FMD and SCAD: Sex-Biased Arterial Diseases With Clinical and Genetic Pleiotropy

Circ Res. 2021 Jun 11;128(12):1958-1972. doi: 10.1161/CIRCRESAHA.121.318300. Epub 2021 Jun 10.

Abstract

Multifocal fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection are both sex-biased diseases disproportionately affecting women over men in a 9:1 ratio. Traditionally known in the context of renovascular hypertension, recent advances in knowledge about FMD have demonstrated that FMD is a systemic arteriopathy presenting as arterial stenosis, aneurysm, and dissection in virtually any arterial bed. FMD is also characterized by major cardiovascular presentations including hypertension, stroke, and myocardial infarction. Similar to FMD, spontaneous coronary artery dissection is associated with a high prevalence of extracoronary vascular abnormalities, including FMD, aneurysm, and extracoronary dissection, and recent studies have also found genetic associations between the two diseases. This review will summarize the relationship between FMD and spontaneous coronary artery dissection with a focus on common clinical associations, histopathologic mechanisms, genetic susceptibilities, and the biology of these diseases. The current status of disease models and critical future research directions will also be addressed.

Keywords: aneurysm; biology; fibromuscular dysplasia; genetics; prevalence.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aneurysm / etiology
  • Angiography
  • Aortic Dissection / etiology
  • Constriction, Pathologic / etiology
  • Coronary Vessel Anomalies* / diagnostic imaging
  • Coronary Vessel Anomalies* / epidemiology
  • Coronary Vessel Anomalies* / genetics
  • Coronary Vessel Anomalies* / pathology
  • Female
  • Fibromuscular Dysplasia* / diagnostic imaging
  • Fibromuscular Dysplasia* / epidemiology
  • Fibromuscular Dysplasia* / genetics
  • Fibromuscular Dysplasia* / pathology
  • Genetic Predisposition to Disease
  • Genetic Variation
  • Humans
  • Hypertension / etiology
  • Male
  • Myocardial Infarction / etiology
  • Sex Factors*
  • Stroke / etiology
  • Vascular Diseases / congenital*
  • Vascular Diseases / diagnostic imaging
  • Vascular Diseases / epidemiology
  • Vascular Diseases / genetics
  • Vascular Diseases / pathology

Supplementary concepts

  • Coronary Artery Dissection, Spontaneous