@Article{chd.12824,
AUTHOR = {Kristina K. Rauser‐Foltz, Lois J. Starr, Anji T. Yetman},
TITLE = {Utilization of echocardiography in Ehlers‐Danlos syndrome},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {5},
PAGES = {864--867},
URL = {http://www.techscience.com/schd/v14n5/38860},
ISSN = {3071-1738},
ABSTRACT = {<b>Objectives:</b> This study assessed the frequency and utility of echocardiographic 
examination in patients with all forms of Ehlers‐Danlos syndrome and sought to iden‐
tify clinical variables associated with an abnormal echocardiogram.<br/>
<b>Design/setting:</b> This was a retrospective study of all patients carrying a diagnosis of 
Ehlers‐Danlos syndrome of any type who were evaluated by a pediatrician or pediatric 
subspecialist at a single tertiary medical center with an affiliated children’s hospital 
during the period January 2013 to December 2018.<br/>
<b>Patients:</b> Chart review was performed on all patients carrying a diagnosis of Ehlers‐
Danlos syndrome in the electronic medical record.<br/>
<b>Outcome Measures:</b> Data from genetics examination, cardiovascular examination 
where applicable, genetic test results when available, and echocardiography were 
recorded.<br/>
<b>Results:</b> Of 262 patients identified, echocardiography and cardiac evaluation were 
common occurring in 90% and 50% of patients with any form of Ehlers‐Danlos 
syndrome. Cardiovascular complications occurred in 50% of patients with vascular 
Ehlers‐Danlos syndrome but echocardiography was normal in all. Aortic dilation was 
common in classic Ehlers‐Danlos syndrome but absent in hypermobile Ehlers‐Danlos 
syndrome. Mitral valve prolapse and bicuspid aortic valve occurred at the same inci‐
dence as the general population. Cardiac symptoms were present in 12% but did not 
correlate with abnormal cardiac structure. Presentation with symptoms of musculo‐
skeletal pain was inversely related to the presence of cardiac pathology.<br/>
<b>Conclusions:</b> In light of the absence of cardiac pathology in patients with hypermo‐
bile Ehlers‐Danlos syndrome, routine cardiac evaluation and echocardiography are 
not required for patients with hypermobile Ehlers‐Danlos syndrome.},
DOI = {10.1111/chd.12824}
}