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Although survival has significantly improved in the last four decades, the diagnosis of Ebstein’s anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. With increasing age of presentation, appropriate timing of intervention is challenged by a wide spectrum of disease and paucity of data on patient-tailored interventional strategies. The present review sought to shed light on the wide grey zone of post-neonatal Ebstein’s manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy.A ‘wait-and-see’ approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebstein’s anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome.In order to tackle the extreme heterogeneity of Ebstein’s anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention.

BackgroundAlthough tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation.MethodsRetrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo Clinic Rochester (2000–2019) and had preoperative and postoperative cardiopulmonary exercise tests and echocardiograms. The patients were divided into aerobic capacity(+) and aerobic capacity(-) groups depending on whether they had postoperative improvement in %-predicted peak oxygen consumption (VO2).ResultsOf 76 patients with severe tricuspid regurgitation due to Ebstein anomaly, 28 (37%) and 48 (63%) were in aerobic capacity(+) and aerobic capacity(-) groups, respectively. The average improvement in peak VO2 was 2.1±1.4 mL/kg/min and −0.9±0.4 mL/kg/min in the in aerobic capacity(+) and aerobic capacity(-) groups, respectively. Although both groups had similar severity of residual tricuspid regurgitation, the aerobic capacity(+) group had more postoperative improvement in right atrial (RA) function, left atrial (LA) function and left ventricular preload and stroke volume. Of the preoperative variables analysed, RA reservoir strain (relative risk 1.12; 95% CI 1.06 to 1.18); LA reservoir strain (relative risk 1.09; 95% CI 1.04 to 1.14) and LV stroke volume index (OR 1.04; 95% CI 1.01 to 1.07) were predictors of postoperative improvement in peak VO2.ConclusionsOne-third of patients with severe tricuspid regurgitation due to Ebstein anomaly had postoperative improvement in aerobic capacity, and atrial function indices were the best predictors of postoperative improvement in aerobic capacity. These data provide new insight into the haemodynamic determinants of exercise capacity and lay the foundation for further studies to determine whether postoperative improvement in aerobic capacity translates to improved long-term survival, and whether timing of tricuspid valve surgery based on these echocardiographic indices will improve long-term outcomes.

Objectives We aimed to evaluate immediate and midterm cardiac remodeling after surgery by cardiac magnetic resonance (CMR) in Ebstein’s anomaly (EA), and also to investigate preoperative predictors of right ventricular (RV) normalization. Methods We retrospectively analyzed CMR parameters of the whole heart in adult patients with EA before surgery, at discharge and follow-up. Results A total of 26 patients were included and performed CMR at 7 days (interquartile range, 3–13 days) before surgery. Immediate postoperative CMR was finished at discharge (median: 8 [7–9] days; n = 18) and follow-up CMR at 187 days (interquartile range, 167–356 days; n = 17). RV and right atrial (RA) volumes promptly decreased immediately after surgery and at follow-up (all p < 0.05). RV ejection fraction decreased significantly at discharge ( p < 0.05) but recovered at follow-up ( p = 0.18). However, RV global longitudinal strain and RA reservoir strain were significantly impaired immediately and midterm after surgery (all p < 0.05). Indexed left ventricular (LV) end-diastolic volume, stroke volume, as well as global longitudinal strain increased from preoperative to follow-up (all p < 0.05). Patients who achieved normalization of RV volumes after surgery had smaller severity index and RV and RA volumes and higher LV ejection fraction and RA reservoir strain at baseline than patients without RV normalization (all p < 0.05). Conclusions Reverse biventricular remodeling took place in EA after tricuspid valve surgery. Tricuspid valve reconstruction should be performed before deterioration of RV volume overload and LV function to achieve reverse RV remodeling. Key Points • After removing the volume load of tricuspid regurgitation in Ebstein’s anomaly, reverse remodeling was detected by CMR in both left and right heart at midterm follow-up . • Tricuspid valve reconstruction should be performed before deterioration of RV volume overload and LV function to achieve reverse RV remodeling .

Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation. In this report, we present the results of a strategy to redirect patients utilizing the Da Silva Cone operation to achieve a 2 or 1.5 ventricle circulation. Single-center retrospective study including all consecutive cases of Da Silva Cone operation after Starnes procedure. Between 2019 and 2023, six conversions from Starnes procedure to Cone reconstruction were performed. All were critically ill before their Starnes procedure; four on extracorporeal membrane oxygenation. Two patients were successfully rerouted to a two-ventricle repair; the remainder to 1.5 ventricle circulation. RV pressure estimates showed no correlation with success. Post-Cone intensive care and hospital stays were brief, median 5 and 6 days, respectively. All are between 2.5 and 6 years old, without indications for SV palliation. There were no deaths, with follow up ranging 1 month–4 years. No repeat interventions were performed on the tricuspid valves. One subject had a surgical pulmonary valve replacement. Tricuspid regurgitation was mild in all. The Da Silva Cone operation offers successful redirection of EA patients from a SV pathway to a 1.5 or 2 ventricle pathway after Starnes procedure. The approach is feasible and durable in midterm follow-up. The decision to initially proceed with Starnes need not be an irrevocable decision to continue down a SV palliation pathway.

Background Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. Methods A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. Results Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14–57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60 o , relative risk of dehiscence was 3.2 (CI 1.3–4.9, p  = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3–100, p  < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p  = 0.04), and were older (median 38 vs 19 years, p  = 0.01). TR %, chamber area ratios and LV parameters were not different. Conclusions Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.

Ebstein's anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein's anomaly patients are limited. To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein's anomaly patients, and to determine factors affecting arrhythmia presence and mortality. Clinical data, echocardiography, treatment results, and outcomes of patients followed in our centre between 2000 and 2017 were retrospectively evaluated. A total of 79 patients (61 children, median diagnosis age: 1.5 years [1 day-24 years]) were included. Eight patients (10.1%) were deceased during the study period. Common associated anomalies were atrial septal defect/patent foramen ovale (56.9%), mitral regurgitation (25.3%), pulmonary stenosis/atresia (17.7%), and ventricular septal defect (16.5%). Genetic diseases/congenital anomalies were present in 5/3.8%. Tricuspid regurgitation was present in 75.9%, and severe in 50%. Arrhythmias were detected in 30.4%, and accessory pathway-mediated re-entrant tachycardia was the most common (67%). Wolff-Parkinson-White syndrome was present in 12.7%. Twenty-one ablation procedures (radiofrequency ablation [85.7%]/cryoablation [14.3%]) were performed in 16 patients (median age: 13.3 years [4.9-17]). Acute success/recurrence rates: 87.5/25%. Surgery was performed in 31.6% (median age: 6.5 years [4 days-29 years]), 7.6% were operated during the first month, and 12.6% during the first year. Second surgery was required in 28%. Perioperative mortality rate was 12%, and median mortality age was 25 days (1 day-17 years). Median follow-up period was 5.3 years (1 day-32 years). Older diagnosis age ( = 0.005) and mild-moderate mitral regurgitation ( = 0.036) were associated with arrhythmias. Younger age at diagnosis ( = 0.012), younger age at first surgery ( = 0.004), surgery before age three years ( = 0.037), and presence of pulmonary atresia ( = 0.000014) were associated with mortality. Gender, diagnosis age, congenital anomalies/genetic disorders, tricuspid regurgitation, arrhythmias, and surgery history did not have an independent effect on survival. In children and young adults presenting with Ebstein's anomaly, younger age at presentation and at surgery, surgery before age three years, pulmonary atresia were associated with death. Ablation procedures can be successfully performed but recurrence rate is still high.

Background In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. Methods All Ebstein anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs. Results Twenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p  < 0.001), RV end-diastolic volume (EDV) (242 ± 110 ml/m 2 vs. 137 ± 82 ml/m 2 , p  < 0.001), and RV stroke volume (SV) (101 ± 35 vs. 51 ± 7 ml/m 2 , p  < 0.001). RV EF was unchanged. Conversely, there was an increase in both LV EDV (68 ± 13 vs. 85 ± 13 ml/m 2 , p  < 0.001) and LV stroke volume (37 ± 8 vs. 48 ± 6 ml/m 2 , p  < 0.001). There was no change in LV EF, or global circumferential and longitudinal strain but basal septal circumferential strain improved (16 ± 7% vs. 22 ± 5%, p  = 0.04). LV contraction become more synchronous (dyssynchrony index: 32 ± 17 vs. 21 ± 9 msec, p  = 0.02), and the extent correlated with the reduction in RV EDV and TR. Conclusions In patients with the Ebstein anomaly, the cone operation led to reduced TR and RV stroke volume, increased LV stroke volume, improved LV basal septal strain, and improved LV synchrony. Our data demonstrates that the detrimental effect of the RV on LV function can be mitigated by the cone operation.

