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Background: Ebstein's anomaly is a rare complex congenital heart disease, first described in 1866 by physician Wilhelm Ebstein, characterized by anatomical and functional malformations of the tricuspid valve and the right ventricle because of inadequate delaminization of the tricuspid valve tissue. By presenting a clinical case and reviewing the literature, we analyzed the approach of an adult patient with Ebstein anomaly with ventricular preexcitation., Clinical Case: We describe the case of a 34-year-old male patient, with a clinical history of palpitations and dyspnea, and his paraclinics documented Ebstein type B anomaly associated with patent foramen oval and paroxysmal supraventricular tachycardia, Successful radiofrequency ablation was performed and tricuspid valvuloplasty was proposed., Conclusion: It is concluded that Ebstein's anomaly is complex with clinical, morphological and physiopathological heterogeneous spectrum. Manifested from severe symptomatic forms in the neonatal period to asymptomatic or minimally symptomatic ones detected incidentally. Its diagnosis is based on clinical suspicion and confirmed by echocardiography or imaging studies. The therapeutic strategy may be based on medical management in its mild asymptomatic forms up to surgical interventions that include tricuspid valvuloplasty, palliative surgery, valve replacement or cardiac transplantation., (© 2022 Revista Medica del Instituto Mexicano del Seguro Social.)

Background: Ebstein's anomaly (EA) is a congenital heart disorder characterized by abnormal function of the tricuspid valve. There are several ways to study tissue composition using magnetic resonance imaging (MRI). One of the most accurate methods is strain calculation using the feature tracking (FT) technique. Due to the novelty of the FT technique in cardiac magnetic resonance (CMR) imaging, there is a lack of comprehensive guidelines to conduct FT-MRI and to present a quantitative report. The current study is aimed to evaluate the FT technique in EA patients and to compare the obtained numerical values with those of healthy individuals., Methods: A total of 33 individuals were enrolled in a study conducted in 2018-2019 at Shahid Rajaei Hospital, Tehran, Iran. Radial, longitudinal, and circumferential strain patterns of the left and right ventricles were determined in both the patients and the controls using the FT technique. Data were analyzed using SPSS software, version 22.0., Results: The results showed a significantly lower left ventricular (LV) radial strain in EA patients compared to the control group (P=0.002). In addition, the right ventricular (RV) global longitudinal strain (GLS) in EA patients was significantly lower than in the controls (P=0.001). Other parameters (LV global longitudinal strain, RV radial strain, LV circumferential strain, and RV circumferential strain) did not differ significantly between the two groups., Conclusion: Determination of strain patterns using cardiac MRI is a promising method for the diagnosis of EA. Markers such as LV longitudinal strain and RV-GLS are the most suitable parameters for the early diagnosis of heart dysfunction., (Copyright: © Iranian Journal of Medical Sciences.)

Background: The objective of this study is to assess the safety and early impact of intramyocardial delivery of autologous bone marrow-derived mononuclear cells (BM-MNC) at time of surgical Ebstein repair., Methods: Patients with Ebstein anomaly (ages 6 months to 30 years) scheduled to undergo repair of the tricuspid valve were eligible to participate in this open-label, non-randomized phase I clinical trial. BM-MNC target dose was 1-3 million cells/kg. Ten patients have undergone surgical intervention and cell delivery to the right ventricle (RV) and completed 6-month follow-up., Results: All patients underwent surgical tricuspid valve repair and uneventful BM-MNC delivery; there were no ventricular arrhythmias and no adverse events related to study product or delivery. Echocardiographic RV myocardial performance index improved and RV fractional area change showed an initial decline and then through study follow-up. There was no evidence of delayed myocardial enhancement or regional wall motion abnormalities at injection sites on 6-month follow-up magnetic resonance imaging., Conclusions: Intramyocardial delivery of BM-MNC after surgical repair in Ebstein anomaly can be performed safely. Echocardiography variables suggest a positive impact of cell delivery on the RV myocardium with improvements in both RV size and wall motion over time. Additional follow-up and comparison to control groups are required to better characterize the impact of cell therapy on the myopathic RV in Ebstein anomaly., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: To present a case with Ebstein anomaly, a rare congenital heart disorder, with ophthalmological and neurophthalmological signs and symptoms. To date, retinal venous dilatation and visual snow syndrome have not been previously been published in this anomaly., Case Presentation: A 10-year-old white girl was diagnosed with Ebstein anomaly. From age 12, she regularly suffered from migraines with auras and photophobia. At age 16 she started to see short-term bouts of white snow, that after a year were present all day. At age 20, she was found to have a decreased retinal arteriovenous ratio., Conclusions: Retinal arterial tortuosity and venular dilatation are common in congenital heart disease but have not been described in Ebstein anomaly, nor has the visual snow syndrome., (© 2022. The Author(s).)

