Abstract
BACKGROUND: As surgical recommendations in adults based on size criteria of ascending aortic aneurysms become more refined, criteria for childhood/adolescence remains less clear. Multiple pathologic factors may predispose younger patients to thoracic aortic aortopathy and increase the risk of rupture. An evolving field of research is how to identify thoracic aortic dilation earlier in patients, risk stratify, and to obtain objective measures beyond size for proceeding with surgical intervention in order to prevent catastrophic thoracic aortic dissection. CASE DESCRIPTION: We report an adolescent case of dilated ascending aortic aneurysm with a functionally unicuspid/bicuspid aortic valve. This patient was taken to surgery electively, given the gradual increasing size of the ascending aorta. Intraoperatively, there was an unexpected intraoperative finding of a contained aortic rupture. The patient underwent an aortic root replacement with mechanical valve composite graft and coronary artery reimplantation (modified Bentall) with ascending hemiarch replacement. The patient did well with no post-operative complications. Aortic pathology and genetic analysis were performed. The patient was discovered to have a heterozygous variant in PTPN11 which is typically associated with Noonan syndrome; however, this is not known to be associated with aortopathy. CONCLUSIONS: As criteria for surgical intervention in adult thoracic ascending aortic aneurysms continues to evolve, this case illustrates challenges when determining the optimal criteria for surgical intervention in adolescent patients.