Search

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., (Copyright © 2022 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Purpose: Patients with Ebstein anomaly (EA) have a variety of clinical manifestation. The assessment of structural and geometric characteristics of the heart is important for optimal management., Methods: We retrospectively analyzed echocardiography database from 2009 to 2020. We evaluate patients in two groups: patients with EA were in Group 1 and children without cardiovascular pathology were in Group 2. All children in both groups underwent echocardiography according to American Society of Echocardiography recommendations. The shape of the heart chambers and their function were studied in both groups., Results: There were 153 in Group 1 and 2000 children without cardiovascular disease in Group 2. It was shown that in children with EA, the shape of the ventricle became less spherical, which was accompanied by a decrease in myocardial mass, and the ejection fraction was reduced 34% of patients. The functional volume (non-atrialized part) of the right ventricle in patients with EA was reduced, and its contractility was preserved in 62% of cases. Preservation of the contractile properties of the right ventricle in most cases was associated with higher systolic pressure in its cavity., Conclusion: TAPSE, TESV, and the velocity of the annulus fibrous ring movement according to tissue dopplerography in patients with EA do not allow us to assess the contractility of the right ventricle. The myocardial performance index (MPI) characterizes a decrease in the functional volume of the right ventricle., (© 2022 Wiley Periodicals LLC.)

Objective: To define the impact of tricuspid valve cone reconstruction (CR) on ventricular performance in Ebstein anomaly, both independently and after stem cell therapy., Patients and Methods: The control group included 257 patients who had CR between June 2007 and December 2019. Ten subjects of a phase I stem cell therapy trial (May 2017 - March 2019) were compared with the controls to assess the echocardiographic impact on ventricular remodeling., Results: After CR, right ventricular (RV) size decreased and left ventricular (LV) volume increased in all patients. Apical and biplane RV fractional area change (FAC) initially decreased, but rebounded by 6 months postoperation. Short-axis FAC increased early and was maintained at 6 months post-CR in the control group. At 6 months post-CR, cell therapy patients showed a significantly larger increase in short-axis FAC (24.4% vs 29.9%, P=.003). In addition, whereas LV ejection fraction (EF) was unchanged at 6 months post-CR in controls, cell therapy patients showed a significant increase in EF relative to baseline and to controls (55.6% vs 65.0%, P=.007)., Conclusion: Cone reconstruction reduces tricuspid regurgitation and RV size, but is also associated with increased RV FAC and LV volume. Furthermore, injection of bone marrow-derived stem cells augmented the increase in RV FAC and was associated with improved LV EF at 6 months post-CR. This is evidence of a favorable interventricular interaction. These findings provide motivation for continued investigation into the potential benefits of stem cell therapy in Ebstein anomaly and other congenital cardiac malformations., Trial Registration: clinicaltrials.gov identifier: NCT02914171., (Copyright © 2021 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly., Methods: We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning., Results: Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed "at risk" for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time., Conclusion: Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.

Background: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established., Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS., Materials and Methods: A retrospective review of all patients <21 years of age with EA who had a CR at Mayo Clinic from June 2007 to December 2015 was performed. Surveys were mailed and telephone calls were made to all individuals to assess antiarrhythmic medication use and EP/device procedures performed after CR., Results: There were 143 patients; median age, 10 years (0.1-20.9 years). Thirty-five (24%) patients had a preoperative EPS of which 26 (18%) had a preoperative ablation. Indications for EPS were Wolff-Parkinson-White (WPW), documented arrhythmia, or suspected arrhythmia. Posthospital discharge data were available for 140 (98%) patients. Mean follow-up was 2.9 years (0.1-9.2 years). At follow-up, 7 (5%) patients were receiving antiarrhythmic medications. After CR, only 3 (2%) patients who did not have a preoperative EPS have required an ablation., Conclusions: The risk of arrhythmia after CR for EA in young patients is very low when a preoperative EPS is limited to those with WPW, known arrhythmia, or suspected arrhythmia. In smaller patients, it may be reasonable to defer the EPS., (© 2018 Wiley Periodicals, Inc.)

