Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling

Neijenhuis, Ralph M. L.; Tsang, Victor; Marek, Jan; Issitt, Richard; Bonello, Béatrice; Klemperer, Kate von; Hughes, Marina

doi:10.1016/j.jtcvs.2020.10.124

Cited by 20 publications

(9 citation statements)

References 19 publications

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“…Currently, CR is the preferred technique of choice for TV repair in the overall EA population, and the reproducibility of the favourable outcomes has been echoed by many teams [ 12–14 ]. Notably, that paediatric patients with EA have a higher likelihood of late reoperation than adult patients warrants discussion [ 6 , 20 ].…”

Section: Discussionmentioning

confidence: 99%

“…Several plausible explanations are as follows: first, previous evidence [ 23 ] have indicated a concerning rise in SD risk triggered by the ventricular/atrial arrhythmias in the EA population. Delaying repair potentially exposes patients to the development of cardiomegaly and haemodynamic instabilities, which are the substrates giving rise to such arrhythmias; second, the echocardiographic [ 13 ] and cardiac magnetic resonance (CMR) [ 12 , 24 ] data have revealed sustainable improvement of biventricular function and remodelling after a successful EA surgery, which is crucial in maintaining sinus rhythm and atrioventricular synchrony. In addition, the risk of arrhythmias and SD increased over time [ 23 ], and data from the adult EA [ 25 ] indicated that earlier surgery could unburden the RV from the abnormal physiology and yield more favourable outcomes.…”

Section: Discussionmentioning

confidence: 99%

“…Cone reconstruction (CR) has now become an accepted method for EA. Several centres have adopted the technique and reported encouraging results [ 12–14 ]. We hypothesized that CR may be beneficial in a paediatric-specific setting.…”

Section: Introductionmentioning

confidence: 99%

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Review of surgical experience in 188 infants and young children with Ebstein anomaly

Liu

Wen

Shentu

et al. 2023

Interdisciplinary CardioVascular and Thoracic Surgery

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OBJECTIVES This study sought to review our single-institutionally surgical experience in paediatric Ebstein anomaly (pEA). METHODS We retrospectively reviewed the paediatric patients with Ebstein anomaly undergoing operation between 2004 and 2020. Time-to-event analysis was studied using Kaplan–Meier estimates. Cox regression model was used to identify risk factors for recurrent moderate-severe or greater tricuspid regurgitation (TR). RESULTS A total of 188 patients at a median age of 3.0 (interquartile range [IQR], 1.6–5.6) years were included, among whom 108 (57.4%) underwent cone reconstruction (CR). Bidirectional cavopulmonary shunt (BCPS) was required in 53 patients (28.2%). There were no in-hospital death. The median follow-up time was 5.6 (IQR, 2.9–8.9) years. Twenty-three (12.2%) developed recurrent moderate-severe or greater TR, among whom 9 required reoperation and 1 had late death. There was a lower incidence of recurrent TR (P = 0.006) and reoperation for TR (P = 0.037) in CR group compared with non-CR group. There was no difference in the incidence of recurrent TR (P = 0.61), reoperation (P = 0.9) and death (P = 0.48) among patients less than 1 year old, patients with 1–4 and 4–18 years of age. CONCLUSIONS Acceptable outcomes can be anticipated in pEA undergoing CR in terms of freedom from TR of > moderate degree at a mid-term follow-up.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Review of surgical experience in 188 infants and young children with Ebstein anomaly

Liu

Wen

Shentu

et al. 2023

Interdisciplinary CardioVascular and Thoracic Surgery

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“…668 In another 3% (at 10 years), problems other than tricuspid valve regurgitation were treated. 668 The cone procedure might decrease the reoperation rate after initial repair, 680 but it remains unknown whether the method is feasible as redo surgery.…”

Section: Surgical Revisionmentioning

confidence: 99%

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

Ohuchi

Kawata²,

Uemura

et al. 2022

Circ J

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9JCS 2022 Guideline on Management/Re-Intervention in ACHD comorbidity, 172 and cardiac catheterization is fundamental for its evaluation. 173 ▋ 3.4.4 Left Heart Catheterization and Coronary Angiography Echocardiography, CT, and MRI are often substituted for left ventriculography, aortography, or coronary angiography. CT precisely illustrates abnormal origins and courses of the coronary arteries in many cases, which is why coronary angiography is not recommended as a routine examination (Class III). In some patients, nonetheless, coronary angiography is quite informative beyond CT resolution; for example, anastomotic stenosis at the coronary arterial origin would be better demonstrated by catheter angiography after the arterial switch procedure. 174 ▋ 3.4.5 Endomyocardial Biopsy Endomyocardial biopsy is rarely required in patients with postoperative CHD. ▋ 3.4.6 Catheter InterventionTranscatheter closure of atrial septal defect or patent ductus arteriosus is carried out routinely in the cardiac catheterization room, as is percutaneous pulmonary angioplasty. Interventional closure of ventricular septal defect needs more evidence before becoming established in Japan.Transcatheter pulmonary valve replacement will be introduced in the near future.Electrophysiological study and catheter ablation are common practices for arrhythmias in the CHD field. Recent advances in technology and devices, such as 3D mapping systems, have contributed to notable results in anti-arrythmic treatments in patients with repaired CHD. 175

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“…Previously, Ibrahim and colleagues 5 demonstrated improved forward pulmonary blood flow per heartbeat (effective right ventricular stroke volume) and increased left ventricular diastolic filling indexed to body surface area after cone reconstruction, and this was associated with improved peak oxygen uptake on a bicycle ergometer in a subset of young adults with near-term follow up (2.7 years). In this more recent update from the same group at Great Ormond Street Hospital for Children, 6 they have expanded their data set and duration of follow-up, now evaluating global ventricular function late after cone reconstruction. The most significant findings are that both right ventricular and left ventricular effective stroke volume are increased in these patients at a median follow-up of 5.1 years, with the latter parameter more closely associated with exercise functional capacity.…”

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confidence: 99%

Commentary: Cone reconstruction for Ebstein's anomaly is here to stay

Nido

2021

The Journal of Thoracic and Cardiovascular Surgery

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complete anatomic RV that consists of both aRVand fRV preoperatively. 3 5. The LV end-diastolic volume increased postoperatively owing to the ventricular septal shift back toward the RV after surgery and better forward flow from the RV, resulting in improved LV preload. The LV end-systolic volume did not show a significant change.The major limitation of this study is the small number of patients that had follow-up CMR imaging. Although it is well recognized that CMR imaging can provide valuable data and is superior in quantifying ventricular volumes to echocardiography, the routine use of CMR scans in patients who are doing clinically well might not be justifiable given the cost, the potential need for general anesthesia in small children, and the fact that most surgeons rely on echocardiography to guide feasibility and strategies for tricuspid repair. This highlights the need for a prospective CMR imaging study for patients with EA undergoing cone repair, preferably as a multicenter study to provide a definitive assessment of late outcome regarding RV remodeling. Assessment of RV deformation by feature tracking on CMR imaging also may provide insight into the RV myopathy of EA. Such natural history data are imperative for assessing adjunctive therapeutic options for RV myopathy, for example, cell-based therapy. 4

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Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling

Cited by 20 publications

References 19 publications

Review of surgical experience in 188 infants and young children with Ebstein anomaly

Review of surgical experience in 188 infants and young children with Ebstein anomaly

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

Commentary: Cone reconstruction for Ebstein's anomaly is here to stay

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