A Comparison of Echocardiogram Parameters in Ehlers-Danlos Syndrome (EDS)

Patients With and Without Mast Cell Activation Syndrome (MCAS)

1 1 1 1 1
Anisa Raidah OMS III , Nolberto Jaramillo OMS III , Casey Sciandra OMS III , Bernadette Riley DO , Todd J. Cohen, MD
1
New York Institute of Technology College of Osteopathic Medicine, Department of Clinical Specialties, Old Westbury, NY

INTRODUCTION Value MCAS No MCAS Unit

RESULTS
Mean EF 60.025 60.32609 %
• Mast Cell Activation Syndrome (MCAS) • 35 EDS patients with transthoracic echocardiograms were SD EF 3.409645 4.610681 %
is a chronic multisystem disorder caused divided into two groups: MCAS vs. no MCAS EF P value 0.693812
by inappropriate mast cell activation. • MCD group: n=12, age 36.4 ± 13.2 years, 11 females, 1 male Mean IVSd 0.78875 0.8730435 cm
• It is commonly associated with EDS, a • No MCD group: n=23, age 37.5 ± 12.1 years, 23 females SD IVSd 0.1981978 0.1645223 cm
connective tissue disorder. [1] • There was no significant difference in mean body surface IVSd P value 0.2215969
• Cardiovascular manifestations include area, left-ventricular ejection fraction, interventricular septal Mean LVIDd 4.340833 4.483913 cm
arrhythmias, blood pressure thickness, left ventricular diameter during systole and SD LVIDd 0.5570125 0.4539097 cm
dysregulation, pericarditis, heart failure, diastole, aortic diameter, and left atrial diameter (Figure 1A-F LVIDd P value 0.4526917
aneurysms, and vasculitis. [1] & Table 1). Mean LVIDs 2.728333 2.908696 cm
• EDS patients are known to have an SD LVIDs 0.3647373 0.303679 cm
increased risk of aortic dilation and mitral LVIDs P value 0.1583498
valve prolapse on echocardiogram. [2] A B Mean AoD 2.71 2.939565 cm
• Echocardiogram parameters in patients SD AoD 0.2623668 0.441593 cm
with MCD and EDS have not been AoD P Value 0.06301829
previously studied. Mean LAD 2.833333 3.063043 cm
SD LAD 0.6139786 0.5195578 cm
LAD P value 0.2823271
METHODS
• Table 1. Mean values, P values, and standard deviations of
• This study included all MCD patients with cardiac parameters amongst the MCAS and No MCAS groups.

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.
• These parameters included ejection
fraction (EF), interventricular septal
diameter (IVSd), left ventricular internal
diameter in diastole (LVIDd), aortic
diameter (AoD), left atrial diameter (LAD)
• Data are reported as mean + standard
deviation.
C D
CONCLUSION
• Patients with MCAS and EDS have
normal echocardiogram parameters
that are not significantly different from
those without.
• More studies on a larger cohort are
needed to characterize these groups
E F as well as the differences in valvular
abnormalities.
REFERENCES
1. Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med.
2004;55:419-432. doi:10.1146/annurev.med.55.091902.103822
2. Pepin MG, Byers PH. Ehlers-Danlos Syndrome Type IV. In: Pagon RA,
Bird TD, Dolan CR, et al., eds. GeneReviews [Internet]. Seattle (WA):
University of Washington, Seattle; 1993-.
Figure 1. Bar Graphs demonstrating no significant differences in http://www-ncbi-nlm-nih-gov.arktos.nyit.edu/books/NBK1494/?report=pri
cardiac parameters amongst the groups. ntable (Accessed on May 18, 2013).