Background/Purpose:

Joint  Hypermobility  Syndrome (JHS) may predispose  to ligamentous rupture, joint dislocations, fibromyalgia,  premature osteoarthritis and non-articular complications include Mitral Valve Prolapse (MVP) (Barron J et al Clin Cardiology 1988;11:401).  

MVP is a common cardiac abnormality that affects 2 – 5% of the population.  It is  defined   as the systolic billowing of one or both of the mitral leaflets into the left atrium with or without evidence of mitral regurgitation.  The current standardized echocardiography (ECHO) diagnostic criteria for MVP is billowing of any portion of the mitral leaflets >2 mm above the annular plane in a parasternal long axis view.

There are limited published studies concerning JHS and MVP however they are thought to be associated.  The incidence of MVP in individuals with JHS has been reported to be 8%- 60% (Mishra B et al  Br J Rheumatol 1996;35:861)

This study would be the first to examine MVP in patients with JHS using updated diagnostic ECHO criteria.

Methods:

This is a retrospective study of 127 patients ages ≥ 16, diagnosed with JHS (ICD 9: 728.5), based on the revised Beighton criteria (Grahame R et al   J Rheumatol   2000;27:1777), seen by a rheumatologist (NS) between 1/1/2011-10/31/2014. The   diagnosis of JHS required  presence of   2 Major criteria: Beighton score of ≥4 and arthralgia ≥ three months in ≥ four joints, or 1 major and at least 2 minor criteria.  ECHOs were performed   and read using the new MVP criteria by ECHO experts (LC, DS). Past medical history that could increase risk to develop MVP including rheumatic fever, endocarditis, polycystic kidney disease, and family history of MVP was reviewed.

Results:

All patients included in the study had a Beighton score of ≥4 (Major Criterion). Most patients   were female n=114 (90%).  Mean age: 48 (range 16-87 years). Average Beighton score: 6.6 (range: 4-9). Average BMI: 27.5. 73 (57%)   were diagnosed with  fibromyalgia (ICD 9:729.1)

Echocardiograms were performed in 45 patients (35%) of which 43 (96%) were female with a mean age of 50.5 (range 22-86 years).  Average Beighton score: 6.6 (range: 4-9). Average BMI: 28. None of the patients had underlying rheumatic fever, endocarditis or adult polycystic  kidney disease.

None of the patients met the updated echocardiographic criteria for MVP.

Conclusion:

This is the first study examining   MVP in patients with JHS using the updated MVP ECHO criteria.   We did not find   ECHO findings consistent with MVP  in our JHS patients.  This should be studied in other   Rheumatology practices since   this  observation  may be cost saving in patients with JHS, who otherwise would have ECHO studies as part of their routine work up.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mitral-valve-prolapse-in-patients-with-joint-hypermobility-syndrome/