Clinical Features and Surgical Strategy of Ebstein's Anomaly in Adults_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

 ZHANGHuai-jun , XUJian-ping , SONGYun-hu

Department of Cardiac Surgery, Fu Wai Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Center for Cardiovascular Diseases, Beijing 100037, P. R. China;

Corresponding author：

ZHANGHuai-jun, Email: zhanghj6662@163.com

Keywords：

Ebstein's anomaly in adults; Clinical feature; Surgical strategy

DOI：

10.7507/1007-4848.20140132

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

Objective To analyze clinical features and surgical strategies of Ebstein's anomaly (EA) in adults. Methods Seventy-eight adult patients with EA underwent surgical treatment in Fu Wai Hospital from January 2008 to December 2011. There were 24 males and 54 females with their age of 18-54 (33.0±9.5) years. Preoperatively, 72 patients were in NYHA class Ⅰ or Ⅱ, and 6 patients were in NYHA class Ⅲ or Ⅳ. Clinical presentations mainly included exercise capacity deterioration and exertional dyspnea. Preoperative echocardiography showed downward displacement of the septal leaflet (SL) of the tricuspid valve (TV) of 34.8±12.7 (20-60) mm. Three patients had severe dysplasia or agenesis of tricuspid SL. Downward displacement of the posterior leaflet (PL) of TV was 46.8±11.6 (20-70) mm, and 1 patient had agenesis of tricuspid PL. Average TV annulus was significantly enlarged with 60±10 (37-70) mm. Mean atrialized portion of the right ventricle was about 40%. There were 18 patients with moderate tricuspid regurgitation (TR) and 60 patients with moderate-to-severe TR. Seventy-five patients received tricuspid valvuloplasty (TVP). Fifty-six patients received plication of the atrialized right ventricle (ARV), 20 patients received ARV resection, and 2 patients didn't receive any specific management of ARV. Thirty-two patients received TVP with a prosthetic ring. Three patients underwent tricuspid valve replacement. Results Two patients died posto-peratively, and in-hospital mortality was 2.5%. Postoperative recovery of the survival patients was good. There was no severe atrioventricular block or other complication. Echocardiography before discharge showed good function of TV without moderate or more severe TR. Mean follow-up was 26 months. None of the patients needed re-operation. Conclusion The incidence of acute heart failure in EA adults is low. TVP is the main surgical procedure to achieve main goals of surgical treatment including improvement in heart function, exercise capacity and quality of life.

Citation： ZHANGHuai-jun, XUJian-ping, SONGYun-hu. Clinical Features and Surgical Strategy of Ebstein's Anomaly in Adults. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2014, 21(4): 465-467. doi: 10.7507/1007-4848.20140132 Copy

1.	Attenhofer Jost CH, Connolly HM, Dearani JA, et al. Ebstein's Anomaly. Circulation, 2007, 115 (2):277-285.
2.	Sata, S, Murín P, Hraška V. Cone reconstruction of Ebstein's anomaly in a neonate. Ann Thorac Surg, 2012, 94 (4):e99-e100.
3.	Badiu CC, Schreiber C, Hörer J, et al. Early timing of surgical inter-vention in patients with Ebstein's anomaly predicts superior longterm outcome. Eur J Cardiothorac Surg, 2010, 37 (1):186-192.
4.	Reemtsen BL, Fagan BT, Wells WJ, et al. Current surgical therapy for Ebstein's anomaly in neonates. J Thorac Cardiovasc Surg, 2006, 132 (6):1285-1290.
5.	Knott-Craig CJ, Goldberg SP, Overholt ED, et al. Repair of neonates and young infants with Ebstein's anomal and related disorders. Ann Thorac Surg, 2007, 84 (2):587-593.
6.	Dearani JA. Behavior of Ebstein's anomaly. Cardiology, 2010, 117 (2):88-89.
7.	Frescura C, Angelini A, Daliento L, et al. Morphological aspects of Ebstein's anomaly in adults. Thorac Cardiov Surg, 2000, 48 (4):203-208.
8.	Legius B, Van De Bruaene AV, Van Deyk K, et al. Behavior of Ebstein's anomaly:single-center experience and midterm follow-up. Cardiology, 2010, 117 (2):90-95.
9.	Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg Summer, 2005, 17 (2):148-154.
10.	Chauvaud S. Ebstein's malformation. Surgical treatment and results. Thorac Cardiov Surg, 2000, 48 (4):220-223.
11.	Hebe J. Ebstein's anomaly in adults:arrhythmias:diagnosis and therapeutic approach. Thorac Cardiovasc Surg, 2000, 48 (4):214-219.
12.	Boston US, Dearani JA, O'Leary PW. Tricuspid valve repair for Ebstein's anomaly in young children:a 30-year experience. Ann Thorac Surg, 2006, 81 (2):690-696.
13.	Bichel DP, Mora BN, Mathewson JW, et al. Modified technique for the surgical treatment of severe tricuspid valve deformity in Ebstein's anomaly. Ann Thorac Surg, 2007, 83 (2):678-680.
14.	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):220-228.
15.	Viola N, Vettukattil JJ, Haw MP. Restoring anterior leaflet continuity:the Spinnaker repair of Ebstein's anomaly. Ann Thorac Surg, 2011, 92 (2):752-754.
16.	Brown ML, Dearani JA, Danielson GK, et al. The outcomes of opera-tions for 539 patients with Ebstein's anomaly. J Thorac Cardiovasc Surg, 2008, 135 (5):1120-1136.

