Surgical Management of Ebstein Anomaly: The Australia and New Zealand Experience.

BACKGROUND: Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood. METHODS: A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed. Patients from 1985 to 2019 were included in the study. RESULTS: A total of 125 patients were included in the study, 76 patients (60%) undergoing tricuspid valve repair, of which 23 patients underwent a Cone repair and 49 (40%) had a tricuspid valve replacement. The mean follow-up was 7.9 ± 7.3 years. Postoperatively, early mortality was 3 patients (2%) and 10-year survival was 91.5%. A postoperative pacemaker was required in 24 patients (19%). Reoperation was required in 21 patients (17%). There was no statistically significant difference in survival or reoperation between patients who underwent repair or replacement of the tricuspid valve; however, with a small number of patients in long-term follow-up. CONCLUSION: Older children and adult patients undergoing surgery for Ebstein anomaly in Australia and New Zealand experience good medium-term postoperative survival. Repair of the valve is achieved in a significant proportion of patients with increasing use and success with the Cone repair technique.

A rare case of severe Ebstein's anomaly and hepatoblastoma in a neonate.

Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.

Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices.

Detailed three-dimensional cardiac segmentations using cardiac computed tomography (CT) data is technically feasible in patients with Ebstein anomaly, but its complementary role has not been evaluated. This single-center, retrospective study was aimed to evaluate the complementary role of cardiac CT ventricular volumetry in evaluating the severity of Ebstein anomaly. Preoperative cardiac CT ventricular volumetry was performed in 21 children with Ebstein anomaly. CT-based ventricular functional measures were compared between Carpentier types, and between definitive surgical repair types. The Celermajer severity index measured with echocardiography was correlated with CT-based functional parameters. Total right ventricle (RV) and functional RV (fRV) volumes, fRV fraction, fRV/left ventricle (LV) volume ratio, and end-diastolic CT severity index demonstrated statistically significant differences between Carpentier type A/B and Carpentier type C/D (p < 0.05). The Celermajer severity index measured with echocardiography showed a high positive correlation with the end-diastolic CT severity index (R = 0.720, p < 0.002). There were no statistically significant differences in both echocardiography- and CT-based functional measures between patients with biventricular repair and patients with one-and-a-half or univentricular repair (p > 0.05). Compared with echocardiography, cardiac CT ventricular volumetry can provide the severity of Ebstein anomaly objectively and may be used in select patients when echocardiographic results are inconclusive or inconsistent.

Ebstein's anomaly in children and adults: multidisciplinary insights into imaging and therapy.

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.

Cardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.

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/m2, 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.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

Tricuspid cleft or tetracuspid valve? Usefulness of three-dimensional echocardiogram in the assessment of isolated tricuspid regurgitation in pediatrics.

Tricuspid regurgitation (TR) in children may be secondary to congenital anomalies of the tricuspid valve complex which is composed by annulus, leaflets, commissures, chordae tendineae, and papillary muscles. The most common congenital cause is Ebstein's anomaly; however, there are less frequent causes such as abnormal number of tricuspid leaflets, tricuspid cleft, leaflet prolapse, double orifice tricuspid valve, and congenital tricuspid valve dysplasia. Identifying the precise cause is important to plan an appropriate repair surgery. In this article, the case of a 4-year-old patient with a tetracuspid valve with significant tricuspid regurgitation is presented and the morphological analysis was made by two-dimensional (2D) and three-dimensional (3D) transthoracic echocardiography. The morphological differences between a tetracuspid valve and a cleft of the anterior leaflet tricuspid valve are exposed. 3D echocardiographic evaluation of the tricuspid valve allowed a better understanding of the tricuspid valve anatomy, which includes evaluation of the tricuspid annulus, leaflets, commissures, and subvalvular apparatus. Recognizing the accurate cause of isolated tricuspid regurgitation allows better planning of the surgical technique.

Permanent pacemaker implantation in post one-and-a-half ventricle repair with tricuspid valve replacement in Ebstein anomaly: An innovative technique.

INTRODUCTION: Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified surgical technique for Glenn anastomosis and a combined interventional and electrophysiologic approach, the transvenous pacemaker was successfully implanted. FINDINGS AND CONCLUSIONS: We reported a novel technique of pacemaker implantation in a 27-year-old woman, underlying Ebstein anomaly of the tricuspid valve, who developed intermittent complete atrioventricular block at 5 years after surgical repair. The patient had a tricuspid valve replacement and a novel modified bidirectional Glenn anastomosis for one-and-a-half ventricle repair. The Glenn circuit was conducted by opening a window between the posterior wall of the superior vena cava (SVC) and the anterior wall of the right pulmonary artery (RPA), combined with putting a Goretex membrane in the SVC below the SVC-RPA window without disconnecting the SVC from the right atrium. The transvenous pacemaker was implanted by perforating the Goretex membrane, then passing the leads from the axillary vein through the perforated membrane and placing them in the coronary sinus and right atrium.

Early spontaneous closure of large arterial ducts in two term neonates with Ebstein anomaly after failed attempts of transcatheter closure.

