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Utilization of echocardiography in Ehlers-Danlos syndrome.
Congenit Heart Dis 2019; 14(5):864-867CH

Abstract

OBJECTIVES

This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers-Danlos syndrome and sought to identify clinical variables associated with an abnormal echocardiogram.

DESIGN/SETTING

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.

PATIENTS

Chart review was performed on all patients carrying a diagnosis of Ehlers-Danlos syndrome in the electronic medical record.

OUTCOME MEASURES

Data from genetics examination, cardiovascular examination where applicable, genetic test results when available, and echocardiography were recorded.

RESULTS

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 incidence as the general population. Cardiac symptoms were present in 12% but did not correlate with abnormal cardiac structure. Presentation with symptoms of musculoskeletal pain was inversely related to the presence of cardiac pathology.

CONCLUSIONS

In light of the absence of cardiac pathology in patients with hypermobile Ehlers-Danlos syndrome, routine cardiac evaluation and echocardiography are not required for patients with hypermobile Ehlers-Danlos syndrome.

Authors+Show Affiliations

Division of Cardiology, Department of Pediatrics, University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, Nebraska.Division of Clinical Genetics, Department of Pediatrics, University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, Nebraska.Division of Cardiology, Department of Pediatrics, University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, Nebraska.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31328377

Citation

Rauser-Foltz, Kristina K., et al. "Utilization of Echocardiography in Ehlers-Danlos Syndrome." Congenital Heart Disease, vol. 14, no. 5, 2019, pp. 864-867.
Rauser-Foltz KK, Starr LJ, Yetman AT. Utilization of echocardiography in Ehlers-Danlos syndrome. Congenit Heart Dis. 2019;14(5):864-867.
Rauser-Foltz, K. K., Starr, L. J., & Yetman, A. T. (2019). Utilization of echocardiography in Ehlers-Danlos syndrome. Congenital Heart Disease, 14(5), pp. 864-867. doi:10.1111/chd.12824.
Rauser-Foltz KK, Starr LJ, Yetman AT. Utilization of Echocardiography in Ehlers-Danlos Syndrome. Congenit Heart Dis. 2019;14(5):864-867. PubMed PMID: 31328377.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Utilization of echocardiography in Ehlers-Danlos syndrome. AU - Rauser-Foltz,Kristina K, AU - Starr,Lois J, AU - Yetman,Anji T, Y1 - 2019/07/22/ PY - 2019/06/10/received PY - 2019/06/20/revised PY - 2019/07/08/accepted PY - 2019/7/23/pubmed PY - 2019/7/23/medline PY - 2019/7/23/entrez KW - Ehlers-Danlos syndrome KW - aneurysm KW - aorta KW - echocardiography SP - 864 EP - 867 JF - Congenital heart disease JO - Congenit Heart Dis VL - 14 IS - 5 N2 - OBJECTIVES: This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers-Danlos syndrome and sought to identify clinical variables associated with an abnormal echocardiogram. DESIGN/SETTING: 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. PATIENTS: Chart review was performed on all patients carrying a diagnosis of Ehlers-Danlos syndrome in the electronic medical record. OUTCOME MEASURES: Data from genetics examination, cardiovascular examination where applicable, genetic test results when available, and echocardiography were recorded. RESULTS: 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 incidence as the general population. Cardiac symptoms were present in 12% but did not correlate with abnormal cardiac structure. Presentation with symptoms of musculoskeletal pain was inversely related to the presence of cardiac pathology. CONCLUSIONS: In light of the absence of cardiac pathology in patients with hypermobile Ehlers-Danlos syndrome, routine cardiac evaluation and echocardiography are not required for patients with hypermobile Ehlers-Danlos syndrome. SN - 1747-0803 UR - https://www.unboundmedicine.com/medline/citation/31328377/Utilization_of_echocardiography_in_Ehlers_Danlos_syndrome_ L2 - https://doi.org/10.1111/chd.12824 DB - PRIME DP - Unbound Medicine ER -