Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle

Luis G. Quinonez, Joseph A. Dearani, Francisco J. Puga, Patrick W. O'Leary, David J. Driscoll, Heidi M. Connolly, Gordon K. Danielson

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

Objective: Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. Methods: Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5% (range 35%-72%). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair. Results: Procedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50% improved in 3 patients to greater than 50% postoperatively. Conclusions: The 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.

Original languageEnglish (US)
Pages (from-to)1303-1310
Number of pages8
JournalJournal of Thoracic and Cardiovascular Surgery
Volume133
Issue number5
DOIs
StatePublished - May 2007

Fingerprint

Ebstein Anomaly
Heart Ventricles
Fontan Procedure
Right Ventricular Function
Tricuspid Valve
Heart Transplantation
Stroke Volume
Right Ventricular Dysfunction
Multiple Organ Failure
Cardiopulmonary Bypass

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

Cite this

Quinonez, L. G., Dearani, J. A., Puga, F. J., O'Leary, P. W., Driscoll, D. J., Connolly, H. M., & Danielson, G. K. (2007). Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. Journal of Thoracic and Cardiovascular Surgery, 133(5), 1303-1310. https://doi.org/10.1016/j.jtcvs.2006.12.007

Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. / Quinonez, Luis G.; Dearani, Joseph A.; Puga, Francisco J.; O'Leary, Patrick W.; Driscoll, David J.; Connolly, Heidi M.; Danielson, Gordon K.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 133, No. 5, 05.2007, p. 1303-1310.

Research output: Contribution to journalArticle

Quinonez, LG, Dearani, JA, Puga, FJ, O'Leary, PW, Driscoll, DJ, Connolly, HM & Danielson, GK 2007, 'Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle', Journal of Thoracic and Cardiovascular Surgery, vol. 133, no. 5, pp. 1303-1310. https://doi.org/10.1016/j.jtcvs.2006.12.007
Quinonez, Luis G. ; Dearani, Joseph A. ; Puga, Francisco J. ; O'Leary, Patrick W. ; Driscoll, David J. ; Connolly, Heidi M. ; Danielson, Gordon K. / Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. In: Journal of Thoracic and Cardiovascular Surgery. 2007 ; Vol. 133, No. 5. pp. 1303-1310.
@article{4aa957973bb44f8c868c53ec5b2477b8,
title = "Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle",
abstract = "Objective: Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. Methods: Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5{\%} (range 35{\%}-72{\%}). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair. Results: Procedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50{\%} improved in 3 patients to greater than 50{\%} postoperatively. Conclusions: The 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.",
author = "Quinonez, {Luis G.} and Dearani, {Joseph A.} and Puga, {Francisco J.} and O'Leary, {Patrick W.} and Driscoll, {David J.} and Connolly, {Heidi M.} and Danielson, {Gordon K.}",
year = "2007",
month = "5",
doi = "10.1016/j.jtcvs.2006.12.007",
language = "English (US)",
volume = "133",
pages = "1303--1310",
journal = "Journal of Thoracic and Cardiovascular Surgery",
issn = "0022-5223",
publisher = "Mosby Inc.",
number = "5",

}

TY - JOUR

T1 - Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle

AU - Quinonez, Luis G.

AU - Dearani, Joseph A.

AU - Puga, Francisco J.

AU - O'Leary, Patrick W.

AU - Driscoll, David J.

AU - Connolly, Heidi M.

AU - Danielson, Gordon K.

PY - 2007/5

Y1 - 2007/5

N2 - Objective: Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. Methods: Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5% (range 35%-72%). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair. Results: Procedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50% improved in 3 patients to greater than 50% postoperatively. Conclusions: The 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.

AB - Objective: Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. Methods: Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5% (range 35%-72%). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair. Results: Procedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50% improved in 3 patients to greater than 50% postoperatively. Conclusions: The 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.

UR - http://www.scopus.com/inward/record.url?scp=34247375146&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34247375146&partnerID=8YFLogxK

U2 - 10.1016/j.jtcvs.2006.12.007

DO - 10.1016/j.jtcvs.2006.12.007

M3 - Article

VL - 133

SP - 1303

EP - 1310

JO - Journal of Thoracic and Cardiovascular Surgery

JF - Journal of Thoracic and Cardiovascular Surgery

SN - 0022-5223

IS - 5

ER -