Modified cone reconstruction in the treatment of Ebstein’s anomaly in 18 patients_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LI Junsheng ¹ ,  MA Jie ² , YAN Zixing ² , ZHANG Haichun ² , CHANG Liang ² , SHI Yangyang ¹

1. Shanxi Medical University, Taiyuan, 030001, P.R.China;
2. Department of Cardiothoracic Surgery, Shanxi Medical University Second Hospital, Taiyuan, 030001, P.R.China;

Corresponding author：

MA Jie, Email: majiexxwk@163.com

Keywords：

Ebstein's anomaly; modified cone reconstruction; autologous pericardial; double-orifice technique

DOI：

10.7507/1007-4848.201608050

Video：

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Objective To investigate the early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of Ebstein’s anomaly (EA). Methods Clinical data of 18 consecutive patients with EA in our hospital between May 2008 and August 2015 were retrospectively analyzed. All patients were diagnosed by echocardiography. There were 8 males and 10 females with an average age of 20.3 years ranging from 5 to 41 years. According to New York Heart Association classification, 12 patients were classified into grade Ⅱ and 6 grade Ⅲ. One patient had acute arterial embolism and amputation of left lower extremity caused by paradoxical embolism of combined secundum atrial septal defect, and another one was combined with double-orifice technique due to postoperative poor closure of tricuspid valve. The modified cone reconstruction was used to correct the EA, to make leaflets coapted well and form central blood flow. For those patients whose anterior leaflet developed poor and smaller, valve leaflet was widened by using autologous pericardial. For all patients, tricuspid annulus was reinforced by autologous pericardial. Results Two patients suffered arrhythmia, and returned to normal after medication. The rest patients recovered well without death. Echocardiography found 1 patient with moderate regurgitation and the rest of patients’ leaflets coapted well and had no tricuspid stenosis. They were followed up 9 to 38 months postoperatively, and cardiac function of gradeⅠin 14 patients and gradeⅡin 4 patients. Conclusion The early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of EA are affirmative which can make leaflets coapt completely and have a strong anti-regurgitation ability, reducing the incidence of re-operation, valve replacement and postoperative mortality.

Citation： LI Junsheng, MA Jie, YAN Zixing, ZHANG Haichun, CHANG Liang, SHI Yangyang. Modified cone reconstruction in the treatment of Ebstein’s anomaly in 18 patients. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2017, 24(11): 863-866. doi: 10.7507/1007-4848.201608050 Copy

1.	Robicsek F. Wilhelm Ebstein and the history of surgery for Ebstein's Disease. Thorac Cardiovasc Surg, 2013, 61(4): 286-292.
2.	Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly: prevalence and patterns in Texas, 1999-2005. Am J Med Genet A, 2011, 155A(5): 1007-1014.
3.	Rahman F, Salman M, Akhter N, et al. Pattern of congenital heart diseases. Mymensingh Med J, 2012, 21(2): 246-250.
4.	Danielson GK, Fuster V. Surgical repair of Ebstein's anomaly. Ann Surg, 1982, 196(4): 499-504.
5.	Attenhofer Jost CH, Connolly HM, Edwards WD, et al. Ebstein's anomaly--review of a multifaceted congenital cardiac condition. Swiss Med Wkly, 2005, 135(19-20): 269-281.
6.	Dearani JA, Said SM, O'Leary PW, et al. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg, 2013, 95(1): 220-226.
7.	Gentles TL, Calder AL, Clarkson PM, et al. Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve. Am J Cardiol, 1992, 69(4): 377-381.
8.	Hennebry TA, Calkins HG, Chandra-Strobos N. Successful interventional treatment of an octogenarian presenting with syncope and Ebstein's anomaly of the tricuspid valve. J Invasive Cardiol, 2002, 14(1): 44-47.
9.	Kipps AK, Graham DA, Lewis E, et al. Natural history of exercise function in patients with Ebstein anomaly: A serial study. Am Heart J, 2012, 163(3): 486-491.
10.	郑慕白, 郭文彬. 实用超声心电图解析与诊断. 北京: 科学技术文献出版社, 2000. 152-156.
11.	Cherry C, DeBord S, Moustapha-Nadler N. Ebstein's anomaly: a complex congenital heart defect. Aorn J, 2009, 89(6): 1098-1110.
12.	Legius B, Van De Bruaene A, Van Deyk K, et al. Behavior of Ebstein's anomaly: Single-center experience and midterm follow-up. Cardiology, 2010, 117(2): 90-95.
13.	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(1): 92-101.
14.	Hunter SW, Lillehei CW. Ebstein's malformation of the tricuspid valve; study of a case together with suggestion of a new form of surgical therapy. Dis Chest, 1958, 33(3): 297-304.
15.	梁志强, 高夏, 王平凡, 等. Ebstein 心脏畸形 42 例外科治疗. 中国心血管病研究杂志, 2006, (11): 813-815.
16.	何维来, 周汝元, 葛圣林, 等. 改良 Carpentier 法矫治三尖瓣下移畸形. 中国胸心血管外科临床杂志, 2012, 19(1): 22-25.
17.	张泽伟, 姜力骏, 齐建川, 等. 改良 Carpentier 法治疗三尖瓣下移畸形 32 例病例分析. 心肺血管病杂志, 2013, 32(6): 685-688.
18.	马捷, 林文杰, 孙宗全, 等. 改良 Carpentier 法矫治 Ebstein 畸形 21 例. 中华胸心血管外科杂志, 2008, 24(3): 173-174.
19.	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(6): 4-6.
20.	da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg, 2007, 133(1): 215-223.
21.	Silva JP, Silva Lda F, Moreira LF, et al. Cone reconstruction in Ebstein's anomaly repair: early and long-term results. Arq Bras Cardiol, 2011, 97(3): 199-208.
22.	da Silva JP, da Silva Lda F. Ebstein's anomaly of the tricuspid valve: the cone repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2012, 15(1): 38-45.
23.	Liu J, Qiu L, Zhu Z, et al. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg, 2011, 141(5): 1178-1183.
24.	Vogel M, Marx GR, Tworetzky W, et al. Ebstein's malformation of the tricuspid valve: short-term outcomes of the "cone procedure" versus conventional surgery. Congenit Heart Dis, 2012, 7(1): 50-58.
25.	Reddin G, Poterucha JT, Dearani JA, et al. Cone reconstruction of atypical ebstein anomaly associated with right ventricular apical hypoplasia. Tex Heart Inst J, 2016, 43(1): 78-80.
26.	Sata S, Murin P, Hraska V. Cone reconstruction of Ebstein's anomaly in a neonate. Ann Thorac Surg, 2012, 94(4): e99-e100.
27.	Pizarro C, Bhat MA, Temple J. Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly. Multimed Man Cardiothorac Surg, 2012, 2012: mms014.

