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Abstract Details

Shunting Prevalence in Patients with Moyamoya
Child Neurology and Developmental Neurology
P9 - Poster Session 9 (5:30 PM-6:30 PM)
Moyamoya is a rare progressive stenosis of anterior cerebral circulation of unknown etiology that occurs at higher prevalence in patients of East Asian descent, and those with neurofibromatosis 1, trisomy 21, or sickle cell disease (SCD).  

Our objective was to compare the prevalence of shunting in patients with Moyamoya (MM) versus controls without MM. We hypothesized that vasoactive substances, normally inactivated in the lungs, may escape inactivation through right-to-left shunting and contribute to the development of MM in at-risk groups. 

In an IRB-approved, retrospective case-control study we identified 67 MM patients who had echocardiograms between 1991-2018 and compared the prevalence of potential right-to-left shunting with that of a control group of 123 patients without MM or stroke from a prior study. 
In the MM group, 32 (48%) had potential shunting identified on echocardiogram, 23 (34%) of which had intracardiac shunting, and 9 (13%) of which had intrapulmonary shunting. This was compared to our control group, with 29 (24%) patients with potential shunting (P=0.0006). We also evaluated our MM patients considering possible bias regarding SCD patients and known increased prevalence of pulmonary shunting. In the 43 MM patients without SCD, 21 (49%) had potential shunting identified on echocardiogram versus 29 (24%) with potential shunting in our control group (P=0.0018).
In conclusion, these observations support our hypothesis that shunting could contribute to MM development, possibly through unknown vasoactive substances escaping inactivation in the pulmonary system, subsequently inducing vascular changes in patients with predisposing conditions.  This has potential mechanistic and therapeutic implications and will need to be verified in larger prospective studies.
Lori Xu, MD (Children's Medical Center - Specialty Center)
Dr. Xu has nothing to disclose.
Wilmot Bonnet, MD (University of Texas SW Medical School, Child Neurology) Dr. Bonnet has nothing to disclose.
Michael M. Dowling, MD, PhD, FAAN Dr. Dowling has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson and Johnson. Dr. Dowling has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NHLBI. The institution of Dr. Dowling has received research support from NIH.