Ebstein's Anomaly In Adult Research Articles

A simplified repair method for adult Ebstein's anomaly.

Ebstein's anomaly (EA) is a rare but difficult to manage congenital heart disease with a wide spectrum of clinical manifestations. We present a simplified repair method which combines the plication of the atrialized right ventricle, tricuspid leaflet repair and ring annuloplasty. This method is suitable for older adult EA patients with progressive right heart dysfunction symptoms. Compared with complex repair methods (such as Cone reconstruction) this simplified repair method can reduce surgical risk, and achieve mild or less tricuspid regurgitation with acceptable long-term effects compared with prosthetic valve replacement.

Percutaneous Atrial Septal Defect Closure in Adult Ebstein's Anomaly with Exertional Hypoxemia

In Ebstein's anomaly, percutaneous atrial septal defect (ASD) closure for the treatment of hypoxemia due to a right-to-left interatrial shunt remains controversial. We report the case of a 40-year-old woman with Ebstein's anomaly who developed cyanosis and shortness of breath on exercise. Her symptoms improved after percutaneous ASD closure and her clinical course has been good during follow-up. The balloon ASD occlusion test, combined with dobutamine stimulation before the procedure, is useful to confirm treatment indication. A prior electrophysiological evaluation is also important because Ebstein's anomaly is often complicated by atrioventricular recurrent tachycardia.

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

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.

Features of accessory pathways in adult Ebstein's anomaly

The aim of the study was to describe the complex electrophysiological features of accessory pathways (APs) in adult Ebstein's anomaly (EA). We performed a retrospective study of 17 consecutive adult EA cases with APs who underwent electrophysiological study and radiofrequency catheter ablation (RFCA) from November 2011 to May 2013. There were a total of 24 atrioventricular reentrant tachycardias (AVRTs) due to 23 APs, including 20 (87.0%) non-decremental conducting, 2 (8.7%) decremental conducting, and 1 (4.3%) nodofascicular bundle. Six (6/17 = 35.3%) patients had two APs while others had only one. Twenty-one APs (91.3%) in 15 patients were manifested and 2 APs (8.7%) in 2 patients were concealed. Six APs (26.1%) were broad, while 17 APs (73.9%) were narrow in width. Two patients suffered from duodromic tachycardias mediated by two APs. Accessory pathways were mainly located on the posterior, posteroseptal, and posterolateral tricuspid annulus (TA). Right ventriculography confirmed that all APs were located on the anatomic TA. All the patients remained free from tachycardias during 11.9 ± 6.8 months of follow-up after RFCA. For the 15 patients with manifest APs, 10 patients' electrocardiograms (ECGs) after RFCA demonstrated morphologies of right bundle branch block, while 5 patients' ECGs were normal. Accessory pathways in EA are predominantly right-sided, manifest and localize to the lower half of the anatomic TA. A number of APs in EA have broad widths. The incidence of multiple APs is high in these patients and RFCA is effective.

Ebstein's anomaly repair in infant and adult: Report of two cases

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. It is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Moderate to severe Ebstein's malformation remains a surgical challenge. We report our institutional experience with the surgical management of Ebstein's anomaly in a severely symptomatic four months old infant and a twenty year old grown up adult. The young infant with Ebstein's anomaly had severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly, hepatomegaly. Echocardiography severity score was 1.1 in infant (grade 3/4). Repair consisted of tricuspid valve repair, reduction annuloplasty, partial closure of atrial septal defect, and correction of all associated cardiac defects (PDA ligation).Adult Ebstein's anomaly patient underwent One and half ventricle repair which includes De-Vega repair of tricuspid valve, plication of atrialized RV , closure of ASD and a bidirectional Glenn shunt as part of the repair. The postoperative course was uneventful in adult patient except mild RV diastolic dysfunction. The infant required prolonged mechanical ventilatory support. She also developed lungs collapse-consolidation following extubation and septicaemia. At 2 years follow-up both patients showed improved exercise tolerance, infant showed significant weight gain. Echocardiographic studies reveal satisfactory monocusp valve function in both patients, with mild non- coaptation of the anterior leaflet and the septal structures with mild tricuspid valve regurgitation. Ebstein's repair has a good functional and hemodynamic outcome in infant and adult patients. One and one-half ventricular repair can be performed with an acceptable risk in adult and a biventricular repair is feasible and safe in a symptomatic infant. J Shaheed Suhrawardy Med Coll, 2011;3 (2): 48-52 DOI: http://dx.doi.org/10.3329/jssmc.v3i2.12079

A Multicenter, Long-Term Study on Arrhythmias in Children with Ebstein Anomaly

To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff–Parkinson–White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.