paranon,Philippe Acar.Ebstein's anomaly of the tricuspid valve from fetus to adult. Heart 2008;94;237-243
4. Attie F,Rosas M,Rijlaarsdam M,et al:The adult patient with Ebstein's anormaly;outcome in 72 unoperated patients.Medline 2000,79;27-36
5. Yetman AT, Freedom RM, McCrindle BW.Outcome in cyanotic neonates with Ebstein's anomaly.Am J Cardiol. 1998 Mar 15;81(6):749-54.
6. Zalzstein E, Koren G, Einarson T. Freedom RM. A case-control study on the association between first trimester exposure to lithium and Ebstein's anomaly. Am J Cardiol. 1990 Mar 15;65(11):817-8.
7. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications.J Am Coll Cardiol.1992 Apr;19(5):1049-53.
8.Seshadri Balaji,Nicholas R Dennis,Barry R Keeton.Familial Ebstein's anomaly:a report of six cases in two generations associated with mild skeletal abnormalities.Br Heart J 1991;66:26-8
9.杜亮,郭兰敏.Ebstein畸形中三尖瓣的解剖特征及其临床意义.中国临床解剖学杂志2000年第18卷第2期
10.汪曾炜,刘维永,张宝仁.手术学全集·心血管外科卷.北京:人民卫生出版社,1995.51-60.
11.Kirklin JW,BarrattBoyes BG.Cardiac surgery ed.New York:John Wiley & Sons Inc.,1993.1105-1130.
12.褚衍林,吴英渝,李鲁等.三尖瓣下移畸形的诊断及外科治疗心肺血管病杂志2004年8月第23卷第3期
13.丛涛,王珂.超声心动图对三尖瓣下移畸形术前诊断及术后疗效评价的作用 Chinese Journal of Cardiovascular Medicine,October 2006,Vol 11.No 5
14.陆培明,黄新胜Ebstein畸形的超声心动图诊断 南方医科大学学报 2007;27(1)
15.谷颖,车正兰 超声心动图诊断三尖瓣下移畸形 中国现代医学杂志Jan.2006 Vol.16 No.1
16.Joseph J.Vettukattil,Tara Bharucha and Robert H.Anderson.Defining Ebstein's malformation using three-dimensional echocardiography.Interact CardioVasc Thorac Surg 2007;6:685-690
17.耿庆国 吕增城 王丽娣 二维及彩色多普勒超声心动图对Ebstein畸形的诊断 天津医药1999年1月第27卷第1期
18.Oechslin E,Buchholz S,Jenni R.Ebstein's anomaly in adults:Doppler-echocardiographic evaluation.Thorac Cardiovasc Surg.2000 Aug;48(4):209-13
19.Quaegebeur JM,Sreeram N,Fraser AG,et al.Surgery for Ebstein's anomaly:the clinical and echocardiographic evaluation of a new technique[J].J Am Coll Cardiol,1991,17(3):722-728.
20.Carpentier A,Chauvaud S,Mace L,et al.A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.J Thorac Cardiovasc Surg,1988,96:92-101.
21.汪曾炜,刘维永,张宝仁主编.心血管外科卷[M].北京:人民军医出版社,1995.4962503.
22.吴清玉,张怀军,许建屏.108例三尖瓣下移的矫治.中华外科杂志1999年11月第37卷第11期
23.Mazzera E,Corno A,Picardo S,Di Donato R,Marino B,Costa D,Marcelletti C.Bidirectional cavopulmonary shunts:clinical applications as staged or definitive palliation.Ann Thorac Surg.1989 Mar;47(3):415-20.
