We evaluated 62 exercise treadmill tests (ETTs) in equal numbers of heterozygous for familial hypercholesterolemia (hFH) and healthy (HLY) women, matched for age, baseline systolic and diastolic blood pressure (BP) and baseline heart rate (HR), using the Bruce protocol. Both groups had similar rate pressure product (RPP) and workload in metabolic equivalents (METs) (27,563+/-3124 vs. 29,090+/-4077, p=0.103 and 11.2+/-1.7 vs. 11.5+/-1.8, p=0.473, respectively). Women with hFH had lower delta (difference of peak to baseline) and peak exercise systolic and diastolic BP (systolic: 48+/-12 vs. 58+/-17 mmHg, p=0.010 and 167+/-19 vs. 177+/-17 mmHg, p=0.042, respectively; diastolic: 11+/-7 vs. 15+/-7 mmHg, p=0.028 and 85+/-7 vs. 91+/-7 mmHg, p<0.001, respectively). Furthermore, women with hFH had higher delta percentage (%) of HR, compared to HLY; (106+/-25 vs. 95+/-20, p=0.047). In conclusion, hFH women possibly have an inadequate rise in systolic BP during ETT. Diastolic BP increased more in the HLY than in the hFH group, but still remained within normal limits. These findings may reflect preclinical changes of atherosclerosis in hFH women, however further research should be undertaken.