The cone reconstruction technique, first described by da Silva and modified by Dearani and by others, has become the repair method of choice in patients with Ebstein anomaly of the tricuspid valve. This report details the outcome of the modified cone reconstruction technique in 6 children who underwent surgical correction of Ebstein anomaly at the Tomsk Institute of Cardiology in Siberia. From 2012 through 2015, 4 boys and 2 girls (age range, 11 mo-12 yr) underwent surgery to correct Ebstein anomaly. All had presented with cyanosis, exertional dyspnea, fatigue, or new-onset atrial arrhythmia, and none had undergone previous cardiac surgery. All survived the operation. One patient needed tricuspid valve replacement with a bioprosthesis after early breakdown of the cone reconstruction. As of December 2016, all the patients had no symptoms, tricuspid stenosis, or arrhythmia. This series indicates that cone reconstruction-the most anatomic repair technique for the dysmorphic Ebstein tricuspid valve-can be successfully performed in pediatric heart centers with a large experience.

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: 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.)

Background: Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique., Methods: Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20%) had prior bidirectional cavopulmonary anastomosis (BDCPA). Preoperative heart failure was present in 8 patients (40%). Recurrent tricuspid regurgitation (TR) was due to incomplete leaflet coaptation with tethered anterior leaflet in all patients; and 10 patients (50%) had diminutive septal leaflet. Prior repair techniques consisted of annuloplasty maneuvers in all patients with no or incomplete surgical delamination in all patients., Results: The TV re-repair using cone technique was possible in all patients. Plication of atrialized right ventricle was done in 6 patients (30%). Modifications included annuloplasty band in 16 patients (80%) and TV leaflet augmentation in 8 (40%). The BDCPA was performed in 3 patients (15%) and modified Cox-maze procedure was done in 10 (50%). There were no mortalities or reoperations. Mean follow-up was 7.7 ± 10.7 months; during follow-up, 18 patients had no or mild TR and 2 had moderate TR., Conclusions: TV re-repair in EM using the cone technique is feasible, particularly when there has been no surgical leaflet delamination at initial operation. Leaflet augmentation facilitates and annuloplasty band stabilizes a competent TV re-repair. Longer follow-up is required to assess durability., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction., Methods: Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%)., Results: All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01)., Conclusions: The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Our objective was to determine the relative merits of using a bioprosthetic porcine valve (BPV) versus a mechanical valve (MechV) when tricuspid valve (TV) replacement is required in patients with Ebstein anomaly. From 1972 to 2006, 333 patients received a BPV and 45 received a MechV. Patient records were reviewed, vital status ascertained, and all patients not known to be deceased were mailed a medical questionnaire or contacted by telephone. Early mortality was not statistically higher for patients who had a MechV (11%) than for those who had a BPV (5%) inserted in the TV position (p = 0.173). The only independent preoperative predictor of operative mortality was moderate to severe left ventricular dysfunction (odds ratio 3.1, p = 0.03); 20-year survival was better in patients who had a BPV (75%) than for those who had a MechV (43%, p = 0.003). On multivariate analysis, after adjusting for ablation of accessory pathways, sinus rhythm at dismissal, and concomitant repair of pulmonary valve stenosis, a BPV remained a predictor of late survival (hazard ratio 0.42, p = 0.004). Survival free of reoperation on the TV at 20 years postoperatively was similar for patients who had a MechV (49%) compared with those who had a BPV (42%) inserted (p = 0.941). A greater percentage of patients who had a MechV reported endocarditis (12% vs 2%), bleeding requiring hospitalization (6% vs 3%), and thrombosis (12% vs 6%); however, none of these differences were statistically significant. In conclusion, a BPV in the tricuspid position was an independent predictor of improved survival. This may be related to the higher incidence of bleeding and thrombotic complications in the patients with MechVs or may be related to differences between the 2 groups. A BPV may offer superior late survival when compared with a MechV when TV replacement is required in patients with Ebstein anomaly, but patient selection must be individualized.

Our objective was to examine the outcomes of patients with left ventricular (LV) dysfunction who underwent operation for Ebstein anomaly. From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly underwent operation at Mayo Clinic. LV function was determined by echocardiography. Of the 495 patients with preoperative echocardiographic assessment of LV function, 50 had moderate or severe LV systolic dysfunction. In patients with LV dysfunction, the tricuspid valve (TV) was repaired in 12 patients and replaced in 36 patients; 1 patient had a 1.5 ventricle repair, and 1 patient had cardiac transplantation. There were 5 early deaths (10%). LV function improved in all but 4 patients after operation. In no patient did LV function worsen after operation. The 1-, 5-, and 10-year survival was 86%, 77%, and 67%, respectively. On univariate analysis, absence of sinus rhythm at dismissal (p = 0.003) was associated with greater overall mortality. For the entire cohort of 539 patients, LV dysfunction was independently predictive of late mortality (hazard ratio 3.76, p <0.001). At late follow-up (mean 6.9 years), 86% of patients were in New York Heart Association class I or II. In conclusion, LV systolic dysfunction occurs infrequently in patients with Ebstein anomaly and is a risk factor for increased late mortality. Although early mortality is greater in patients with LV dysfunction, the late results are favorable. Decreasing LV function should be an indication to promptly restore TV competence rather than a contraindication to TV operation.

