Aortic compliance in young patients with heterozygous familial hypercholesterolaemia.

1. Aortic compliance and plasma lipid and lipoprotein levels were measured in 20 young patients with heterozygous familial hypercholesterolaemia and in 20 age- and sex-matched control subjects. 2. Patients with familial hypercholesterolaemia had significantly higher plasma cholesterol, low-density lipoprotein-cholesterol and triacylglycerol levels than control subjects (P < 0.001, P < 0.001 and P < 0.005, respectively). The patients with familial hypercholesterolemia also had significantly more compliant (distensible) aortas than the control subjects (P < 0.001), a significant inverse correlation being observed between compliance and age (r = 0.73, P < 0.001) and between compliance and mean blood pressure (r = -0.60, P < 0.005). 3. When the effects of age and sex on aortic compliance were corrected for, the blood pressure effect disappeared, significant correlations being observed between normalized compliance and cholesterol (r = 0.50, P < 0.03), low-density lipoprotein-cholesterol (r = 0.54, P < 0.01), high-density lipoprotein-cholesterol (r = -0.44, P < 0.05), low-density lipoprotein-/high-density lipoprotein-cholesterol ratio (r = 0.60, P < 0.0006) and duration of disease (r = 0.67, P < 0.002). Multivariate regression analysis showed that the low-density lipoprotein-/high-density lipoprotein-cholesterol ratio (P < 0.03) and duration of disease (P < 0.04) were the best predictors of normalized compliance. 4. We suggest that the measurement of aortic compliance in young patients with familial hypercholesterolaemia may potentially be a useful, non-invasive, research tool for assessing their susceptibility to atheroma.

Non-invasive Doppler ultrasound technique for the in vivo assessment of aortic compliance.

A non-invasive Doppler ultrasound technique for the assessment of aortic compliance is described. This is based on the in vivo measurement of pulse wave velocity along the thoraco-abdominal aortic pathway. A structured protocol has been developed to improve the reproducibility of the technique as well as for the purposes of operator training. Preliminary results of a study of both the intra- and inter-observer variability of the method are provided and problems associated with the technique are discussed. Medical disorders such as atherosclerosis, diabetes mellitus and familial hypercholesterolaemia have all been shown to affect arterial wall compliance. We suggest that the measurement of aortic compliance in vivo may be a useful, non-invasive tool for assessing these patients' susceptibility to atheromatous arterial disease and for monitoring their response to therapy.