Heart rate, conduction and ultrasound abnormalities in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type
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Joint hypermobility syndrome (JHS) and Ehlers-Danlos syndrome, hypermobility type (EDS-HT) are two clinically overlapping heritable connective tissue disorders strongly associating with pain, fatigue and other secondary aspects. Though not considered a diagnostic criterion for most EDS subtypes, cardiovascular involvement is a well-known complication in EDS. A case-control study was carried out on 28 adults with JHS/EDS-HT diagnosed according to current criteria, compared to 29 healthy subjects evaluating resting electrocardiographic (ECG), 24-h ECG and resting heart ultrasound data. Results obtained in the ECG studies showed a moderate excess in duration of the PR interval and P wave, an excess of heart conduction and rate abnormalities and an increased rate of mitral and tricuspid valve insufficiency often complicating with “true” mitral valve prolapse in the ecocardiographic study. These variable ECG subclinical anomalies reported in our sample may represent the resting surrogate of such a subnormal cardiovascular response to postural changes that are known to be present in patients with JHS/EDS-HT. Our findings indicate the usefulness of a full cardiologic evaluation of adults with JHS/EDS-HT for the correct management.
KeywordsEhlers-Danlos syndrome Electrocardiographic abnormalities Hypermobility
- 5.Mayer K, Kennerknecht I, Steinmann B (2013) Clinical utility gene card for: Ehlers–Danlos syndrome types I–VII and variants—update 2012. Eur J Hum Genet 21:e1–e4Google Scholar
- 7.Remvig L, Engelbert RH, Berglund B, Bulbena A, Byers P, Grahame R, Kristensen BJ, Lindgren KA, Uitto J, Wekre L (2011) Need a consensus on the methods by which to measure joint mobility and the definition of norms for hypermobility that reflect age, gender, and ethnic-dependent variation: is revision of criteria for joint hypermobility syndrome and Ehlers-Danlos syndrome hypermobility type indicated? Rheumatology 50:1169–1171PubMedCrossRefGoogle Scholar
- 8.Remvig L, Flycht L, Christensen KB, Juul-Kristensen B. (2014) Lack of consensus on tests and criteria for generalized joint hypermobility, Ehlers-Danlos syndrome: hypermobile type and joint hypermobility syndrome. Am J Med Genet A. Jan 24Google Scholar
- 19.Castori M, Morlino S, Celletti C, Ghibellini G, Bruschini M, Grammatico P, Blundo C, Camerota F (2013) Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. Am J Med Genet A 161A:2989–3004PubMedCrossRefGoogle Scholar