J Korean Med Sci.  2016 Nov;31(11):1749-1754. 10.3346/jkms.2016.31.11.1749.

Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation

• Kim HY
• Jang SY
• Moon JR
• Kim EK
• Chang SA
• Song J
• Huh J
• Kang IS
• Yang JH
• Jun TG
• Park SW

• KMID: 2470270
• DOI: http://doi.org/10.3346/jkms.2016.31.11.1749

Abstract

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.

Keyword

Ebstein Anomaly; Clinical Outcome; Treatment; Adults

MeSH Terms

Adolescent
Adult
Aged
Arrhythmias, Cardiac/etiology
Cerebrovascular Disorders/etiology
Disease-Free Survival
Ebstein Anomaly/mortality/pathology/*surgery
Echocardiography
Female
Heart/diagnostic imaging
Heart Failure/etiology
Humans
Male
Middle Aged
Proportional Hazards Models
Reoperation
Retrospective Studies
Treatment Outcome
Young Adult

Figure

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  Fig. 1 Cumulative Event free survival curves stratified by 3 treatment strategy groups. Event free survival for MACCE was not significantly different between immediate operative group and delayed operative group but it showed significant difference between overall surgical treatment group and medical treatment group (immediate op. vs. delayed op.: P = 0.274, non-operative vs. operative: P = 0.007). MACCE = major adverse cardiovascular and cerebrovascular events, op. = operation.

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