Cone Repair Research Articles

Commentator Discussion: The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure

Commentator Discussion: The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure

The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure

BackgroundWe present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology. MethodsThis is a retrospective database analysis from 2 institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR). ResultsEleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation at a median age of 27 months (IQR, 20.5 months). All patients were critically ill before their Starnes procedure, and 4 were on extracorporeal membrane oxygenation. Before the Cone repair, the median preoperative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR, 1.3 m/s). During the Cone procedure, 9 patients required a concomitant pulmonary valve repair, of whom 3 needed a transannular monocusp patch. Four patients were successfully rerouted to a 2-ventricle repair, and 7 patients with a previous Glenn achieved 1.5-ventricle circulation. There were no cases of heart block and no deaths. Seven patients had trivial, 3 patients had mild, and 1 patient had moderate tricuspid regurgitation (TR) at a median follow-up of 11 months (IQR, 21.5 months). There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in 1 patient. ConclusionsAfter the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TR at a midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single-ventricle physiology.

Repair of Ebstein Anomaly in Children: Avoiding Pitfalls

Ebstein anomaly (EA) is a congenital heart disease, which main feature is the rotational displacement of the tricuspid valve (TV) into the right ventricle (RV). Tricuspid regurgitation and RV dysfunction are common findings. The Cone procedure is a well-recognized technique for anatomical repair of EA. The basic principles of Cone repair are mobilization of all available TV leaflets and construction of a cone-like valve that is reattached to the normal atrioventricular junction. Since creation of the technique in 1993, we have introduced many technical refinements to improve TV performance, prevent cardiac arrhythmias, and improve RV function. Here, we provide some surgical details to avoid atrioventricular block and coronary compromise, facilitate patient recovery, and provide sustainable long-term results. From 2016, we have applied these refinements to 110 consecutive patients at our institution, including those with previous Starnes procedure or valve replacement, resulting in no mortality, very low morbidity, and excellent valve function.

Modified Starnes Procedure With Patch Occlusion of the Main Pulmonary Artery and Other Technical Modifications to Facilitate Subsequent Biventricular Repair of Ebstein Anomaly

The Starnes procedure, introduced in 1991, has been a valuable advancement in the surgical treatment of critically ill neonates with Ebstein's anomaly (EA). However, it used to be followed by the single ventricle pathway, which presents long-term outcome limitations. In 2019, this management concept was modified by taking down the Starnes procedure and performing the Cone repair of the tricuspid valve, which resulted in a biventricular reconstruction. This approach rehabilitates the right ventricle, likely improving the patients' long-term outcomes. However, it requires modifications in the classical Starnes operation and observing certain surgical details. In cases of circular shunting, we use a patch to occlude the pulmonary artery instead of dividing and oversewing it, simplifying the succeeding pulmonary artery reconstruction. We use a PTFE fenestrated patch placed in a supra-valvar position, preserving the underneath tricuspid valve integrity, facilitating the tricuspid valve repair, and decreasing the possibility of heart block.Central message: In the current era of biventricular repair after the Starnes procedure, using a non-stick supravalvar patch to exclude the tricuspid valve and preserving the pulmonary valve structure by patch occlusion of the main pulmonary artery instead of division and oversewing it are valuable modifications of the classic Starnes operation to optimize a future biventricular repair.

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

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. 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. 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. 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.

Kiosk 2R-TC-08 - Smaller Functional Right Ventricular Mass Predicts Need for Bidirectional Glenn Shunt After Cone Repair for Patients with Ebstein Anomaly

Kiosk 2R-TC-08 - Smaller Functional Right Ventricular Mass Predicts Need for Bidirectional Glenn Shunt After Cone Repair for Patients with Ebstein Anomaly

Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants

ObjectiveTo describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). MethodsRetrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. ResultsFifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). ConclusionsMortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.

Cone repair after tricuspid valve replacement in Ebstein anomaly.

Cone repair after tricuspid valve replacement in Ebstein anomaly.

The concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.

Isolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation. Three adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result. The patient was discharged home after 7days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved. Over the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

