High cardiac output and arterial stiffness evaluated by research cardiovascular profiling system in a hypertensive patient with pseudoxanthoma elasticum

Abstract

Pseudoxanthoma elasticum (PXE) is an inherited systemic disorder characterized by generalized fragmentation of elastic fibers and progressive calcification of elastic tissue, affecting the skin, the eyes and the vascular system. Typically these patients present arteriosclerosis, hypertension, ischemic attacks and occlusive vascular changes at young ages. The aim of our study was to specify the cardiovascular changes in a case of pseudoxanthoma elasticum by a non-invasive haemodynamic evaluation. We present a case of a 50 years-old woman with a clinical diagnostic of pseudoxanthoma elasticum. She had no toxic habits and refered an active lifestyle but without regular exercise. Except for hypertension history, treated during the last four years, there was no other modificable cardiovascular risk factor. The patient had familiar history of early cardiovascular death. In the physical examination were present the typical skin lesions and the angioid streak of the retina. Age, sex and blood pressure are determinant of elastic properties, so we compared our patient with age, sex and BP matched controls. In both cases we determinated the following parameters by CR-2000 Research Cardiovascular Profiling System: Large and Small artery elasticity index (ml/mmHgx100), systemic vascular resistance (dyne.sec.cm5), total vascular impedance (dyne.sec.cm5), average pulse rate (beats / minute), Stroke volume (ml/beat), Cardiac output (ml/minute). Statistical analysis: Signed-Rank test (G-Stat 2.0) There is lower elasticity in large arteries (p = 0,006) but not in small arteries. There is no difference if we compare this value with an elder control group. There is a high cardiac output with respect control group (p = 0,006). There is higher total vascular impedance (p = 0,006) respect the control group but there is no difference compared with the elder group. There is a high cardiac output, total vascular impedance and large arterial stiffness in our patient with respect control group. We suggest that patients with PXE present vascular changes comparable with elder patients. These differences can not be explained by the hypertension.