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Background: Patients diagnosed with 22q11.2 deletion syndrome, also known as 22q11DS or DiGeorge syndrome, have a diverse array of congenital anomalies and chronic health conditions. Because no study has reported on the coordination of multidisciplinary health services in British Columbia required by individuals with 22q11DS, we set out to determine which health care services these patients access and what barriers in health informatics affect institutional and provincial data sharing. Methods: A retrospective review of patient care at BC Children's Hospital (BCCH) was conducted using the provincial discharge abstract database and databases from hospital specialty services involved in the care of patients with 22q11DS. Data were collected for patients seen at the hospital from April 2001 to March 2018. This 17-year review considered patient age at diagnosis, distribution of patient ages within the study cohort, number and type of specialty services accessed, and length of follow-up. Results: A total of 293 patients with 22q11DS were identified from the hospital discharge abstract databases (DAD) and seven BCCH specialty clinic databases. Data could not be retrieved from some clinic databases due to coding and reporting discrepancies, which prevented the amalgamation of data from all hospital specialty services. Data from the DAD and seven BC Children's Hospital specialty services were then amalgamated, and duplicates were removed to identify 293 unique patients with 22q11DS. On average, patients accessed five services, with 13 new patients being seen each year and followed for an average of 7 years. Most of the pediatric care was provided by the Cleft Palate/ Craniofacial Program, Cardiology, Endocrinology, and Otolaryngology. Using BC Women's Hospital Medical Genetics 22q11DS data (n = 400), we were able to corroborate the size of our study cohort and to estimate the prevalence of 22q11DS in BC for the study period. Conclusions: Barriers to data retrieval and sharing at institutional and provincial levels were found to limit care coordination for patients with 22q11DS, who require ongoing, complex, multidisciplinary management. Incongruent and incomplete data systems in BC are hindering our ability to provide this coordinated care and plan transition, leaving us without the evidence needed by provincial health care providers, local experts, policymakers, and national and international 22q11DS research centres. [ABSTRACT FROM AUTHOR]

Amongst patients with suspected obstructive coronary artery disease (CAD), less than a third of patients have obstructive disease on invasive coronary angiography (ICA) and fewer still have flow-limiting obstructive disease as determined by invasive fractional flow reserve (FFR). FFR is a powerful tool in guiding revascularization of flow-limiting lesions which in turn improves clinical outcome in those with haemodynamically significant obstructive disease. However FFR is infrequently performed due to the cost, time and patient discomfort the procedure entails. Further advances in non-invasive imaging has allowed FFR to be derived non-invasively by applying computational fluid dynamic (CFD) modeling to the coronary computed tomography angiography (CCTA) dataset without the need to induce hyperemia or modify the standard CCTA acquisition protocol. FFR derived from CCTA has been shown to have excellent correlation with invasive FFR and remains diagnostically robust in presence of reduced signal-to-noise ratio (SNR), coronary calcification and motion artifact. More recently, new data have emerged evaluating the clinical impact of fractional flow reserve computed tomography (FFRCT) on the assessment and management of patients with stable chest pain. One such study is the Prospective LongitudinAl trial of FFRCT: Outcome and Resource IMpacts (PLATFORM) study which showed an improved patient selection for ICA using CCTA-FFRCT approach by increasing the likelihood of identifying obstructive CAD at ICA amongst those intended for invasive testing. CCTA-FFRCT may therefore serve as efficacious gatekeeper to ICA that enriches the ICA population. The utility of FFRCT has also helped deepened our understanding of CAD. Through CFD modeling, it is now recognized that there are mechanistic forces of wall shear stress (WSS) and axial plaque force acting on coronary plaques. This has created further interest in exploring the possible interplay between these mechanistic forces on the development of coronary plaque and vulnerability of these plaques to rupture., Competing Interests: Conflicts of Interest: Jonathon Leipsic serves as a consultant for and has received research support from Heartflow. Shaw Hua Kueh is supported by the New Zealand Heart Foundation Fellowship grant. And Matthew Boroditsky has no conflicts of interest to declare.