1. Robicsek F. Wilhelm Ebstein and the history of surgery for Ebstein's Disease. Thorac Cardiovasc Surg, 2013, 61(4): 286-292.
2. Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly: prevalence and patterns in Texas, 1999-2005. Am J Med Genet A, 2011, 155A(5): 1007-1014.
3. Rahman F, Salman M, Akhter N, et al. Pattern of congenital heart diseases. Mymensingh Med J, 2012, 21(2): 246-250.
4. Danielson GK, Fuster V. Surgical repair of Ebstein's anomaly. Ann Surg, 1982, 196(4): 499-504.
5. Attenhofer Jost CH, Connolly HM, Edwards WD, et al. Ebstein's anomaly--review of a multifaceted congenital cardiac condition. Swiss Med Wkly, 2005, 135(19-20): 269-281.
6. Dearani JA, Said SM, O'Leary PW, et al. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg, 2013, 95(1): 220-226.
7. Gentles TL, Calder AL, Clarkson PM, et al. Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve. Am J Cardiol, 1992, 69(4): 377-381.
8. Hennebry TA, Calkins HG, Chandra-Strobos N. Successful interventional treatment of an octogenarian presenting with syncope and Ebstein's anomaly of the tricuspid valve. J Invasive Cardiol, 2002, 14(1): 44-47.
9. Kipps AK, Graham DA, Lewis E, et al. Natural history of exercise function in patients with Ebstein anomaly: A serial study. Am Heart J, 2012, 163(3): 486-491.
10. 郑慕白, 郭文彬. 实用超声心电图解析与诊断. 北京: 科学技术文献出版社, 2000. 152-156.
11. Cherry C, DeBord S, Moustapha-Nadler N. Ebstein's anomaly: a complex congenital heart defect. Aorn J, 2009, 89(6): 1098-1110.
12. Legius B, Van De Bruaene A, Van Deyk K, et al. Behavior of Ebstein's anomaly: Single-center experience and midterm follow-up. Cardiology, 2010, 117(2): 90-95.
13. 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(1): 92-101.
14. Hunter SW, Lillehei CW. Ebstein's malformation of the tricuspid valve; study of a case together with suggestion of a new form of surgical therapy. Dis Chest, 1958, 33(3): 297-304.
15. 梁志强, 高夏, 王平凡, 等. Ebstein 心脏畸形 42 例外科治疗. 中国心血管病研究杂志, 2006, (11): 813-815.
16. 何维来, 周汝元, 葛圣林, 等. 改良 Carpentier 法矫治三尖瓣下移畸形. 中国胸心血管外科临床杂志, 2012, 19(1): 22-25.
17. 张泽伟, 姜力骏, 齐建川, 等. 改良 Carpentier 法治疗三尖瓣下移畸形 32 例病例分析. 心肺血管病杂志, 2013, 32(6): 685-688.
18. 马捷, 林文杰, 孙宗全, 等. 改良 Carpentier 法矫治 Ebstein 畸形 21 例. 中华胸心血管外科杂志, 2008, 24(3): 173-174.
19. 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(6): 4-6.
20. da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg, 2007, 133(1): 215-223.
21. Silva JP, Silva Lda F, Moreira LF, et al. Cone reconstruction in Ebstein's anomaly repair: early and long-term results. Arq Bras Cardiol, 2011, 97(3): 199-208.
22. da Silva JP, da Silva Lda F. Ebstein's anomaly of the tricuspid valve: the cone repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2012, 15(1): 38-45.
23. Liu J, Qiu L, Zhu Z, et al. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg, 2011, 141(5): 1178-1183.
24. Vogel M, Marx GR, Tworetzky W, et al. Ebstein's malformation of the tricuspid valve: short-term outcomes of the "cone procedure" versus conventional surgery. Congenit Heart Dis, 2012, 7(1): 50-58.
25. Reddin G, Poterucha JT, Dearani JA, et al. Cone reconstruction of atypical ebstein anomaly associated with right ventricular apical hypoplasia. Tex Heart Inst J, 2016, 43(1): 78-80.
26. Sata S, Murin P, Hraska V. Cone reconstruction of Ebstein's anomaly in a neonate. Ann Thorac Surg, 2012, 94(4): e99-e100.
27. Pizarro C, Bhat MA, Temple J. Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly. Multimed Man Cardiothorac Surg, 2012, 2012: mms014.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Modified cone reconstruction in the treatment of Ebstein’s anomaly in 18 patients

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