Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Cardiology
Volume 2015, Article ID 167513, 3 pages
http://dx.doi.org/10.1155/2015/167513
Case Report

A Report of Accelerated Coronary Artery Disease Associated with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

1Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA
2Department of Medical Ethics and Health Policy, University of Pennsylvania, Philadelphia, PA 19104, USA
3Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA
4Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA

Received 10 June 2015; Revised 25 August 2015; Accepted 27 August 2015

Academic Editor: Kjell Nikus

Copyright © 2015 Courtney B. Rubin et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable form of vascular dementia and it is caused by mutations in the NOTCH3 gene. The neurologic manifestations of CADASIL syndrome have been well characterized; however, here we report one of the first de novo cases of CADASIL-associated coronary artery disease. A 45-year-old woman with a history of CADASIL and remote tobacco use presented with unstable angina. She was found to have diffuse and irregular narrowing of the left anterior descending artery and a drug eluting stent was deployed. Months later, she developed two subsequent episodes of unstable angina, requiring stent placement in the distal left anterior descending artery and the right coronary artery. Though the neurologic manifestations of CADASIL have been well described, these patients may also be predisposed to developing premature coronary artery disease. Patients with CADASIL and their physicians should be aware of this possible association because these patients may not be identified as high risk by traditional cardiovascular risk estimators. These patients may benefit from more aggressive interventions to reduce cardiac risk.