Anatomic repair of Ebstein's malformation

Lessons learned with cone reconstruction

Joseph A. Dearani, Sameh M. Said, Patrick W. O'Leary, Harold M. Burkhart, Roxann D. Barnes, Frank Cetta

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Background: Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. Methods: Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%). Results: All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01). Conclusions: The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.

Original languageEnglish (US)
Pages (from-to)220-228
Number of pages9
JournalAnnals of Thoracic Surgery
Volume95
Issue number1
DOIs
StatePublished - Jan 2013

Fingerprint

Ebstein Anomaly
Tricuspid Valve Insufficiency
Reoperation
Mortality
Right Heart Bypass
Cyanosis
Learning Curve
Tricuspid Valve
Cardiomegaly

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Dearani, J. A., Said, S. M., O'Leary, P. W., Burkhart, H. M., Barnes, R. D., & Cetta, F. (2013). Anatomic repair of Ebstein's malformation: Lessons learned with cone reconstruction. Annals of Thoracic Surgery, 95(1), 220-228. https://doi.org/10.1016/j.athoracsur.2012.04.146

Anatomic repair of Ebstein's malformation : Lessons learned with cone reconstruction. / Dearani, Joseph A.; Said, Sameh M.; O'Leary, Patrick W.; Burkhart, Harold M.; Barnes, Roxann D.; Cetta, Frank.

In: Annals of Thoracic Surgery, Vol. 95, No. 1, 01.2013, p. 220-228.

Research output: Contribution to journalArticle

Dearani, JA, Said, SM, O'Leary, PW, Burkhart, HM, Barnes, RD & Cetta, F 2013, 'Anatomic repair of Ebstein's malformation: Lessons learned with cone reconstruction', Annals of Thoracic Surgery, vol. 95, no. 1, pp. 220-228. https://doi.org/10.1016/j.athoracsur.2012.04.146
Dearani, Joseph A. ; Said, Sameh M. ; O'Leary, Patrick W. ; Burkhart, Harold M. ; Barnes, Roxann D. ; Cetta, Frank. / Anatomic repair of Ebstein's malformation : Lessons learned with cone reconstruction. In: Annals of Thoracic Surgery. 2013 ; Vol. 95, No. 1. pp. 220-228.
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abstract = "Background: Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. Methods: Between June 2007 and December 2011, 89 patients (47 female; 53{\%}) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48{\%}), cyanosis in 29 (33{\%}), and heart failure in 13 (15{\%}). Prior tricuspid valve repair was performed in 12 patients (13{\%}). Severe tricuspid regurgitation (TR) was present in 75 patients (84{\%}). Results: All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64{\%}), leaflet augmentation in 28 patients (31{\%}), and autologous chordae in 17 patients (19{\%}). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24{\%}). Early mortality occurred in 1 patient (1{\%}). Early reoperation for recurrent TR occurred in 12 patients (13{\%}); re-repair was performed in 6 patients (50{\%}), and 6 (50{\%}) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87{\%}) had no or mild TR, 9 (11{\%}) had moderate TR, and 2 patients (2{\%}) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01). Conclusions: The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.",
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