Objectives: The objective of this study was to review the long-term functional outcome of patients with Ebstein anomaly who had cardiac operation at our institution., Background: Ebstein anomaly is a spectrum of tricuspid valvular and right ventricular dysplasia. Many patients will require operation in an attempt to improve quality of life., Methods: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly underwent 604 cardiac operations at the Mayo Clinic in Rochester, Minnesota. Patient records were reviewed, and all patients known to still be alive were mailed a medical questionnaire or contacted by telephone., Results: At the initial operation at our institution, the mean age of the patients was 24 years (range 8 days to 79 years) and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Surveys were returned by 285 of 448 (64%) patients known to be alive at the time of this study. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. One hundred three patients (36%) reported an incident of atrial fibrillation or flutter, 5 patients (2%) reported having had endocarditis, and 1 patient (<1%) reported having a stroke. There were 275 pregnancies among 82 women. The recurrence of congenital heart disease was reported in 9 of 232 (3.9%) liveborn children., Conclusions: Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly. Atrial arrhythmias are common both before and after surgery. Many patients have had one or more successful pregnancies with a low-recurrence risk of congenital heart disease.

Objective: Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution., Methods: Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded., Results: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations. The mean age at the time of the initial operation at our institution was 24 years (range, 8 days-79 years). Three hundred seventeen of the patients were female. One hundred forty-three (26.5%) patients had a prior invasive cardiac procedure before coming to Mayo Clinic. At the time of the first operation at Mayo Clinic, 182 patients had tricuspid valve repair, and 337 had tricuspid valve replacement. The 30-day mortality was 5.9% for the entire cohort (2.7% after 2001). Late survival was 84.7% at 10 years and 71.2% at 20 years. In a multivariate analysis of overall mortality for the patients' first operation at Mayo Clinic, increased hematocrit values, pulmonary valve stenosis, tricuspid valve replacement, absence of ablation of an accessory pathway, miscellaneous arrhythmia procedure, branch pulmonary artery enlargement, need for mechanical support postoperatively, emergency chest opening in the intensive care unit, and absence of sinus rhythm at dismissal were all predictive of mortality. When only preoperative characteristics were included, increased hematocrit values, mitral valve regurgitation requiring surgical intervention, prior cardiac procedure, and moderate-to-severe to severe reduction in right ventricular systolic function were associated with mortality. Preoperative sinus rhythm and an accessory pathway were associated with survival. Patients rated their health as excellent or good (New York Heart Association class I or II) in 83% of surveys returned., Conclusion: Ebstein anomaly can be surgically treated with low perioperative mortality. Both tricuspid valve repair and tricuspid valve replacement are associated with good long-term survival. Risk factors for poorer outcome included right, and/or left ventricular systolic dysfunction; increased hemoglobin/hematocrit values; male sex; right ventricular outflow tract obstruction; or hypoplastic pulmonary arteries.

Assessment of ventricular systolic function has been based on the geometric models of ventricular shape. This study was designed to define normal values for a nongeometric myocardial performance index (MPI) in children and to evaluate the utility of MPI in congenital heart disease. The MPI measures the ratio of total time spent in isovolumic activity (isovolumic contraction time and isovolumic relaxation time) to the ejection time. The right ventricular (RV) and left ventricular (LV) MPI were measured in 152 normal children (ages 3 to 18 years) and 45 preoperative patients with Ebstein anomaly (age 1 week to 52 years). In normal children, the RV MPI was 0.32+/-0.03 and the LV MPI was 0.35+/-0.03. In the Ebstein group, both RV and LV MPI were abnormally increased compared with age-matched normal subjects (Ebstein group: RV MPI=0.49+/-0.12, LV MPI=0.42+/-0.09, P < .001). Increasing RV dysfunction was associated with progressively increasing (abnormal) values of RV MPI (P < .001). The myocardial performance index quantitatively reflects ventricular performance in patients with complex ventricular geometry (ie, Ebstein anomaly). In the absence of a geometric solution, this nongeometric index is particularly appealing for the assessment of RV or LV performance.