Surgical Outcomes of Repairing Ebstein Anomaly: A Single-Center Experience

Background: Ebstein anomaly (EA) is a rare congenital disorder of the tricuspid valve (TV), accounting for about one percent of congenital heart defects. Considering highly variable anatomy, diverse corrective surgical methods are currently utilized. Cone reconstruction provides a near anatomic tricuspid restoration method that has triggered enthusiasm for early repair over recent years. Objectives: In the current study, we aimed to evaluate the clinical and echocardiographic features and outcomes of patients who underwent surgical correction of EA in our referral center. Methods: In this study, we reviewed the medical records of 35 patients with EA who underwent cone repair or TV replacement and had accessible echocardiographic data before and at least one year following the corrective surgery. The patients were evaluated for residual tricuspid regurgitation (TR) severity, right ventricular (RV) size, RV fractional area change (FAC), left ventricular end-systolic diameter (LVESD), left ventricular end-diastolic diameter (LVEDD), and 2D left ventricular ejection fraction (LVEF) before and after the operation. Results: Mean age of the patients was 29.49 ± 9.13 years, and 54.3% of them were female. Twenty-one (60%) patients underwent cone repair, and 14 were subjected to TV replacement. Seven (20%) patients required reoperation; redo TV repair was conducted in three patients (8.6%); two (5.7%) patients underwent biologic TV replacement; mechanical TV replacement was performed in one patient (2.9%) who had degenerative biologic TVs, and one patient (2.9%) was subjected to redo-operation due to mechanical TV malfunction. Positive changes in the means of RV-FAC and 2D-LVEF were statistically significant in all patients irrespective of the type of surgery (P < 0.001), but changes in the means of RV size (P = 0.38) and LVESD (P = 0.302) were not statistically significant. There was a significant increase in functional RV size in both groups of patients who underwent repair (P = 0.063) or replacement (P = 0.02). Conclusions: The present study revealed satisfactory post-op results for cone TV repair in patients with EA, evidenced by reduced TR severity and improved biventricular functional parameters. Assessing the durability of the outcomes of cone TV repair needs longer-term follow-ups.

Surgical Management of Ebstein’s Anomaly in Adults

Ebstein’s anomaly is a rare congenital anomaly of the tricuspid valve, which presents challenges to cardiac surgeons due to the spectrum of anatomical variation of the disease and the technical difficulty of valve repair. Given the evolving surgical techniques being implemented in repair, a review of regional results for surgery in Ebstein’s anomaly is important. This study aimed review surgical techniques being used in the management of Ebstein’s anomaly in adult patients and the outcomes of patients undergoing tricuspid valve repair, including the Cone repair, and tricuspid valve replacement for Ebstein’s anomaly in Australia and New Zealand. Outcome measures include postoperative survival and freedom from reoperation.

Comparative outcomes and risk analysis after cone repair or tricuspid valve replacement for Ebstein's anomaly

ObjectiveEbstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement. MethodsA total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan–Meier analyses were used to evaluate operative and long-term outcomes. ResultsResidual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P = .005) and tricuspid stenosis (21% vs 0%; P = .002) compared with the cone repair group. Kaplan–Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively (P = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group (P = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group. ConclusionsThe cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.

Contemporary Early Postoperative Cone Repair Outcomes for Patients With Ebstein Anomaly

ObjectiveTo describe the early postoperative outcomes after cone repair (CR) for Ebstein anomaly (EA) across the age spectrum. Patients and MethodsFor 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. ResultsMortality 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). ConclusionChildren 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.

Modified cone procedure along with bidirectional cavopulmonary shunt for Ebstein anomaly in adult patients

Ebstein anomaly is an uncommon congenital cardiac disease affecting the tricuspid valve and right ventricle. Da silva pioneered the cone reconstruction of tricuspid valve for surgical repair of this anomaly in 2007. We describe our experience of 5 adult patients who underwent cone repair along with a bidirectional cavopulmonary shunt and tricuspid annuloplasty ring placement at our institute. Five adult patients with Ebstein anomaly with progressive disease and symptoms were taken up cone reconstruction from 2019 to 2021. Along with cone reconstruction of tricuspid valve, we routinely added an end to side superior vena cava to right pulmonary artery shunt and a tricuspid annuloplasty ring in the newly formed tricuspid annulus in all cases. All patients tolerated the procedure well and easily weaned off cardiopulmonary bypass. There were no complications regarding bidirectional cavopulmonary shunt and only one patient had a temporary heart block which was managed conservatively. This series shows routine addition of a cavopulmonary shunt and an annuloplasty ring improves outcomes and can be performed at other experienced cardiac surgery centres.

Ebstein's Anomaly: From Fetus to Adult-Literature Review and Pathway for Patient Care.

Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.

New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique. This study demonstrates that our modifications of the cone repair procedure to correct Ebstein's anomaly improve valve coaptation and stabilization in the early and midterm. Of the 134 patients diagnosed with Ebstein's anomaly in our clinic between January 2012 and October 2020, 10 underwent a cone procedure and its modifications and were thus included in the study. The mean age of these patients was 28.6 ± 18.79 years and ranged from 1 to 61 years. Thirty percent of the patients were male and 70% were female. The additional procedures performed were an isolated cone procedure (two patients), anomalous pulmonary venous return repair in addition to a cone procedure (one patient), single-vessel coronary artery bypass (one patient), Glenn procedure (two patients), and tricuspid ring annuloplasty (four patients). Based on the results of this study, which assesses the outcomes of patients who underwent cone repairs with surgical modifications, we argue that right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty protect valve coaptation and function in the early and midterm, thereby having beneficial effects on right ventricle remodeling.