1. Attenhofer Jost CH, Connolly HM, Dearani JA, et al. Ebstein's Anomaly. Circulation, 2007, 115 (2):277-285.
2. Sata, S, Murín P, Hraška V. Cone reconstruction of Ebstein's anomaly in a neonate. Ann Thorac Surg, 2012, 94 (4):e99-e100.
3. Badiu CC, Schreiber C, Hörer J, et al. Early timing of surgical inter-vention in patients with Ebstein's anomaly predicts superior longterm outcome. Eur J Cardiothorac Surg, 2010, 37 (1):186-192.
4. Reemtsen BL, Fagan BT, Wells WJ, et al. Current surgical therapy for Ebstein's anomaly in neonates. J Thorac Cardiovasc Surg, 2006, 132 (6):1285-1290.
5. Knott-Craig CJ, Goldberg SP, Overholt ED, et al. Repair of neonates and young infants with Ebstein's anomal and related disorders. Ann Thorac Surg, 2007, 84 (2):587-593.
6. Dearani JA. Behavior of Ebstein's anomaly. Cardiology, 2010, 117 (2):88-89.
7. Frescura C, Angelini A, Daliento L, et al. Morphological aspects of Ebstein's anomaly in adults. Thorac Cardiov Surg, 2000, 48 (4):203-208.
8. Legius B, Van De Bruaene AV, Van Deyk K, et al. Behavior of Ebstein's anomaly:single-center experience and midterm follow-up. Cardiology, 2010, 117 (2):90-95.
9. Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg Summer, 2005, 17 (2):148-154.
10. Chauvaud S. Ebstein's malformation. Surgical treatment and results. Thorac Cardiov Surg, 2000, 48 (4):220-223.
11. Hebe J. Ebstein's anomaly in adults:arrhythmias:diagnosis and therapeutic approach. Thorac Cardiovasc Surg, 2000, 48 (4):214-219.
12. Boston US, Dearani JA, O'Leary PW. Tricuspid valve repair for Ebstein's anomaly in young children:a 30-year experience. Ann Thorac Surg, 2006, 81 (2):690-696.
13. Bichel DP, Mora BN, Mathewson JW, et al. Modified technique for the surgical treatment of severe tricuspid valve deformity in Ebstein's anomaly. Ann Thorac Surg, 2007, 83 (2):678-680.
14. 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):220-228.
15. Viola N, Vettukattil JJ, Haw MP. Restoring anterior leaflet continuity:the Spinnaker repair of Ebstein's anomaly. Ann Thorac Surg, 2011, 92 (2):752-754.
16. Brown ML, Dearani JA, Danielson GK, et al. The outcomes of opera-tions for 539 patients with Ebstein's anomaly. J Thorac Cardiovasc Surg, 2008, 135 (5):1120-1136.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Clinical Features and Surgical Strategy of Ebstein's Anomaly in Adults

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content