A Comparison of Echocardiogram Parameters in Ehlers-Danlos Syndrome Patients with and Without Mast Cell Activation Syndrome

Student Presenter(s): Anisa Raidah, Nolberto Jaramillo, Casey Sciandra, and Bernadette Riley
Faculty Mentor: Todd J. Cohen
School/College: Osteopathic Medicine, Old Westbury

Background: Mast Cell Activation Syndrome (MCAS) is a chronic multisystem disorder caused by inappropriate mast cell activation. It is commonly associated with EDS, a connective tissue disorder. Cardiovascular manifestations include arrhythmias, blood pressure dysregulation, pericarditis, heart failure, aneurysms, and vasculitis. Echocardiogram parameters in patients with MCD and EDS have not been previously studied. Methods: This study included all MCD patients with EDS and an echocardiogram seen at the Long Island Heart Rhythm Center. Echocardiographic parameters were compared between patients with and without MCD, using the Wilcoxon rank sum test. Data are reported as mean + standard deviation. Results: 35 EDS patients with transthoracic echocardiograms were divided into two groups: MCD (n=12, age 36.4 ± 13.2 years, 11 females, 1 male) and no MCD (n=23, age 37.5 ± 12.1 years, 23 females). There was no significant difference in mean body surface area, left-ventricular ejection fraction, interventricular septal thickness, left ventricular diameter during systole and diastole, aortic diameter, and left atrial diameter. Conclusion: Patients with MCAS have normal echocardiogram parameters that are not significantly different from those without. More studies are needed to characterize this difference.