24.Lemes V,Samuel B.Ritcher,M.D.et al.Enhancement of Ventricular Mechanics Following Bidirectional Superior Cavopulmonary Anastomosis in Patients with Single Ventricle.J Card Surg,1995;10:119-124
25.Vaughn A.Starnes MD,Paul T.Pitlick MD,et al.Ebstein's anomaly appearing in the neonate:A new surgical approach.J Thorac Cardiovasc Surg 1991;101:1082-7
26.Hunter SW.Lillehei.Ebstein's malformation of the tricuspid valve.Study of a case together with suggestion of a new form of surgical therapy.Circulation,1958;33:297-304
27.Hardy KL,Roe BB Ebstein's anomaly.Further experience with definitive repair.Thorac cardiovasc surg,1969;58:533
28.Danielson GK,Maloney J D,Devloo RAE.Surgical repair of Ebstein's anomaly[J].Mayo Clin Proc,1979,54:185-192.
29.尤斌,陈英淳,梁宜武等.改良术式矫正Ebstein畸形.中华胸心血管外科杂志,2000,16:321-3231
30.Carpentier A,Chauvaud S,Mace L,et al.A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.J Thorac Cardiovasc Surg,1988,96:92-101
31.陈胜喜,郗二平,罗万俊等.改良Carpentier法治疗埃布斯坦综合征25例 中国胸心血管外科临床杂志.2005.12(2):114-115
32.DeVega NG.Selective,adjustable and permanent annuloplasty.An original technic for the treatment of tricuspid insufficiency.Rev Esp Cardiol,1972,25:555-556.
33.Wei J,Chang CY,Lee FY,et al.De Vega's semicircular annuloplasty for tricuspid valve regurgitation.Ann Thorac surg,1993,55:482-485.
34.于存涛 胡晓鹏 常谦 改良DeVega三尖瓣成形术的临床应用 中华胸心血管外科杂志Chinese Journal of Thoracic and Cardiovascular Surgery 2006年,第04期
35.肖学钧,张镜芳,吴若彬,等.左心瓣膜置换术后三尖瓣关闭不全的外科处理[J].中华胸心血管外科杂志,2003,19(5):2602-262.
36.Roland Hetzer MD.phD,Nicole Nagdyman MD,et al.A modified repair technique for tricuspid incompetence in Ebstein's anomaly.The Jounral of Thoracic and Cardiovascular Surgery 1998;15(4):857-868
37.Norbert Augustin MD,Peter Schmidt-Habelmann MD,et al.Results after surgical repair of Ebstein's anomaly.Ann-Thorac-Surg 1997;63:1650-6
38.Vargas FJ,Mengo G,etal.Tricuspid annuloplasty and ventricular plication for Ebstein's malformation.Ann-Thorac-Surg.1998 Jun;65(6):1755
39.朱晓东,主译.(deLevals.主编)先天性心脏病外科学.北京:人民卫生出版社,1996.543-549
40.Singh AK,Christian FD,Williams DO,et al.Follow-up assessment of the St.jude medical prosthetic valve in the tricuspid position:clinical and hemodynamic results.Ann Thorac Surg,1984,37:324-327.
41.Scully HE,Armstrong CS.Tricuspid valae replacement.Fifteen years of experience with mechanical prostheses and bioprostheses.J Thorac Cardiovasc Surg,1995,109:1035-1041.
42.Glower DD,White WD,Young WG,et al.In hospital and long-term outcome after porcine tricuspid valve replacement.J Thorac Cardiovasc Surg,1995,109:877-884
43.Nakano K,Eishi K,Kosakai Y,et al.Ten-year experience with the carpentier-edwards pericardial xenograft in the tricuspid position.J Thorac Cardiovasc Surg,1996,111:605-612.
44.Gersh BJ,Schaff HV,Vatterott PT,et al.Results of triple valve replacement in 91 patients.Perioperative mortality and long-term follow up.Circulation,1985,72:130-137.
45.Van Nooten GJ,Caes F,Taeymans Y,et al.Tricuspid valve replacement:postoperative and long-term results.J Thorac Cardiovasc Surg,1995,110:672-679.
46.邹良建,汪曾炜,张宝仁等.Ebscein心脏畸形的外科治疗.第二军医大学学报.1999,20(12):1012-1014
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