Individualized Surgical Treatments for Children with Ebstein Anomaly
31 March 2016
17 June 2016
10 August 2016 (online)
Objective Ebstein anomaly is a rare type of tricuspid malformation. The present surgical methods to resolve this anomaly include tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement. The purpose of this study was to evaluate the short- and midterm outcomes of different surgical treatments among children with Ebstein anomaly.
Methods This was a retrospective study of 136 Ebstein anomaly patients undergoing surgery at our institution from January, 2006 to August, 2015. This cohort included 118 patients receiving tricuspid valvuloplasty, 14 patients receiving palliative surgery and 4 patients receiving tricuspid valve replacement.
Results There were two in-hospital deaths and one delayed death 6 months after bidirectional cavopulmonary shunt during a second-stage operation; thus, the mortality rate was 2.2% (3/136). The follow-up variables included echocardiography, chest radiography, oxygen saturation, and cardiac function. After a mean follow-up duration of 35.8 ± 16.5 months (range: 6–98 months), all survivors had no indications for reoperation; the oxygen saturation following radical surgery and palliative surgery was 95 to 100% and 85 to 95%, respectively. Most of the patients exhibited an improved New York Heart Association (NYHA) functional class from III or IV preoperatively to I or II at follow-up; only two patients who underwent the Fontan procedure continued to exhibit NYHA functional class III. In most patients, tricuspid regurgitation (TR) degree decreased from moderate or severe preoperatively to mild or moderate at follow-up and only six patients continued to exhibit severe TR at follow-up.
Conclusions Individualization of surgical methods based on different indications, including age, associated malformations, tricuspid anatomy, cardiac function, and intraoperative status, could effectively improve the outcomes of Ebstein anomaly patients.
- 1 Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69 (4, Suppl) S106-S117
- 2 Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation 2007; 115 (02) 277-285
- 3 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 (01) 215-223
- 4 Dearani JA, Said SM, O'Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg 2013; 95 (01) 220-226 , discussion 226–228
- 5 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 (01) 50-58
- 6 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 (01) 38-45
- 7 Dearani JA, Said SM, Burkhart HM, Pike RB, O'Leary PW, Cetta F. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. Ann Thorac Surg 2013; 96 (01) 202-208 , discussion 208–210
- 8 Anderson HN, Dearani JA, Said SM. , et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis 2014; 9 (03) 266-271
- 9 Ibrahim M, Tsang VT, Caruana M. , et al. Cone reconstruction for Ebstein's anomaly: Patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg 2015; 149 (04) 1144-1150
- 10 Lange R, Burri M, Eschenbach LK. , et al. Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg 2015; 48 (02) 316-320 , discussion 320–321
- 11 Kim S, Al-Radi O, Friedberg MK. , et al. Superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair: 38-year follow-up. Ann Thorac Surg 2009; 87 (05) 1475-1482 , discussion 1482–1483
- 12 Raju V, Dearani JA, Burkhart HM. , et al. Right ventricular unloading for heart failure related to Ebstein malformation. Ann Thorac Surg 2014; 98 (01) 167-173 , discussion 173–174
- 13 Quinonez LG, Dearani JA, Puga FJ. , et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007; 133 (05) 1303-1310
- 14 Malhotra SP, Petrossian E, Reddy VM. , et al. Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly. Ann Thorac Surg 2009; 88 (06) 1975-1981 , discussion 1981
- 15 Liu J, Qiu L, Zhu Z, Chen H, Hong H. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg 2011; 141 (05) 1178-1183
- 16 Raikhelkar J, Lin HM, Neckman D, Afonso A, Scurlock C. Isolated tricuspid valve surgery: predictors of adverse outcome and survival. Heart Lung Circ 2013; 22 (03) 211-220
- 17 Davies RR, Pasquali SK, Jacobs ML, Jacobs JJ, Wallace AS, Pizarro C. Current spectrum of surgical procedures performed for Ebstein's malformation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg 2013; 96 (05) 1703-1709 , discussion 1709–1710
- 18 Said SM, Burkhart HM, Schaff HV, Johnson JN, Connolly HM, Dearani JA. When should a mechanical tricuspid valve replacement be considered?. J Thorac Cardiovasc Surg 2014; 148 (02) 603-608