Evidence of microvascular dysfunction in patients with cystic fibrosis

Am J Physiol Heart Circ Physiol. 2016 Jun 1;310(11):H1479-85. doi: 10.1152/ajpheart.00136.2016. Epub 2016 Apr 15.

Abstract

Cystic fibrosis (CF) is a genetic, multisystemic disorder with broad clinical manifestations apart from the well-characterized pulmonary dysfunction. Recent findings have described impairment in conduit vessel function in patients with CF; however, whether microvascular function is affected in this population has yet to be elucidated. Using laser-Doppler imaging, we evaluated microvascular function through postocclusive reactive hyperemia (PORH), local thermal hyperemia (LTH), and iontophoresis with acetylcholine (ACh). PORH [518 ± 174% (CF) and 801 ± 125% (control), P = 0.039], LTH [1,338 ± 436% (CF) and 1,574 ± 620% (control), P = 0.045], and iontophoresis with ACh [416 ± 140% (CF) and 617 ± 143% (control), P = 0.032] were significantly lower in patients with CF than control subjects. In addition, the ratio of PORH to LTH was significantly (P = 0.043) lower in patients with CF (55.3 ± 5.1%) than control subjects (68.8 ± 3.1%). Significant positive correlations between LTH and forced expiratory volume in 1 s (%predicted) (r = 0.441, P = 0.013) and between the PORH-to-LTH ratio and exercise capacity (r = 0.350, P = 0.049) were observed. These data provide evidence of microvascular dysfunction in patients with CF compared with control subjects. In addition, our data demonstrate a complex relationship between microvascular function and classical markers of disease severity (i.e., pulmonary function and exercise capacity) in CF.

Keywords: cystic fibrosis; endothelial function; endothelium-dependent vasodilation; microvascular function.

Publication types

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

MeSH terms

  • Acetylcholine / administration & dosage
  • Administration, Cutaneous
  • Adolescent
  • Adult
  • Blood Flow Velocity
  • Case-Control Studies
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / physiopathology*
  • Exercise Tolerance
  • Female
  • Forced Expiratory Volume
  • Forearm / blood supply*
  • Humans
  • Hyperemia / physiopathology
  • Iontophoresis
  • Laser-Doppler Flowmetry
  • Lung / physiopathology
  • Male
  • Microcirculation* / drug effects
  • Microvessels / drug effects
  • Microvessels / physiopathology*
  • Regional Blood Flow
  • Severity of Illness Index
  • Vasodilation* / drug effects
  • Vasodilator Agents / administration & dosage
  • Vital Capacity
  • Young Adult

Substances

  • Vasodilator Agents
  • Acetylcholine