Korean Circ J.  2016 Sep;46(5):706-713. 10.4070/kcj.2016.46.5.706.

Long-Term Results after Surgical Treatment of Ebstein's Anomaly: a 30-year Experience

• Kim MS
• Lim HG
• Kim WH
• Lee JR
• Kim YJ

• KMID: 2389629
• DOI: http://doi.org/10.4070/kcj.2016.46.5.706

Abstract

BACKGROUND AND OBJECTIVES
The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly.
SUBJECTS AND METHODS
Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively. Regarding the type of surgical treatment, bi-ventricular repair (n=38), one-and-a half ventricular repair (n=5), and single ventricle palliation (n=5) were performed. Of 38 patients who underwent a bi-ventricular repair, TV repairs were performed by Danielson's technique (n=20), Carpentier's technique (n=11), Cone repair (n=4), and TV annuloplasty (n=1). Two patients underwent TV replacement. Surgical treatment strategies were different according to Carpentier's types (p<0.001) and patient's age (p=0.022).
RESULTS
There were 2 in-hospital mortalities (4.2%; 1 neonate and 1 infant) and 2 late mortalities during follow-up. Freedom from recurrent TV regurgitation rates at 5, 10, and 15 years were 88.6%, 66.3%, 52.7%, respectively. TV regurgitation recurrence did not differ according to surgical method (p=0.800). Survival rates at 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively, and freedom from reoperation rates at 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively.
CONCLUSION
Surgical treatment strategies were decided according to Carpentier's type and patient's age. Overall survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Approximately 25% of patients required a second operation for TV during the follow-up.

Keyword

Heart defects, congenital; Cyanosis; Outcomes

MeSH Terms

Classification
Cyanosis
Ebstein Anomaly*
Follow-Up Studies
Freedom
Heart Defects, Congenital
Hospital Mortality
Humans
Infant, Newborn
Methods
Mortality
Recurrence
Reoperation
Survival Rate
Thoracic Surgery
Tricuspid Valve

Figure

• 

  Fig. 1 Comparison of severity of TR. Number of patients according to the severity of TR before and after the repair is shown in graph. The severity of TR was also compared according to the Carpentier's type. TR: tricuspid regurgitation.

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  Fig. 2 Kaplan-Meier estimated overall freedom from recurrent TR (A). Freedom from recurrent TR rates at 5, 10, and 15 years were 88.6%, 66.3%, and 52.7%, respectively. Kaplan-Meier estimated freedom from recurrent TR according to surgical strategies (B). Log-rank test showed no significant difference in recurrent TR rates between surgical strategies (Danielson repair, Carpentier repair, and cone repair). TR: tricuspid regurgitation.

• 

  Fig. 3 Kaplan-Meier estimated survival after surgical repair of Ebstein's anomaly.

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  Fig. 4 Kaplan-Meier estimated freedom from reoperations after initial surgical repair of Ebstein's anomaly.

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