P2A166: The Uncertain Journey for Parents of Children with Anomalous Aortic Origin of a Coronary Artery

Background: Anomalous aortic origin of a coronary artery (AAOCA), is the second leading cause of sudden cardiac death in young athletes. Individuals with AAOCA can be diagnosed after a sudden cardiac event, secondary to symptoms, or as an incidental finding. The diagnosis can be acutely life altering and, as a relatively rare entity, with limited long-term outcome data, management approaches can vary from center to center. This multi-center qualitative investigation sought to gain a deeper understanding of the lived experiences and challenges of parents of children diagnosed with AAOCA.

Methods: This study brought together parents of children with AAOCA cared for at four institutions to participate in lightly facilitated group conversations that places them as experts of their lived experience. Session transcripts were inductively coded, with no a priori codebook, by multiple coders and frequently expressed themes were identified. Identified themes and supporting quotes were reviewed and agreed upon by the study team.

Results: A total of 42 individuals from 41 families participated in a session. Thirty-eight (90%) were female and median age of their children was 15[IQR:12-18] years. Of those known, 29% (10/34) had an isolated left coronary anomaly and 43% (18/42) had undergone surgery. Twenty-nine themes supported by quotes (Table) were identified which were categorized into four main groups, emotional and mental health impact (n=13), treatment and care (n=9), access to information(n=4), and transition to adulthood (n=3) (Figure). In the emotion category, parents spoke of the fear and uncertainty around the diagnosis and future, the need for support from friends, family and formal mental health clinicians, and coping with the diagnosis. In transition, parents identified the need for age-appropriate education for children, challenges with the transition to self-management, and the logistics of transferring from a pediatric to adult provider. In access to information they spoke about obtaining information from clinicians and on their own as well as the overall lack of data available. Lastly, in treatment and care, parents identified challenges around treatment decision-making, the convoluted and uncertain diagnostic and care journey, and the impact of both clinician and parental placed activity restrictions. Overall, the rarity, sudden and unexpected diagnosis, a lack of significant data to support decision-making, and relatively unknown long-term outcomes permeated through most of the themes identified.

Conclusion: AAOCA can result in devastating consequences. This in conjunction with a frequently convoluted diagnostic journey, inconsistent management strategies, and an overall lack of outcomes (clinical and quality of life) data creates a complex and anxiety-filled experience for parents of children with AAOCA. Additional work must be focused on the needs of parents and patients, measurement of meaningful long-term outcomes, and transforming AAOCA care delivery to improve those outcomes.