In neonatal Ebstein's anomaly of the tricuspid valve, prolonged ductal patency in patients without anatomic pulmonary valve atresia can be deleterious. Circular shunts may develop in patients with different degrees of pulmonary and tricuspid insufficiency. Closure of the arterial duct may result in haemodynamic improvement in particular scenarios. The transcatheter approach is a valuable closure alternative despite some technical difficulties in large-sized arterial ducts and low birth weight neonates. Herein, we report on two consecutive term newborns with Ebstein's anomaly and large arterial ducts in whom mechanical stimulus of the arterial duct during failed attempts of transcatheter closure led after two days to definitive closure followed by good clinical outcomes.

Contemporary Early Postoperative Cone Repair Outcomes for Patients With Ebstein Anomaly.

OBJECTIVE: To describe the early postoperative outcomes after cone repair (CR) for Ebstein anomaly (EA) across the age spectrum. PATIENTS AND METHODS: For this study, 284 patients from 1 to 73 years of age who underwent CR at Mayo Clinic from June 1, 2007, to December 21, 2018, were separated by age group (1-<4, 4-<19, 19-<40, and 40+ years) and by disease severity for analysis. Outcomes of interest included death, reoperation, readmission, early postoperative complications, cardiac intensive care unit and hospital length of stay, and need for superior cavopulmonary anastomosis. RESULTS: Mortality within 30 days was 0%. The reoperation rate was 4.9% (n=14) and the median hospital length of stay was 5 days, with no statistical difference between ages at time of CR or severity groups. The readmission rate was 2% (n=6). Postoperative complications were seen in 8.8% (n=25) of cases overall, with higher rates in the youngest age group (21%, P<.001). Superior cavopulmonary anastomosis was most common in the youngest age group (37% vs 17% overall, P<.001) and in those with severe disease (35%, P<.001). CONCLUSION: Children and adults with Ebstein anomaly have very good early postoperative outcomes with a less than 10% complication and reoperation rate and very low mortality following cone reconstruction. In the setting of good and stable right ventricle function and no symptoms of heart failure or cyanosis, waiting for CR until 4 years of age may minimize early postoperative complications and need for superior cavopulmonary anastomosis.

A rare combination of Ebstein's anomaly with left ventricular outflow tract obstruction.

We describe a rare case of Ebstein's anomaly (EA) combined with left ventricular outflow tract obstruction in a 54-year-old man that was accurately identified by echocardiography, cardiac magnetic resonance imaging (CMR). The imaging result was ultimately validated by surgery. We emphasize the clinical importance of using echocardiography and CMR together to provide a thorough, noninvasive explanation of these results.

A simple-to-use nomogram for predicting prolonged mechanical ventilation for children after Ebstein anomaly corrective surgery: a retrospective cohort study.

BACKGROUND: Prolonged mechanical ventilation (PMV) after pediatric cardiac surgery imposes a great burden on patients in terms of morbidity, mortality as well as financial costs. Ebstein anomaly (EA) is a rare congenital heart disease, and few studies have been conducted about PMV in this condition. This study aimed to establish a simple-to-use nomogram to predict the risk of PMV for EA children. METHODS: The retrospective study included patients under 18 years who underwent corrective surgeries for EA from January 2009 to November 2021. PMV was defined as postoperative mechanical ventilation time longer than 24 hours. Through multivariable logistic regression, we identified and integrated the risk factors to develop a simple-to-use nomogram of PMV for EA children and internally validated it by bootstrapping. The calibration and discriminative ability of the nomogram were determined by calibration curve, Hosmer-Lemeshow goodness-of-fit test and receiver operating characteristic (ROC) curve. RESULTS: Two hundred seventeen children were included in our study of which 44 (20.3%) were in the PMV group. After multivariable regression, we obtained five risk factors of PMV. The odds ratios and 95% confidence intervals (CI) were as follows: preoperative blood oxygen saturation, 0.876(0.805,0.953); cardiothoracic ratio, 3.007(1.107,8.169); Carpentier type, 4.644(2.065,10.445); cardiopulmonary bypass time, 1.014(1.005,1.023) and postoperative central venous pressure, 1.166(1.016,1.339). We integrated the five risk factors into a nomogram to predict the risk of PMV. The area under ROC curve of nomogram was 0.805 (95% CI, 0.725,0.885) and it also provided a good discriminative information with the corresponding Hosmer-Lemeshow p values > 0.05. CONCLUSIONS: We developed a nomogram by integrating five independent risk factors. The nomogram is a practical tool to early identify children at high-risk for PMV after EA corrective surgery.

Cardiac remodeling after tricuspid valve repair in Ebstein's anomaly: a magnetic resonance study.

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.

Sequential percutaneous tricuspid and pulmonary valve implantation in a young child operated previously for Ebstein Anomaly.

We report a successful percutaneous tricuspid valve implantation followed by a percutaneous pulmonary valve implantation in a young child with Ebstein's anomaly of tricuspid valve and pulmonary stenosis who was previously treated surgically at 1 year of age with tricuspid ring annuloplasty and a transannular outflow patch. This article shows the feasibility of sequential implantation of two valves in young patients with severe tricuspid and pulmonary valve insufficiency.