Aortic Root Replacement in Adult Patients with Repaired Congenital Conotruncal Defects - Procedures, Hospital...

To characterize hospital survival and risk factors after aortic root replacement performed by congenital units in adult patients, and to compare risk profiles of repaired conotruncal defects to other underlying diagnoses.

Adult patients after aortic root replacement in a congenital cardiac unit were identified in the European Congenital Heart Surgeons Association (ECHSA) database. Underlying pathology, prior surgeries, and types of index aortic root surgery were characterized. Primary endpoints were hospital and 30-day survival. Secondary endpoints included circulatory arrest, length of intensive care unit and hospital stays, and discharge to chronic care facility. Multiple logistic regression analyses were performed to test for independent risk factors.

N=2544 patients after full aortic root replacement, performed in 101 different hospitals (1999 to 2024), were identified. N=249 patients (10%) with primary conotruncal defects included double-outlet-right ventricle (n=14, 6%), pulmonary atresia with VSD (n=42, 17%), transposition of great arteries (n=71, 29%), Tetralogy of Fallot (n=88, 35%), Truncus arteriosus communis (n=34, 14%). Mechanical Bentall was performed in n=163 cases (65%), valve-sparing in n=43 cases (17%), biological Bentall in n=20 (8%), homograft replacement in n=23 (9%). Conotruncal diagnosis was linked to longer CPB times (p<0.001), longer aortic cross-clamp times (p<0.001), longer ICU stay (p<0.001) and longer hospital stay (p<0.001). Multiple logistic regression revealed duration of CPB (p<0.001) and conotruncal lesion (OR 2.1, p=0.037) as independent risk factors for hospital mortality.

Among adult congenital heart defect patients undergoing aortic root replacement, those with conotruncal defects represent a distinct subgroup characterized by a high-risk operative profile.