BackgroundEbstein’s anomaly (EA) exhibits significant anatomical and clinical heterogeneity, warranting a systematic approach to risk stratification. While the Carpentier classification (CC) is commonly employed for qualitative disease assessment, there is increasing interest in quantitative imaging parameters to personalise monitoring strategies and therapeutic interventions. We sought to evaluate the association between cardiovascular magnetic resonance (CMR) parameters, CC and symptoms of heart failure (HF). Also, we investigated whether CMR-derived markers may predict the need for bidirectional cavopulmonary anastomosis (BCPA) and the occurrence of haemodynamic complications or significant right ventricular (RV) dysfunction in the postoperative stay.MethodsThis retrospective study evaluated consecutive patients diagnosed with EA undergoing 1.5T CMR acquisition at a tertiary care centre. CC and quantitative indices were extracted from steady-state free precession sequences.ResultsIn a total population of 60 patients (53% male, median age 22 years), CMR parameters most strongly associated (p<0.001) with CC included indexed displacement of the septal (SLDi) and inferior (ILDi) leaflets, Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RV end-diastolic volume and the ratio of either atrialised or functional RV to anatomical RV. Decreasing left ventricular ejection fraction (OR 0.85, 95% CI 0.75 to 0.97, p=0.01) and increasing ILDi (OR 1.05, 95% CI 1.00 to 1.10, p=0.04) emerged as the most prominent variables associated with HF symptoms. Additionally, ILDi was significantly linked to the need for BCPA (OR 1.15, 95% CI 1.03 to 1.28), the occurrence of haemodynamic complications (OR 1.09, 95% CI 1.01 to 1.18) and significant RV dysfunction in the postoperative stay (OR 1.08, 95% CI 1.01 to 1.17).ConclusionsQuantitative CMR indices proved to be effective in distinguishing between Carpentier classes and they may be valuable in an integrated CMR-based approach to assess EA severity. Among these, ILDi reflects both the extent of tricuspid valve abnormality and RV atrialisation and may serve as a useful metric in guiding personalised therapeutic strategies.

Abstract Objectives This study compared long-term outcomes of surgical management for Ebstein’s anomaly before and after the introduction of an individualized surgical approach at our centre, including Cone repair. Methods We conducted a retrospective, single-centre analysis of patients who underwent surgery for Ebstein’s anomaly between 1988 and 2022. Since 2012, early intervention to preserve right ventricular (RV) function before severe deterioration was prioritized. Survival was characterized using Kaplan-Meier estimates, and restricted mean survival time (RMST) was computed up to a specified time horizon. Covariate-adjusted hazard ratios (HRs) were modelled using Cox proportional hazards regression with LASSO-based variable selection. Results Out of 162 patients screened, 141 (median age: 14 years; IQR: 5-33 years) were included; 74 underwent surgery before 2012 and 67 after. Patients operated after 2012 were younger (median 7, IQR 1-14 vs 22, IQR 14-44 years; P < 0.001) and more frequently received Cone repair, either alone or combined with a bidirectional Glenn procedure (BDG). Five-year survival improved progressively over the analysed periods: from 81.7% (95% CI: 63.7%-91.3%) before January 2000, to 90% (95% CI: 75.4%-96.1%) between January 2000 and August 2012, reaching 98.5% (95% CI: 89.9%-99.8%) after August 2012. The survival difference before and after 2012 was significant (RMST-based log-rank test: P = 0.0094). Cox regression identified Carpentier classification above type B (HR: 6.19; 95% CI: 1.69-22.68; P = 0.006) and postoperative sepsis within 30 days (HR: 7.75; 95% CI: 2.09-28.75; P = 0.002) as risk factors for increased mortality. Cone repair itself was not associated with increased mortality (HR: 1.01; 95% CI: 0.11-9.57; P = 0.991), whereas BDG was associated with a lower hazard (HR: 0.11; 95% CI: 0.01-0.92; P = 0.041). Conclusions An individualized strategy that favoured earlier intervention, including Cone repair with or without BDG, was associated with improved survival. These findings highlight the importance of tailoring surgical strategies to anatomical and haemodynamic status in Ebstein’s anomaly. Ebstein’s anomaly is a complex congenital heart defect characterized by the apical displacement of the tricuspid valve and malformation of the right ventricle. Graphical abstract

The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021. “Repaired” EA was defined as prior tricuspid valve repair and/or replacement (83% post-tricuspid valve replacement). Repaired patients were younger than those with unrepaired EA (41.3 ± 16.0 versus 50.6 ± 15.6 years, p  = 0.008) and had a lower prevalence of ≥ moderate native or prosthetic tricuspid regurgitation (67% versus 81%, p  = 0.01). Right atrial (RA) pressure was higher among patients with repaired EA than in unrepaired disease [13 (11; 18) versus 10 (8; 15) mmHg; p  = 0.02], but these differences were no longer present when adjusting for ≥ moderate right ventricular systolic dysfunction and ≥ moderate tricuspid regurgitation. Cardiac index (Qs) was lower among those with unrepaired EA than in repaired EA (1.9 ± 0.7 versus 2.3 ± 0.6 l/min/m 2 , p  = 0.01), even after adjusting for similar confounders. During a follow-up of 8.6 (3.2–13.3) years, 16 (18%) patients died. Systolic pulmonary artery pressure was independently associated with all-cause mortality. In summary, higher cardiac indices were found in those with repaired EA compared to those with unrepaired disease. RA hypertension was prevalent in both groups and no differences in right filling pressures were found between groups after adjusting for potential confounders. Elevation in pulmonary pressures was independently associated with survival. The use of pulmonary vasomodulators in EA requires further investigation.