Background: Left ventricular dysfunction in Ebstein's anomaly (EA) is associated with higher mortality. The health of the left ventricular myocardium in children and adolescents with EA has not been investigated in detail., Methods: Patients with unrepaired EA who had undergone cardiac magnetic resonance imaging including T1 mapping were retrospectively reviewed. Patients were compared with age- and sex-matched controls. EA severity index was calculated using volumetric measurements at end diastole ([right atrial+atrialized right ventricular volumes]/[functional right ventricular+left atrial+left ventricular volumes]). Global circumferential and radial strain and as well as strain rate were examined using cardiac magnetic resonance feature tracking., Results: Twelve EA patients and an equal number of controls were included. Functional and atrialized right ventricular end-diastolic volumes were 84±15 and 21±13 mL/m 2 , respectively. Late gadolinium enhancement, confined to the right ventricle, was found in 2 patients (16%). Left ventricular native T1 values and extracellular volume fractions were higher in patients compared with controls (1026±47 versus 956±40 ms, P =0.0004 and 28.5±3.4% versus 22.5±2.6%, P <0.001, respectively). Native T1 times correlated inversely with patients' age, body surface area, and O 2 saturations (r=-0.63, -0.62, and -0.91, respectively; P =0.02, P =0.02, and P <0.0001, respectively). EA severity index ranged between 0.15 and 0.94 and correlated with T1 values (r=0.76, P =0.003). Native T1 correlated with global circumferential strain (r=0.58, P =0.04) but not ejection fraction (EF). EA patients had reduced maximum oxygen uptake (Vo 2 max). Vo 2 max correlated inversely with T1 values (r=-0.79, P =0.01)., Conclusions: Children and adolescents with EA experience an abnormal degree of diffuse myocardial fibrosis. Its association with O 2 saturation points toward a role of hypoxemia in the pathogenesis of fibrosis. Larger and prospective studies are needed to evaluate the value of T1 mapping for risk stratification and monitoring in EA.

Introduction: Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease., Aim: To describe the electrocardiographic features observed in Ebstein's disease., Methods: We conducted a retrospective descriptive study that enrolled 26 patients followed for ED., Results: The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome., Conclusion: Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.

Objectives: We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair., Methods: The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient)., Results: Thirty-nine patients underwent cone repair, 107 patients underwent other repair techniques, and 5 patients underwent valve replacement. The operative mortality was 1.3% (n = 2). Failed valve repair (defined as in-hospital death, conversion to replacement, or in-hospital reoperation) was less frequent after cone repair than after other repair techniques (5%, n = 2 vs 20%, n = 21, P = .039). Mean follow-up was 12.3 years (cone repair: 3.7 years). The 5-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation was lower after cone repair than after other repair techniques (8% vs 32%, P = .03). Among the patients undergoing other repair techniques, the 15-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation, severe tricuspid regurgitation, and reoperation was 58%, 37%, and 31%, respectively. During follow-up, 18 patients died (13 of cardiac and 5 of noncardiac causes). Among patients who died of cardiac causes, 10 of 13 had all 3 characteristics-moderate or greater tricuspid regurgitation, atrial fibrillation, and New York Heart Association classification III and IV-at their last medical evaluation., Conclusions: Before cone repair, recurrent tricuspid regurgitation was considerable. Cone repair provided a higher rate of successful repair and a lower incidence of moderate or greater recurrent tricuspid regurgitation at the midterm follow-up., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; P =0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; P =0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

Objectives: Tricuspid valve dysplasia (TVD) and Ebstein's anomaly (EA) diagnosed by fetal echocardiography vary greatly in terms of clinical severity and prognosis. The Celermajer index and Simpson-Andrews-Sharland (SAS) score have been reported previously for the prediction of prognosis in cases of TVD/EA; however, they do not take into account the hemodynamic impact of left ventricular (LV) function, which has recently been implicated as being important in the pathophysiology of TVD/EA. The aim of this study was to develop a novel scoring system that includes LV function for the prediction of perinatal death in fetuses diagnosed with TVD/EA., Methods: The clinical records of 36 fetuses diagnosed prenatally with TVD/EA between 2000 and 2015 in our hospital were reviewed. Univariate analysis was used to assess the association between perinatal death (defined as death between 22 weeks' gestation and 4 weeks after delivery) and gestational age at diagnosis, cardiothoracic area ratio (CTAR), degree of pulmonary artery flow, direction of ductal flow, right-to-left ventricular diameter ratio, tricuspid regurgitation (TR) maximum velocity, Celermajer index, SAS score and LV-Tei index. A new prognostic score, the TRIPP score (TRIcuspid malformation Prognosis Prediction score), was developed using the parameters found to be associated significantly with perinatal death. The predictive value of this score was assessed in an additional nine fetuses diagnosed with TVD/EA., Results: Thirty-six fetuses were diagnosed prenatally with TVD/EA, two of which were terminated, one was lost to follow-up and two died before 22 weeks' gestation. Of the 31 included fetuses, 10 (32%) died in the perinatal period. Univariate analysis demonstrated that TR maximum velocity was significantly lower (2.22 ± 0.17 m/s vs 3.26 ± 0.12 m/s; P < 0.001) and SAS score was significantly higher (5.7 ± 0.6 points vs 2.8 ± 0.4 points; P = 0.0014) in cases of perinatal death than in surviving fetuses. The degree of pulmonary artery flow and the direction of ductal flow were also associated significantly with perinatal death (P < 0.01 for both). Notably, LV-Tei index was significantly higher in cases of perinatal death than in surviving fetuses (0.81 ± 0.08 vs 0.50 ± 0.05; P < 0.001). In contrast, there was no significant difference in Celermajer index, CTAR or right-to-left ventricular diameter ratio. Finally, we established a novel combinatorial scoring system, the TRIPP score, including the four significant factors: TR maximum velocity, pulmonary artery flow, direction of ductal flow and LV-Tei index. The TRIPP score was found to predict efficiently perinatal mortality in fetuses with TVD/EA., Conclusions: Our novel combinatorial score of echocardiographic parameters, the TRIPP score, including LV-Tei index, is easy to measure and provides a good tool for the prediction of perinatal mortality in fetuses diagnosed prenatally with TVD/EA. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.)

Background: Ebstein's anomaly of the tricuspid valve is a rare and heterogeneous form of congenital heart defect. The purpose of this study was to review the surgical experience with Ebstein's anomaly at a single institution., Methods: This was a retrospective review of 47 patients with Ebstein's anomaly who were previously unrepaired and underwent surgical repair of the tricuspid valve. Our surgical approach repairs the leaflets at the preexisting level and does not attempt to reposition the hinge points at the anatomic annulus. The median age at surgery was 17 years (range, 2-53). Preoperatively the median degree of tricuspid regurgitation was graded as moderate-to-severe, and the median right ventricular function was slightly below normal., Results: Forty-seven patients underwent surgical repair without any mortality. Forty-three patients (91%) had a successful repair as evidenced by a decrease in the amount of tricuspid regurgitation to trace/mild. Four patients (9%) had an unsuccessful initial repair. Thirteen patients (29%) underwent a concomitant bidirectional Glenn procedure. Eight patients (17%) required late reoperation. Seven of these 8 patients initially had undergone a successful repair but returned with recurrent tricuspid regurgitation. Six of the 8 late reoperations entailed a re-repair, whereas 2 (4%) required tricuspid valve replacement., Conclusions: The bicuspidization surgical technique resulted in 91% percent of patients undergoing a successful initial repair. These results suggest this technique may be a viable alternative to the "cone" procedure., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival., Objectives: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era., Methods: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks., Results: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m 2 , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis., Conclusion: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population., (© 2019 Wiley Periodicals, Inc.)

Ripple mapping is a novel, three-dimensional, electroanatomic mapping tool that displays each electrogram at its corresponding 3-dimensional coordinate as a dynamic moving bar, which changes in length according to the electrogram voltage-time relationship. We present the case of a 43-year-old male patient with surgically repaired Ebstein's anomaly who previously underwent two unsuccessful ablation procedures for right atrial flutter (cavotricuspid isthmus and intercaval lines). Ripple mapping was decisive, enabling the arrhythmia mechanism to be appropriately recognized, and a distinction to be made between critical areas of the circuit and delayed activated bystander regions., (Copyright © 2018 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

A 26-year-old woman with a diagnosis of congenital heart disease was admitted to the hospital with exertional dyspnea, cyanotic complexion, and bilateral lower limb edema. All tests revealed isolated severe right ventricular hypertrophy. She was in poor condition, with a diminishing chance for routine surgical treatment, so she was scheduled for orthotopic homologous heart transplantation. After the operation, isolated severe right ventricular hypertrophic cardiomyopathy was confirmed histologically and pathologically. After the operation, the patient's cardiac function was normal. Exertional dyspnea, cyanotic complexion, and bilateral lower limb edema all improved postoperatively., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebstein's anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure. Use of this technique gave clear identification of the epicardial insertion points of two accessory pathways causing Wolff-Parkinson-White syndrome and resistant episodes of supraventricular tachycardia. Successful three-dimensional mapping and cryoablation were performed with a short procedure time and no complications. The patient remains without preexcitation or arrhythmia 6 months postoperatively., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Ebstein anomaly is a rare malformation of the tricuspid valve of the heart. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. Pregnant patients with Ebstein anomaly become a challenge for the physician in terms of management. We report a case in which patient delivered successfully with Ebstein anomaly. The only complication was breathlessness. Authors concluded that women with Ebstein anomaly can have successful outcomes of pregnancy with close monitoring.

Background: Ebstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes., Hypothesis: Phenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors., Methods: A comprehensive cross-sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis., Results: The most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff-Parkinson-White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities., Conclusions: This study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting., (© 2018 Wiley Periodicals, Inc.)

Aims: Atrial arrhythmias are common in patients with Ebstein's anomaly (EA) despite cardiac surgical repair and concomitant Maze procedures. We aimed to evaluate the outcome of radiofrequency catheter ablation in this group of patients., Methods and Results: All patients with EA and atrial arrhythmias who underwent catheter ablation for atrial arrhythmias between 1/1999 and 1/2016 were included. Atrial arrhythmia recurrence was identified as the primary outcome; secondary outcomes included repeat ablation, need for antiarrhythmic medications after ablation, and death. Predictors of recurrence were sought using univariate analysis. 22 patients (median age 42 years, 54.5% male) were included. Atrial flutter was the most common presenting arrhythmia (n = 14 patients, 63.5%), whereas focal atrial tachycardia (FAT) and atrial fibrillation were identified in 5 (22.7%) and 2 patients (9.1%), respectively, with both atrial flutter/fibrillation evident in a single patient 1 (4.5%). 8 patients (36.4%) had a history of right-sided maze procedures. Cavotricuspid isthmus atrial flutter (CTI-AFl) was the most commonly induced arrhythmia (n = 13, 59.1%), followed by incisional intra-atrial re-entrant tachycardia (IART; n = 4, 18.2%), and FAT (n = 4, 18.2%); 3 patients also underwent left-side ablation with concomitant pulmonary vein isolation (13.6%). 1-year and 5-year atrial arrhythmia recurrence rates were 10.0% and 41.2%, respectively. 7 patients (31.8%) underwent redo ablations, and anti-arrhythmic medication was utilized in 8 patients (36.4%) post-ablation. Neither ablation location nor echocardiographic parameters were found to be predictors of arrhythmia recurrence., Conclusion: Catheter ablation of atrial arrhythmias in patients with EA has a favorable outcome overall with an acceptable recurrence and safety profile; left-sided ablations are rarely necessary. Despite prior Maze and catheter ablation procedures, CTI-AFl and IART recurrences predominate., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Objectives: Cone reconstruction (CR) is a novel technique for surgically treating the tricuspid valve and right ventricle (RV) in patients with Ebstein's anomaly. However, precise changes in the RV function after CR remain unclear. This study aimed to evaluate the RV size and New York Heart Association (NYHA) functional class by conducting a meta-analysis of reported data., Methods: The MEDLINE, EMBASE, Cochrane Library and China National Knowledge Infrastructure databases were searched for relevant studies. The variables were RV measurements, NYHA functional classes and tricuspid valve regurgitation grades. A fixed/random effects model was used to summarize the estimates of mean difference with standard error. Sensitivity analysis was conducted to ascertain the primary origin of the heterogeneity., Results: Nine studies that involved 210 patients were included. The results demonstrated that after CR, the functional RV volume significantly decreased, NYHA functional class improved and tricuspid valve regurgitation grade decreased., Conclusions: Thus, CR appeared to be a positive approach for Ebstein's anomaly, with good results being obtained for the RV size and NYHA functional class. Because some limitations could not be overcome, studies with more data on RV and longer follow-ups are required to confirm our study results., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair., Methods: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3)., Results: All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years)., Conclusions: Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Ebstein's anomaly in combination with tetralogy of Fallot (TOF) is rare. We report a male infant with these combined anomalies who presented with severe cyanosis, heart failure, and respiratory distress after birth. Biventricular repair was successfully performed with 1-stage correction of his ventricular septal defect (VSD), right ventricular outflow tract (RVOT) obstruction, and tricuspid regurgitation. The downward displaced tricuspid valve was also restored to the normal annulus position. The infant recovered well. The surgical technique for this rare and severe anomaly is reported here., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others. Asymptomatic patients with Ebstein's anomaly can be conservatively treated and kept under close follow-up, whereas surgical operation is indicated for those patients with evidence of right heart dilation and progressively impaired ventricular systolic function. A biventricular repair consisting of the reconstruction of a competent monocuspid tricuspid valve, right ventriculorrhaphy, subtotal atrial septal defect closure, and aggressive reduction atrioplasty is suitable for most patients, and 1.5-ventricular repair (bidirectional Glenn shunt) is indicated for patients with poor right ventricular function; by contrast, heart transplantation is used in patients with severe left ventricular dysfunction., (Copyright © 2016. Published by Elsevier B.V.)

Objective: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study., Design: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed., Results: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths., Conclusions: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period., (© 2016 Wiley Periodicals, Inc.)

A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein's anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44.3 mmHg. The patient then underwent elective sectio caesaria at 30 weeks of gestational age; both the mother and her baby were alive and were in good conditions., Problem: there was an increasing breathlessness in this patient so that there was an increasing need to take a decision for her pregnancy.Ebstein's anomaly is a complicated congenital anomaly. Medical treatment may be followed for many years in patients with mild forms of Ebstein's anomaly. Surgery should be considered if there is objective evidence of debasement such as significant enlargement of heart size, reduction of systolic function in echocardiography.

A 14-year-old male with a history of symptomatic tachycardia was referred for ablation. Sinus rhythm electrocardiogram was not showing any pre-excitation. Tachycardia episode was showing antidromic tachycardia with left bundle branch block morphology. Echocardiographic examination was showing Ebstein's anomaly with septal tricuspid leaflet displaced 24mm apically. The right bundle branch block (RBBB) was concealed during sinus rhythm. The RBBB was revealed with ablation of right posterior atriofascicular accessory pathway., (Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Background: Ebstein anomaly is an uncommon congenital cardiac lesion that may be associated with cyanosis, arrhythmias and right heart dysfunction. Investigation into patient characteristics and outcomes in pregnant women with Ebstein anomaly has been limited., Aims: To characterize patient characteristics and clinical events for pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; also, to determine the effect of Ebstein anomaly on maternal clinical outcomes and individual predictors of poor outcome at time of delivery., Methods: We screened the Healthcare Cost and Utilization Project's National Inpatient Sample for hospital admissions of pregnant women for delivery (vaginal or caesarean section) in the USA from 2003-2012, and identified a cohort of 7,850,381. Clinical characteristics and maternal outcomes were identified in those with and without Ebstein anomaly. The primary outcome of interest was major adverse cardiac events (MACE), a composite of in-hospital death, acute myocardial infarction, cerebrovascular events, embolic events, cardiac complications of labour and delivery heart failure or arrhythmia., Results: Our study population consisted of 82 hospitalizations of pregnant women with Ebstein anomaly and 7,850,299 without. The Ebstein cohort more frequently had ostium secundum-type atrial septal defect and/or patent foramen ovale and anomalous atrioventricular excitation (P<0.001 for both). The MACE rate was significantly higher among Ebstein patients (P<0.001). Preterm delivery, postpartum haemorrhage and caesarean delivery occurred more frequently among the Ebstein cohort (19.5% vs 7.2%, 8.5% vs 2.8% and 47.6% vs 31.1%, respectively; P≤0.001). In a multivariable analysis, anomalous atrioventricular excitation (odds ratio [OR] 21.75, 95% confidence interval [CI] 1.03-457.91) and preterm delivery (OR 11.71, 95% CI 1.39-98.89) were associated with MACE among those with Ebstein anomaly., Conclusions: Pregnant women with Ebstein anomaly are at higher risk of MACE during pregnancy and delivery. Preterm delivery occurred more frequently in women with Ebstein anomaly., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction. Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient's apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.

Objective: We describe a repertoire of repair techniques according to type of Ebstein's anomaly to correct tricuspid valve (TV) incompetence, and report long-term ventricular function and functional outcomes., Methods: Sixty-eight patients (mean age, 26.9 ± 7.3 years) with Ebstein's anomaly (type A, n = 21; type B, n = 23; type C, n = 15; type D, n = 9) underwent correction of TV incompetence under normothermic cardiopulmonary bypass. The atrialized ventricle, TV, and subvalvar apparatus were inspected to analyze the precise morphology and determine which leaflet was the most mobile. Various repair strategies (anterior and/or posterior annulorrhapy, Sebening stitich, double-orifice valve technique, with bidirectional Glenn anastomosis if necessary) were used according to the presenting morphology and applied according to the type of Ebstein's anomaly. In all, the atrialized right ventricle (RV) was incorporated into the contractile RV by partial closure of the natural annulus using the most mobile leaflet for valve competence., Results: The mean duration of follow-up was 13.25 ± 1.3 years (median, 9.34 years; range, 1-24 years). The mean New York Heart Association class improved from 3.4 to 1.3 (P < .001). The mean severity of TV incompetence was reduced from 3.2 to 1.3 (P < .001). Exercise tolerance tests demonstrated improved maximal oxygen uptake from a mean of 15 ± 7.8 ng/kg/min preoperatively to a mean of 24.9 ± 2.0 ng/kg/min postoperatively (P < .02). Displacement tissue Doppler imaging was used to evaluate overall cardiac performance of the RV and left ventricle and interventricular septum. The mean basal, middle, and apical ventricular strain improved significantly from preoperative values of 18.08%, 15.6%, and 13.9%, respectively to postoperative values of 25.7% (P < .011), 23.7% (P < .001), and 19.36% (P < .05), respectively. Freedom from reoperation was 100% at 1 year, 98.3% at 5 years, and 92.9% at 20 years. Early mortality was 2.9%, and late mortality was 5.8%. The overall survival rate was 97.6% at 30 days, 92.7% at 5 years, and 91.26% at 20 years., Conclusions: The various repair techniques, all of which preserve the atrialized chamber and are used individually according to morphology, provide satisfactory long-term ventricular function and functional outcome even in severe types of Ebstein's anomaly., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction and Objective: Ebstein's anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola., Methods: A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects., Results: Of the 1362 patients studied, eight (0.6%) had Ebstein's anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I., Conclusion: The frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results., (Copyright © 2015 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Objective: Cone reconstruction is advocated to treat severe tricuspid valve (TV) regurgitation associated with Ebstein's anomaly. Data on postoperative clinical status, ventricular adaptation, and objective cardiopulmonary testing are lacking in these patients., Methods: The clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data from 27 consecutive cone reconstructions, undertaken from 2009 to 2013, were retrospectively compared between preoperative baseline and follow-up., Results: There were no deaths. The cone TV functioned well in all but 1 patient with late dehiscence of inferior annuloplasty sutures that were subsequently repaired. Four patients required pacemaker insertion (3 for new complete heart block). At median follow-up of 2.7 ± 1.5 years, tricuspid regurgitation was reduced in all patients, without causing stenosis. Global left ventricle function remained unchanged (pre-operative fraction 60% ± 4% vs postoperative fraction 61% ± 3%; P = .96). MRI showed enhanced forward pulmonary flow (pre 26 ± 1 mL/beat vs post 36 ± 10 mL/beat; P < .005) and increased left ventricle filling (body surface area-indexed left ventricle end-diastolic volume pre 49 ± 14 mL/m(2) vs post 60 ± 14 mL/m(2); P < .005). New York Heart Association functional class improved (pre 2.5 ± 0.6 vs post 1.3 ± 0.6; P < .0001) and there was significant improvement in peak oxygen uptake (pre 54% ± 18% vs post 66% ± 22%; P = .02)., Conclusions: Cone reconstruction of TV offers an effective repair in patients with severe regurgitation associated with Ebstein's anomaly. The patients' clinical status improved with better left ventricle filling and objective exercise capacity. The durability of repair, and mechanisms by which the ventricles adapt to the new loading conditions, need longer-term study., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Ebstein's anomaly (EA) is a rare congenital malformation, characterized by an apical displacement and dysplasia of the septal leaflet of the tricuspid valve with a right ventricular atrialization compromising its function. This malformation includes electrical conduction abnormalities, and very rarely ventricular arrhythmias. We report the case of a 22-year-old male, presenting with dizziness and palpitations. The electrocardiogram showed a sustained monomorphic ventricular tachycardia (SMVT). The presentation of the disease with ventricular tachycardia associated with hemodynamic instability is extremely rare. We placed a surgical implantable cardiac defibrillator when the diagnosis of EA, SMVT, and right ventricular systolic impairment was confirmed.

Aims: The aim of the study was to describe the complex electrophysiological features of accessory pathways (APs) in adult Ebstein's anomaly (EA)., Methods and Results: 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., Conclusions: 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., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Background: The classification of clinical severity of Ebstein anomaly still remains a challenge. The aim of this study was to focus on the interaction of the pathologically altered right heart with the anatomically-supposedly-normal left heart and to derive from cardiac magnetic resonance (CMR) a simple imaging measure for the clinical severity of Ebstein anomaly., Methods and Results: Twenty-five patients at a mean age of 26±14 years with unrepaired Ebstein anomaly were examined in a prospective study. Disease severity was classified using CMR volumes and functional measurements in comparison with heart failure markers from clinical data, ECG, laboratory and cardiopulmonary exercise testing, and echocardiography. All examinations were completed within 24 hours. A total right/left-volume index was defined from end-diastolic volume measurements in CMR: total right/left-volume index=(RA+aRV+fRV)/(LA+LV). Mean total right/left-volume index was 2.6±1.7 (normal values: 1.1±0.1). This new total right/left-volume index correlated with almost all clinically used biomarkers of heart failure: brain natriuretic peptide (r=0.691; P=0.0003), QRS (r=0.432; P=0.039), peak oxygen consumption/kg (r=-0.479; P=0.024), ventilatory response to carbon dioxide production at anaerobic threshold (r=0.426; P=0.048), the severity of tricuspid regurgitation (r=0.692; P=0.009), tricuspid valve offset (r=0.583; P=0.004), and tricuspid annular plane systolic excursion (r=0.554; P=0.006). Previously described severity indices ([RA+aRV]/[fRV+LA+LV]) and fRV/LV end-diastolic volume corresponded only to some parameters., Conclusions: In patients with Ebstein anomaly, the easily acquired index of right-sided to left-sided heart volumes from CMR correlated well with established heart failure markers. Our data suggest that the total right/left-volume index should be used as a new and simplified CMR measure, allowing more accurate assessment of disease severity than previously described scoring systems., (© 2014 American Heart Association, Inc.)

Objective: Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly., Design: Electronic medical records were reviewed for all patients < 21 years old who had surgery to repair Ebstein anomaly at Mayo Clinic Rochester between June 2007 and December 2012. Clinical data including preoperative demographics, intraoperative procedures, and postoperative outcomes were recorded., Results: Eighty-four patients initially had a cone reconstruction (54% male, mean age 10.1 ± 5.9 years). Indications for operation included cardiomegaly (42%), cyanosis (19%), and heart failure (19%). The preoperative echocardiogram demonstrated severe tricuspid regurgitation in 91% of patients. There was one early death and 3 early CR breakdowns requiring reoperation (2 re-repair, 1 tricuspid replacement). Eighty-two patients (98%) had successful CR at the time of hospital discharge. Patient age, gender, cardiopulmonary bypass time, and aortic cross-clamp time were not associated with early CR failure. Use of a partial or eccentric annuloplasty ring correlated with successful initial CR (P = .01). There have been no early CR breakdowns since 2010. Follow-up information was available for 77 patients (longest follow-up 6.5 years; mean 0.8 ± 0.2 years). The most recent postoperative echocardiogram demonstrated mild or no tricuspid regurgitation in 83%. Tricuspid stenosis (mean gradient > 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation., Conclusions: CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended., (© 2013 Wiley Periodicals, Inc.)