Floppy mitral valve/mitral valve prolapse syndrome: Beta-adrenergic receptor polymorphism may contribute to the pathogenesis of symptoms

J Cardiol. 2015 May;65(5):434-8. doi: 10.1016/j.jjcc.2014.07.020. Epub 2014 Sep 22.

Abstract

Background: Certain patients with floppy mitral valve (FMV)/mitral valve prolapse (MVP) may have symptoms that cannot be explained on the severity of mitral valvular regurgitation (MVR) alone; hypersensitivity to adrenergic stimulation has been suggested in this group defined as the FMV/MVP syndrome.

Methods: Ninety-eight patients (75 men, 23 women) with mitral valve surgery for FMV/MVP were studied. Of those 41 (42%) had symptoms consistent with FMV/MVP syndrome [29 men (39%), 12 women (52%)]; median age of symptom onset was 30 years (range 10-63 years) and median duration of symptoms prior to valve surgery was 16 years (range 3-50 years). Ninety-nine individuals (70 men, 29 women) without clinical evidence of any disease were used as controls. Genotyping of β1 and β2 adrenergic receptors was performed.

Results: β-Adrenergic receptor genotypes (β1 and β2) were similar between control and overall FMV/MVP patients. Subgroup analysis of patients, however, demonstrated that the genotype C/C at position 1165 resulting in 389 Arg/Arg of the β1 receptor was more frequent in women compared to those without FMV/MVP syndrome and to normal control women (p<0.025). This polymorphism may be related to hypersensitivity to adrenergic stimulation as reported previously in these patients.

Conclusion: This study shows a large proportion of patients with FMV/MVP, predominantly women, had symptoms consistent with the FMV/MVP syndrome for many years prior to the development of significant MVR, and thus symptoms cannot be attributed to the severity of MVR alone. Further, women with FMV/MVP syndrome, symptoms at least partially may be related to β1-adrenergic receptor polymorphism, which has been shown previously to be associated with a hyperresponse to adrenergic stimulation.

Keywords: Beta-adrenergic receptor polymorphism; Floppy mitral valve; Mitral valve prolapse syndrome; Symptoms.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Case-Control Studies
  • Child
  • Female
  • Genotype
  • Greece
  • Humans
  • Male
  • Middle Aged
  • Mitral Valve Prolapse / ethnology
  • Mitral Valve Prolapse / genetics*
  • Mitral Valve Prolapse / pathology
  • Phenotype*
  • Polymorphism, Genetic / genetics*
  • Receptors, Adrenergic, beta / genetics*
  • Severity of Illness Index
  • Sex Factors
  • Young Adult

Substances

  • Receptors, Adrenergic, beta