Left ventricular function in a large cohort of pseudoxanthoma elasticum patients.

ABSTRACT:

Objective

Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder characterized by the mineralization and fragmentation of elastic fibers in the skin, retina, and vascular walls. While there is no doubt that peripheral arterial disease is associated with PXE, several other cardiac complications have been linked with PXE, mainly based on case reports. It remains unclear, whether cardiac systolic or diastolic function impairment is a common complication of PXE.

Methods

This study conducted systematic assessment of left ventricular systolic and diastolic function via standard echocardiography and two-dimensional strain imaging, in a large cohort of asymptomatic PXE patients (n = 75) and matched healthy controls (n = 30).

Results

PXE and controls did not dif

SUBMITTER: Biere L 

PROVIDER: S-EPMC3961243 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature