Cone Repair In A Child With Severe Ebstein’s Anomaly And A Near-absent Posterior Leaflet Using Autologous And Living Right Atrial Wall Tissue For Patch Augmentation Andrea Orellana, BA1, Ishit Chauhan, MBBS1, Jiovanna Fernandez, BS1, Danyal M. Khan, MD2, Jason Inoa, PA-C1, Enrique Garcia, MD1, David M. Kalfa, MD, PhD1. 1Department of Cardiovascular Surgery, Heart Institute, Nicklaus Children's Hospital; Herbert Wertheim College of Medicine, Florida International University, Miami, FL, USA, 2Department of Cardiology, Heart Institute, Nicklaus Children's Hospital, Miami, FL, USA.
Introduction Cone repair offers excellent reconstruction and outcomes for Ebstein’s disease. However, if native valve leaflets remain underdeveloped despite extensive delamination, leaflet augmentation is needed and can increase repair durability in a growing child. This report describes a cone repair with autologous right atrial wall patch augmentation in a 10-year-old male with severe Ebstein’s anomaly. Description Preoperative imaging confirmed Ebstein’s anomaly with severe tricuspid regurgitation (TR), apical displacement of the septal leaflet (27 mm), sail-like anterior leaflet (40 mm), dilated tricuspid valve annulus (46 mm), severely dilated right atrium (RA) and right ventricle (RV), and normal biventricular systolic function. Progressive dyspnea and episodic supraventricular tachycardia prompted surgical intervention. A cone repair with autologous right atrial wall patch augmentation, RV plication, RA reduction plasty, and atrial septal defect (ASD) closure was performed. Intraoperatively, multiple anterior leaflet fenestrations, severely displaced septal leaflet, and near-absent posterior leaflet were identified. A thinned autologous RA wall patch augmented the septal and posterior leaflet height and cone circumference, while an MC3 26 mm ring stabilized the structure. Postoperative imaging revealed mild TR, no tricuspid stenosis, no residual ASD, and no significant RA dilation. The patient was extubated in the operating room and discharged on postoperative day 5. Echocardiography findings remain unchanged at last follow-up (6 months). Conclusions Autologous RA wall patch augmentation serves as an effective approach for patch-requiring cone repairs and may improve durability of the repairs, given its living, endothelialized, and autologous nature. Long-term follow-up of the autologous leaflet substitute is warranted.
