# Unique Intravascular Ultrasound and Optical Coherence Tomography Features of Multivessel Coronary Stenosis in Pseudoxanthoma Elasticum

## TL;DR

This paper describes unique coronary artery blockages in a rare genetic disease using special imaging techniques.

## Contribution

First report using both IVUS and OCT to characterize coronary stenosis in pseudoxanthoma elasticum.

## Key findings

- IVUS shows concentric high-intensity lesions with mixed acoustic shadowing in PXE-related stenosis.
- OCT reveals thin, sheet-like calcifications with low-intensity heterogeneous tissue in PXE patients.
- PXE stenosis results from calcification-induced fragmentation and tissue repair, distinct from atherosclerosis.

## Abstract

Pseudoxanthoma elasticum (PXE) is a rare disease resulting from ABCC6 gene mutations; it causes elastic fiber calcification with unclear arterial stenosis mechanisms.

A 60-year-old man with PXE developed multivessel coronary stenosis despite low atherosclerotic risk. Coronary computed tomography demonstrated noncalcified stenosis in all vessels, intravascular ultrasound (IVUS) showed concentric high-intensity lesions with mixed acoustic shadowing, and optical coherence tomography (OCT) indicated thin, sheet-like calcifications with low-intensity heterogeneous tissue. Guidance through IVUS/OCT imaging contributed to successful percutaneous coronary intervention.

PXE demonstrates stenotic mechanisms distinct from conventional atherosclerosis. Stenosis develops as a result of tissue thickening caused by calcification-induced fragmentation and repair. To our knowledge, no previous reports have evaluated coronary stenosis in PXE using both IVUS and OCT.

Patients with PXE may develop significant coronary stenosis despite low atherosclerotic risk, requiring early cardiovascular evaluation. Combined IVUS/OCT imaging reveal unique stenotic findings resulting from elastic fiber calcification.