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. 2016 Sep;46(5):706-713.
doi: 10.4070/kcj.2016.46.5.706. Epub 2016 Sep 28.

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

Min-Seok Kim  1 , Hong-Gook Lim  1 , Woong Han Kim  1 , Jeong Ryul Lee  1 , Yong Jin Kim  1
Affiliations

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

Min-Seok Kim et al. Korean Circ J. 2016 Sep.

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.

Keywords: Cyanosis; Heart defects, congenital; Outcomes.

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Conflict of interest statement

The authors have no financial conflicts of interest.

Figures

Fig. 1
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.
Fig. 2
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
Fig. 3. Kaplan-Meier estimated survival after surgical repair of Ebstein's anomaly.
Fig. 4
Fig. 4. Kaplan-Meier estimated freedom from reoperations after initial surgical repair of Ebstein's anomaly.
See this image and copyright information in PMC

References

    1. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein's anomaly - review of a multifaceted congenital cardiac condition. Swiss Med Wkly. 2005;135:269–281. - PubMed
    1. Krieger EV, Valente AM. Diagnosis and management of ebstein anomaly of the tricuspid valve. Curr Treat Options Cardiovasc Med. 2012;14:594–607. - PubMed
    1. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation. 2007;115:277–285. - PubMed
    1. Huang CJ, Chiu IS, Lin FY, et al. Role of electrophysiological studies and arrhythmia intervention in repairing Ebstein's anomaly. Thorac Cardiovasc Surg. 2000;48:347–350. - PubMed
    1. Iturralde P, Nava S, Sálica G, Medeiros A, et al. Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway. J Cardiovasc Electrophysiol. 2006;17:1332–1336. - PubMed

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