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Ebstein’s anomaly is a rare congenital cardiac condition with limited data regarding the impact of this condition in pregnancy. The objective of this study was to describe the cardiac and perinatal outcomes associated with Ebstein’s anomaly in pregnancy. We performed a retrospective cohort of pregnancies from Epic Cosmos, an integrated dataset from over 1,500 hospitals across the United States. Charts were queried for the coexistence of pregnancy and Ebstein’s anomaly diagnosis code between July 5, 2021, and March 5, 2025. They were then compared to patients with other congenital heart disease by ICD-10 code and patients with no history of congenital heart disease. Patients with Ebstein’s anomaly were also then further stratified by cardiac arrhythmia status. Demographics and clinical characteristics were abstracted and compared utilizing student t-test, ANOVA, or Chi-square where appropriate. During the study period, 261 individuals with maternal Ebstein’s anomaly and pregnancy were identified. They were compared to 20,746 pregnant patients with other congenital heart disease and 5,048,035 pregnant patients with no history of Ebstein’s congenital heart disease. There were statistically significant differences by age, race, ethnicity, and body mass index across all 3 groups. There were also statistically significant differences in cardiac outcomes of arrhythmia and heart failure and perinatal outcomes of gestational age of at delivery and mode of delivery across groups. Approximately 41% of pregnant individuals with Ebstein’s anomaly developed a cardiac arrhythmia, while 18.4% developed heart failure. In conclusion, Ebstein’s anomaly is associated with significant cardiac risks in pregnancy, including cardiac arrhythmia and heart failure.

Background Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein’s anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein’s anomaly. Methods Forty-four consecutive patients with unrepaired Ebstein’s anomaly (34.0 ± 16.2 years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed. Results LGE was found in 10 (22.7%) patients. Typical LGE in Ebstein’s anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein’s anomaly were significantly higher than those of the controls (30.0 ± 3.8% vs. 25.3 ± 2.3%, P <  0.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction ( P  <  0.001). Conclusions Both focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein’s anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein’s severity, and worse clinical status.

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.

Prenatal severe Ebstein anomaly might be complicated by a circular shunt. In these cases, persistently elevated right atrial and venous pressure (due to severe tricuspid regurgitation) is complicated by a systemic ineffective blood shunt via a DA, resulting in diminished end-organ perfusion and acidosis, due to overall low cardiac output. Affected fetuses are at a significantly higher risk of intrauterine fetal demise. Reduction of ductal flow by prenatal treatment with nonsteroidal anti-inflammatory drugs has recently been described as a potential treatment option. However, published data are limited and management during the antenatal course is not well defined. We provide a literature review to propose a possible algorithm for prenatal assessment and initiation of treatment.

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.

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

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.

ObjectivesPatients with Ebstein’s anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes.MethodsSeventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4–10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT).ResultsCMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007).ConclusionCMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Purpose Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on current perinatal outcomes of EA/TVD in our center and to investigate clinical and fetal echocardiographic predictors of perinatal mortality. Methods and Results We performed a retrospective study among fetuses diagnosed from January 2014 to December 2023. Clinical and echocardiographic data were obtained from hospital records of Research and Education Hospital. The primary outcome was perinatal mortality. Of 21 fetuses diagnosed, there were 1 lost to follow-up, 1 termination, and 7 demises. In the live-born cohort of 12 live-born patients, 2 died before discharge, yielding an overall perinatal mortality of 50%. The median gestational age at diagnosis was 23 for non-survivors and 24 weeks for survivors. Birth weight was lower in non-survivors (2430 g vs 2990 g). Tricuspid insufficiency severity varied insignificantly. Non-survivors exhibited higher rates of hydrops, functional atresia, and absent antegrade flow ( p  < 0.05). Two infants with severe tricuspid insufficiency and congenital abnormalities died postnatally. The limited dataset enables further analysis for a predictive model. Notably, all non-survivors displayed hydrops, functional atresia, and absent antegrade flow, hindering definitive determination of the most impactful parameter on survival estimation. Conclusion Perinatal mortality remains notably elevated in fetuses with EA/TVD. The individuals at the highest risk are those with antegrade flow loss and functional atresia of pulmonary valve, this high-risk subgroup could benefit from targeted interventions, such as novel prenatal therapies or a more comprehensive perinatal approach involving optimized timing of delivery and postnatal interventional strategies.