Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up

Umar S. Boston, Steven P. Goldberg, Kent E. Ward, Edward D. Overholt, Thomas Spentzas, Thomas Chin, Christopher J. Knott-Craig

Research output: Contribution to journalArticle

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Abstract

Objective: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. Methods: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. Results: Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. Conclusions: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.

Original languageEnglish (US)
Pages (from-to)1163-1169
Number of pages7
JournalJournal of Thoracic and Cardiovascular Surgery
Volume141
Issue number5
DOIs
StatePublished - May 1 2011

Fingerprint

Ebstein Anomaly
Pulmonary Atresia
Newborn Infant
Survival
Tricuspid Valve
Reoperation
Tricuspid Valve Insufficiency
Ventricular Heart Septal Defects
Survivors
Echocardiography
Outcome Assessment (Health Care)
Weights and Measures

All Science Journal Classification (ASJC) codes

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

Cite this

Boston, U. S., Goldberg, S. P., Ward, K. E., Overholt, E. D., Spentzas, T., Chin, T., & Knott-Craig, C. J. (2011). Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up. Journal of Thoracic and Cardiovascular Surgery, 141(5), 1163-1169. https://doi.org/10.1016/j.jtcvs.2011.01.029
Boston, Umar S. ; Goldberg, Steven P. ; Ward, Kent E. ; Overholt, Edward D. ; Spentzas, Thomas ; Chin, Thomas ; Knott-Craig, Christopher J. / Complete repair of Ebstein anomaly in neonates and young infants : A 16-year follow-up. In: Journal of Thoracic and Cardiovascular Surgery. 2011 ; Vol. 141, No. 5. pp. 1163-1169.
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abstract = "Objective: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. Methods: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. Results: Early survival was 78.1{\%} (25/32). There was 1 late death. Fifteen-year survival estimate was 74{\%} ± 8{\%}. For patients with pulmonary atresia, early and late survival was 60{\%} ± 12{\%} (9/15) versus 94.1{\%} (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60{\%} ± 12{\%} versus 85{\%} ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6{\%} (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74{\%} ± 10{\%} for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. Conclusions: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.",
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Boston, US, Goldberg, SP, Ward, KE, Overholt, ED, Spentzas, T, Chin, T & Knott-Craig, CJ 2011, 'Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up', Journal of Thoracic and Cardiovascular Surgery, vol. 141, no. 5, pp. 1163-1169. https://doi.org/10.1016/j.jtcvs.2011.01.029

Complete repair of Ebstein anomaly in neonates and young infants : A 16-year follow-up. / Boston, Umar S.; Goldberg, Steven P.; Ward, Kent E.; Overholt, Edward D.; Spentzas, Thomas; Chin, Thomas; Knott-Craig, Christopher J.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 141, No. 5, 01.05.2011, p. 1163-1169.

Research output: Contribution to journalArticle

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T1 - Complete repair of Ebstein anomaly in neonates and young infants

T2 - A 16-year follow-up

AU - Boston, Umar S.

AU - Goldberg, Steven P.

AU - Ward, Kent E.

AU - Overholt, Edward D.

AU - Spentzas, Thomas

AU - Chin, Thomas

AU - Knott-Craig, Christopher J.

PY - 2011/5/1

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N2 - Objective: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. Methods: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. Results: Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. Conclusions: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.

AB - Objective: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. Methods: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. Results: Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. Conclusions: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.

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