Cone repair after tricuspid valve replacement in Ebstein anomaly.

Central MessageThe Cone repair is viable in selected Ebstein anomaly patients after the tricuspid valve replacement , encouraging the preservation of leaflet tissues at the time of prosthesis implantation.See Commentary on page XXX. The Cone repair is viable in selected Ebstein anomaly patients after the tricuspid valve replacement , encouraging the preservation of leaflet tissues at the time of prosthesis implantation. See Commentary on page XXX. Since its creation in 1993, the cone repair has been applied to all anatomical variations of Ebstein anomaly (EA), achieving durable tricuspid valve (TV) competency.1Da Silva J.P. Baumgratz J.F. Da Fonseca L. Franchi S.M. Lopes L.M. Tavares G.M.P. et al.The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results.J Thorac Cardiovasc Surg. 2007; 133: 215-223Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar, 2Da Silva J.P. Da Fonseca Da Silva L. Ebstein’s anomaly of the tricuspid valve: the cone repair.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2012; 15: 38-45Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar, 3Burri M. Agua K.M. Cleuziou J. Beran E. Nagdyman N. Kuhn A. et al.Cone versus conventional repair for Ebstein’s anomaly.J Thorac Cardiovasc Surg. 2020; 160: 1545-1553Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar Although the cone repair has been described following certain TV repair procedures,4Dearani J.A. Said S.M. Burkhart H.M. Pike R.B. O’Leary P.W. Cetta F. Strategies for tricuspid valve re-repair in Ebstein’s malformation using the Cone technique.Ann Thorac Surg. 2013; 96: 202-208Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar,5Da Silva J.P. Viegas M. Castro-Medina M. Da Fonseca Da Silva L. Da Silva Cone operation after the Starnes procedure for Ebstein’s anomaly: new surgical strategy and initial results.J Thoracic Cardiovasc Surg Tech. 2020; 3: 281-283Google Scholar the literature has not addressed the cone repair after TV replacement in EA. We present 2 cases of TV prosthesis removal and native valve repair using the cone technique. Since 2010, 2 of 5 patients with EA with previous TV prosthesis implants done at other institutions were reoperated on using the cone repair. The patients provided signed consent for medical data publication, and the institutional review board at the University of Pittsburgh approved this study (#20080084) on June 1, 2022. The first patient was a 22-year-old man who presented with venous congestion and poor exercise tolerance 12 years after a TV bioprosthesis implant. The echocardiogram and the magnetic resonance imaging (MRI) of the chest demonstrated normal right ventricle (RV) function, a calcified TV prosthesis with severe stenosis, and regurgitation (Figure 1, A). We were also able to appreciate his native TV leaflets on his cardiac MRI. We removed the calcified prosthesis at the operation, finding preserved native TV leaflets downwardly displaced inside the RV. We conducted a TV repair using the cone technique, achieving a good valve performance (Figure 1, B). Currently, the patient is 34 years old, asymptomatic, and using metoprolol to treat systemic hypertension. His most recent echocardiogram, 12 years following repair, demonstrated normal RV function, mild RV and right atrium enlargement, and trivial TV regurgitation without stenosis (Figure 1, C). The second patient was a 47-year-old woman who had undergone 4 previous open-heart operations: TV repair as a neonate, atrial septal defect closure at 5 years of age, mechanical TV implantation with concomitant homograft pulmonary valve replacement at 24 years of age, and mechanical valve re-replacement and maze procedure at 34 years of age. She was referred for surgical reintervention due to recurrent tachyarrhythmias, fatigue, and lower-extremity edema. Her computed tomography images demonstrated the presence of viable TV tissues for the cone procedure (Figure 1, D). The transesophageal echocardiogram demonstrated TV prosthesis thrombosis with moderate regurgitation and severe stenosis (Figure 1, E). In addition, her homograft pulmonic valve had moderate regurgitation. We proceeded with mechanical valve extraction, TV cone repair, pulmonic valve replacement with a bioprosthesis, and redo bi-atrial maze ablation. The postoperative echocardiogram (Figure 1, F) showed no TV regurgitation or stenosis, with a 2.7-mm Hg mean diastolic TV gradient. She recovered well and was discharged home on postoperative day 6. At a 7-month echocardiographic follow-up, the TV had no stenosis and trivial regurgitation. We detail this operation in Figure 2 and Video 1. The cone procedure has proven suitable in multiple situations and variable spectrums of EA anatomical presentations.2Da Silva J.P. Da Fonseca Da Silva L. Ebstein’s anomaly of the tricuspid valve: the cone repair.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2012; 15: 38-45Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar We describe these 2 cases to highlight a previously untapped EA population that can benefit from the cone repair. Echocardiogram, MRI, and operative reports of previous procedures can indicate the presence of TV leaflets underneath the tricuspid prosthesis, raising the possibility of valve repair. Our decision to repair the TV instead of a prosthetic replacement was based on feasibility and to avoid multiple reinterventions, particularly in young patients. Our first patient excellent clinical condition and normal TV function at a 12 years’ follow-up support our approach. Even 2 previous mechanical prosthesis implantations in our second patient did not preclude the viability of the cone procedure, achieving a competent valve. Considering the option of future valve repair, the surgeons should keep the valvar tissues as much as possible while ensuring no obstruction to the RV inflow or outflow tracts during TV replacement in EA. Any valvar tissue that could cause obstruction or interfere with the prosthesis mechanism should be folded, relocated, or incised without resection. Implanting the prosthesis in a slightly supravalvar position may prevent heart block and facilitate the prosthesis extraction and a future valve repair. The sutures should be placed proximally to the anterior and inferior annulus, avoiding leaflets incorporation. At the septal area, the sutures are placed at the base of the coronary sinus and in the Todaro tendon, away from the atrioventricular node. The Thebesian valve may require an incision to keep the coronary sinus widely opened into the right atrium. We observed that the RV function and structure were preserved in both patients, suggesting that TV replacement may help preserve the RV function or promote reverse remodeling of a dilated and dysfunctional RV in preparation for the cone procedure. In conclusion, cone repair after the TV prosthesis implant is feasible, encouraging the preservation of most leaflet tissues during TV replacement in EA.