A Multi-Dimensional Outcomes Study in an Inception Cohort of Patients with Interrupted Aortic Arch

The fundamental goal of pediatric cardiac surgery is to enable patients with congenital heart disease to achieve a long and healthy life; hopefully, one comparable to that of their peers. While there is an understanding that long-term assessment is required over the course of a patient’s lifetime, the manner in which the success of surgical intervention is measured in these patients should be multi-dimensional with outcomes measured in numerous domains. This thesis explores the outcomes of patients within a multi-institutional prospective inception cohort of patients with interrupted aortic arch in relation to the durability of surgical intervention, functional health status, and transition to adult care from pediatric care. We first demonstrate that there is a persistent risk of subsequent procedures and mortality in patients after interrupted aortic arch repair. We also demonstrated that interrupted aortic arch is a chronic disease of a complex nature whereby patients often require multiple procedures after their index repair, that the risk of aortic arch and left ventricular outflow tract procedures varies by phase (time-related), and the factors associated with both of these in addition to mortality. Our study on functional health status demonstrates that both adolescents and young adults with interrupted aortic arch primarily rated themselves the same or better than their normal peers. Our study on transition to adult care demonstrates that transition occurred in only 32% of patients by age 21, and was associated with Canadian residence. Questionnaire results also demonstrated poor levels of patient knowledge regarding their disease and strong parental involvement. The studies presented represent the diversity of long-term outcomes that should be analyzed in order to improve patient care using a multi-dimensional approach. Future studies will focus on definitive repair techniques, ameliorating factors associated with poor functional health status, and improving transition to adult care.Ph