Heart rate, conduction and ultrasound abnormalities in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type

  title={Heart rate, conduction and ultrasound abnormalities in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type},
  author={Filippo Camerota and Marco Castori and Claudia Celletti and Marco Colotto and Silvia Amato and Alessandra Colella and Mario Curione and Chiara Danese},
  journal={Clinical Rheumatology},
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… 

Differential diagnosis and diagnostic flow chart of joint hypermobility syndrome/ehlers‐danlos syndrome hypermobility type compared to other heritable connective tissue disorders

The differential diagnosis of JHS/EDS‐HT is revised with those heritable connective tissue disorders which show a significant overlap with the former and mostly include EDS classic, vascular and kyphoscoliotic types, osteogenesis imperfecta, Marfan syndrome, Loeys–Dietz syndrome, arterial tortuosity syndrome, and lateral meningocele syndrome.

Mitral valve prolapse and aortic root dilation in adults with hypermobile Ehlers–Danlos syndrome and related disorders

Routine echocardiograms to assess for valvular diseases and ARD may not be necessary unless warranted by presence of symptoms or family history, based on the results of this study and previous studies.

Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder

The known manifestations of the extra-articular systems of Ehlers–Danlos syndrome-HT are described, including cardiovascular, autonomic nervous system, gastrointestinal, hematologic, ocular, gynecologic, neurologic, and psychiatric manifestations.

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history

The hypermobile type of Ehlers–Danlos syndrome is described largely in those with musculoskeletal complaints including joint hypermobility, joint subluxations/dislocations, as well as skin and soft tissue manifestations.

Mitral Valve Prolapse In Patients With Benign Joint Hypermobility Syndrome (BJHS)

  • T. Mohammed
  • Medicine
    AL-Kindy College Medical Journal
  • 2019
The prevalence of mitral valve prolapse prolapse (MVP) in patients with benign hypermobility syndrome (BJHS) was significantly higher among patients with BJHS compared to normal mobile individuals.

Mitral Valve Prolapse and Its Motley Crew‐Syndromic Prevalence, Pathophysiology, and Progression of a Common Heart Condition

It is highlighted that in contrast to early studies describing MVP as a benign entity, the clinical course experienced by patients can be heterogeneous and may cause significant cardiovascular morbidity and mortality, so a review of clinical guidelines to allow for earlier surgical intervention may be warranted to lower cardiovascular risk in patients with MVP.

From the bedside to the bench and backwards: diagnostic approach and management of Ehlers-Danlos syndrome(s) in Italy

Ehlers-Danlos syndrome groups together an increasing number of hereditary connective tissue disorders mainly featuring abnormal skin texture, easy bruising, generalized joint hypermobility, and

Cardiovascular profile in postural orthostatic tachycardia syndrome and Ehlers–Danlos syndrome type III

It was found that the arterial stiffness and cardiac profiles of patients with POTS + EDSIII were comparable to those of age- and sex-matched controls, suggesting an alternate explanation for orthostatic intolerance.

The Ehlers–Danlos syndromes

The epidemiology, mechanisms, diagnosis and treatment of these syndromes are discussed, with common features including joint hypermobility, soft and hyperextensible skin, abnormal wound healing and easy bruising.



Echocardiographic findings in classical and hypermobile Ehlers–Danlos syndromes

The “pouching” shape of the SV was more common in hypermobile type than in the classical type of EDS, and 26/38 subjects demonstrated a prominent right coronary artery easily visualized by trans‐thoracic echocardiography, and 10/38 had an elongated cardiac silhouette on the 4‐chamber apical views.

Re‐writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type

The re‐writing of the natural history of JHS/EDS‐HT is aimed to raise awareness among clinical geneticists and specialists treating chronic pain conditions about pain and other complications of JHP, and symptoms' clustering by disease stage is proposed.

Postural tachycardia syndrome: a heterogeneous and multifactorial disorder.

The spectrum of cardiac defects in the Ehlers-Danlos syndrome, types I and III.

Ehlers-Danlos syndrome types I and III should be excluded in patients with mitral or tricuspid valve prolapse, great vessel dilatation, and congenital heart defects, because of the apparent high prevalence of cardiovascular abnormalities.

Postural tachycardia syndrome—current experience and concepts

The characteristics and neuroepidemiology of PoTS are described, and possible pathophysiological mechanisms of this syndrome are outlined, as well as current and investigational treatments.

Mitral valve prolapse and conduction defects in Ehlers-Danlos syndrome.

It is suggested that the basic underlying collagen defect in this syndrome is responsible for mitral valve prolapse and HV prolongation, and that the association may be more widespread in the syndrome than currently acknowledged.

Prevalence of aortic root dilation in the Ehlers-Danlos syndrome

ARD is a common finding in EDS, with no gender differences, and longitudinal studies are indicated to determine progression of ARD and its clinical significance.

Lack of consensus on tests and criteria for generalized joint hypermobility, Ehlers–Danlos syndrome: Hypermobile type and joint hypermobility syndrome

The level of consensus for the importance of various items for diagnosing GJH, EDS‐HT and JHS, was below the required limit (Cronbach's α >0.90) for clinical decision‐making and diagnosing.

Ehlers-Danlos syndromes and Marfan syndrome.