The Mid-Term Outcomes of Cone Repair or Replacement of Tricuspid Valve in Patients with Ebstein's Anomaly: Our Experience.

Cone repair of the tricuspid valve (TV) is a contemporary reproducible technique for surgical reconstruction of Ebstein's anomaly. Different authorities have shown that this technique restores excellent tricuspid valve function. In Bangladesh, this technique still is unfamiliar to many. We hereby present a case series of cone repair and TV replacement with the mid-term outcome (one year to six years) at the National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh. We prospectively studied 21 patients, who underwent surgical intervention (cone repair or tricuspid valve replacement) for Ebstein's anomaly of TV from March 2014 to June 2020. We divided the total patient population into the cone repair and TV replacement groups. Preoperative, postoperative, and follow-up data were collected from the hospital records, telephone conversations, and clinic visits. All collected data statistically were analyzed. Our patients showed there were statistically significant improvements after surgical intervention with regard to tricuspid regurgitation (TR) (P < 0.001), tricuspid annular plane systolic excursion (TAPSE) (P < 0.001), right ventricular (RV) function (P < 0.001), and New York Heart Association (NYHA) class (P < 0.001). These developments were sustained throughout the follow-up period. Cone repair should be offered to the symptomatic patients of Ebstein's anomaly because symptoms relief, reduction of morbidity, and survival benefits are excellent. Above all, the cone reconstruction shows fantastic results and may well become the surgical technique for patients with Ebstein's anomaly. We hope that new valve repair programs may provide extended longevity and restored quality of life to the patient of Ebstein's anomaly (EA) with the appropriate measures. In case of failed repair, valve replacement is an encouraging option.

Impact of Cone vs. Hetzer Repair on Postoperative Outcomes in Patients With Ebstein's Anomaly: 10-Year Experience From a Single Institution.

Objective: The aim of this study was to compare the early outcomes of the cone and Hetzer procedures for Ebstein's malformation.Methods: This retrospective study included patients who underwent either cone (n = 83) or Hetzer repair (n = 45) with Ebstein's malformation from January 2011 to December 2020.Results: One early death occurred in the cone group due to low cardiac output syndrome. Five cone and three Hetzer repair patients required reoperation before discharge. At discharge, the cone group had a better reduction in tricuspid valve regurgitation (TR) than the Hetzer group (74.7 vs. 51.1%, p = 0.009). Two patients in the cone group and seven patients in the Hetzer group required reoperation >30 days after their initial surgery. The cone group with no/mild TR was 75.6%, and the Hetzer group was 48.9% at the time of last follow-up (p = 0.010).Conclusion: Short-term outcomes of the cone repair are better than the Hetzer procedure. The cone repair should be the better option among patients with Ebstein's malformation who need surgical intervention.

The Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation

Cone repair (CR) uses native tissue for tricuspid valve (TV) repair and provides potential for growth. Results after CR were investigated in different age groups including several surgical modifications. Single institution retrospective analysis of all CR excluding neonatal procedures. Endpoints included TV reoperation, late tricuspid regurgitation (TR) and death. Between April 2006 and August 2019, 157 patients underwent CR at a median age of 11.7 years (range, 0.3-57.2). 20% (n=32) of patients had previous surgery. Repair modifications included atrial reduction (n=111,71%), right ventricular plication (n=85,55%), leaflet augmentation (n=36,23%), papillary muscle repositioning (n=50,32%), ring annuloplasty (n=70,45%). Early re-operation for recurrent TR occurred in 11 patients. Median follow-up time was 4.3 years (range, 9d-12.3y). There was no significant association between age at repair and time to TV reoperation (p=0.25). However, age <4 years at CR was identified as the most discriminating binary age threshold for the patients with TV reoperation (25.0% in <4y group vs 9.3% in the ≥4y group). Placement of an annuloplasty ring was protective against ≥moderate TR (OR=0.39, 95% CI 0.16-0.95, p=0.039). Freedom from late TV re-operation was 94.1% at 7 years. Survival was 97.9% at 6 years. Repair after age 18 years was associated with mortality in early follow-up (p=0.037). Mid-term results for CR are favorable in children and adults. Time to TV reoperation may be shorter when CR is performed before age four years, but this result requires confirmation in a larger sample. An annuloplasty ring should be considered when appropriate.

Contemporary Early Postoperative Cone Repair Outcomes For Patients With Ebstein Anomaly

Contemporary Early Postoperative Cone Repair Outcomes For Patients With Ebstein Anomaly