Neoaortic root and arch aneurysm reconstruction after Norwood procedure in adolescents and adults with Fontan physiology: A case series.

OBJECTIVES: With increased survival of Fontan patients, the incidence of aortic aneurysm post-Norwood procedure is increasingly recognized. Herein, we present our bi-institutional case series of complex aortic repair for post-Norwood aneurysm with medium-term follow-up. METHODS: Five pediatric and adult Fontan patients who underwent neoaortic root and arch aneurysm reconstruction were included in this study. Informed consent was obtained for publication. Results are presented with descriptive statistics. RESULTS: Complex aortic repair was performed at median age of 21.4 years (interquartile range [IQR], 15.2-25.5 years) for median ascending and/or arch aortic aneurysm diameters of 66 mm (IQR, 63-67 mm), of which 4 patients presented asymptomatically. Median cardiopulmonary bypass and aortic cross-clamp times were 257 minutes (IQR, 218-284 minutes) and 149 minutes (IQR, 120-186 minutes), respectively. Mechanical Bentall was performed in 4 of the 5 patients. Our institutional preference is end-to-side anastomosis for reconstruction of the ascending and proximal arch transition when the angulation is acute. Median duration of intensive care unit stay and length of hospital stay was 5 days (IQR, 5-7 days) and 8 days (IQR, 7-21 days), respectively. At median follow-up of 2.5 years, 1 pediatric patient required reintervention for multilevel outflow tract obstruction at 6 years' follow-up. All patients are alive and functionally well. CONCLUSIONS: Despite the heightened risk of extensive aortic repair in patients with Fontan physiology who have a history of multiple redo operations and risk of coagulopathy from liver dysfunction, adequate clinical outcomes can be achieved in expert centers.