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

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.

Contemporary outcomes and mortality risks of Ebstein anomaly: A single‐center experience in Thailand

“…Several series of EA have been reported in terms of their midterm results for management in EA, with either operations or conservative medical treatments . The indications for surgery in patients with EA have been recently published in a guideline for adults .…”

“…Several series of EA have been reported in terms of their midterm results for management in EA, with either operations or conservative medical treatments. 5,11,13,15,17 The indications for surgery in patients with EA have been recently published in a guideline for adults. 8,9 Nonetheless, certain criteria in asymptomatic patients have The perioperative mortality rate (30-day mortality postoperatively) in our center was 14%, which is comparable to the multicenter study from a Dutch pediatric heart center.…”

“…In asymptomatic patients; however, surgical repair should be considered when progressive right heart dilation or reduction of RV systolic function and progressive cardiomegaly on chest radiography are present . Intermediate term outcomes have been reported for patients with EA, who have had either an operation or a conservative medical treatment . In any case, factors that might predict a poor outcome for patients with EA remain of interest.…”

“…8,9 Intermediate term outcomes have been reported for patients with EA, who have had either an operation or a conservative medical treatment. 10,11,[13][14][15][16][17] In any case, factors that might predict a poor outcome for patients with EA remain of interest.…”

Recent Surgical Outcomes of Ebstein’s Anomaly: A Focus on Changes in Outcomes Due to Cone Repair

“…Hesitation regarding the surgical treatment for Ebstein's anomaly has mainly been due to recurrent arrhythmia after surgery and the requirement for repetitive surgery. [3][4][5][6] However, with the introduction of the cone reconstruction technique in 2007 by da Silva and colleagues, 7 more anatomic repair has become possible compared with that with the previous Carpentier technique. Ebstein's anomaly repair with Cone reconstruction is being performed in our hospital since 2008, and this study was conducted to determine whether Cone reconstruction results in better clinical outcomes than conventional repair.…”