Krebushevski, Kaitlyn

Aortic dilation following single ventricle palliation is increasingly recognized, although its long-term clinical implications remain unclear. We present the case of a 38-year-old male born with a bicuspid aortic valve, d-transposition of the great arteries, ventricular septal defect, right ventricular hypoplasia with a straddling right atrioventricular valve. He was palliated with Fontan circulation and developed severe neo-aortic root and arch dilation with native aortic valve stenosis, requiring surgical intervention. His neo-aortic root measured 81 × 65 mm, necessitating a Bentall procedure with total arch and native aortic valve replacement. This case underscores the variable progression of aortic dilation in patients with palliated single ventricle physiology and the need for strict surveillance and individualized management. Larger cohort studies are needed to further refine the criteria for surgical intervention in this unique population.

ABSTRACT Background Preparation of the right ventricular outflow tract (RVOT) using covered stent placement is a common preparatory step for transcatheter pulmonary valve replacement (TPVR) to reduce the risk of disruption associated with dilating calcified RVOT conduits. Aim We present a novel method employing a self‐expanding covered aortic endoprosthesis and discuss its advantages as an alternative strategy. Methods Patients with congenital heart disease undergoing RVOT conduit preparation with the GORE TAG Thoracic Branch Endoprosthesis Extender (W.L. Gore & Associates, Newark, DE) followed by TPVR, were prospectively enrolled in a dedicated database. To date, 14 patients have been included. Data on demographics, diagnoses, procedural details, hemodynamic parameters, and outcomes were analyzed. Results Fourteen patients (median age: 35 years [range: 18–79]; 57% female) successfully underwent endograft placement immediately before TPVR. Treated conduits included pulmonary homografts ( n = 8), surgical bioprostheses ( n = 2), Hancock conduit ( n = 1), Contegra conduit ( n = 1), native RVOT ( n = 1), and Melody valve within a surgical bioprosthesis ( n = 1). Most patients presented with mixed stenosis and regurgitation ( n = 9), with the remaining having stenosis ( n = 4) or regurgitation ( n = 1). Valves implanted post‐preparation included the Sapien S3 valve (Edwards Lifesciences, Irvine, CA) ( n = 10) and the Melody valve (Medtronic, Minneapolis, MN) ( n = 5). Significant reductions in RVOT systolic gradient were observed (median 34 mmHg pre‐procedure to 3 mmHg post‐procedure), along with marked conduit expansion (median diameter 13 mm pre‐procedure to 25.5 mm post‐procedure). No complications were reported. Conclusions This study highlights the feasibility of a self‐expanding aortic endoprosthesis for RVOT preparation before TPVR. Further comparative study is warranted. Procedural success was achieved in all cases.