title: Noninvasive evaluation of coronary flow velocity reserve in homozygous familial hypercholesterolemia by transthoracic Doppler echocardiography.
abstract: OBJECTIVE
  To evaluate the value of transthoracic Doppler echocardiography (TTDE) of the left anterior descending coronary for the detection of early abnormalities of coronary arteries in asymptomatic patients with homozygous familial hypercholesterolemia (HoFH).
  
  
  METHODS
  Seventeen asymptomatic patients with HoFH and 10 controls had plasma total cholesterol, low-density lipoprotein (LDL) cholesterol, and triglycerides measured and underwent TTDE of their left coronary descending artery to determine peak and mean flow velocities under basal conditions and under hyperemia induced by adenosine infusion. Coronary flow velocity reserve (CFVR) was calculated from the mean flow velocities. The presence of aortic supravalvular stenosis was also determined from supravalvular flow velocity and aortic valve thickening values.
  
  
  RESULTS
  HoFH patients had similar basal but significantly lower hyperemic flow velocities and CFVR than control patients. Eight of the 17 HoFH patients had supravalvular aortic stenosis, and these patients had significantly higher LDL cholesterol and lower CFVR than those without this stenosis. Treatment with lipid-lowering drugs lowered lipid levels and increased CFVR values, but neither of these parameters reached normal values.
  
  
  CONCLUSIONS
  TTDE is a suitable noninvasive technique to detect early abnormalities of coronary arteries and to monitor the clinical efficacy of lipid-lowering treatment in asymptomatic HoFH patients.
authors.affiliation: Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
authors.forename: Xiaoshan
authors.initials: X
authors.lastname: Zhang
authors.forename: Ya
authors.initials: Y
authors.lastname: Yang
authors.forename: Zhi'an
authors.initials: Z
authors.lastname: Li
authors.forename: Hongyan
authors.initials: H
authors.lastname: Ren
authors.forename: Jie
authors.initials: J
authors.lastname: Lin
authors.forename: Lvya
authors.initials: L
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D001783:Blood Flow Velocity; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging* / Q000209:etiology*; D003326:Coronary Circulation; D004452:Echocardiography / Q000379:methods*; D018618:Echocardiography, Doppler, Color / Q000379:methods*; D005260:Female; D053805:Fractional Flow Reserve, Myocardial; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications* / Q000000981:diagnostic imaging*; D008297:Male; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity