Matters of Heart: October 2019
Thanks to our September fundraisers!
The Loey-Dietz Angels 5K had great weather in New York on September 28th. Thanks to Kristyn Milan and her wonderful friends and family. Looks like a great time was had by all. Thanks for getting the word out about LDS!
Thanks so much to Shannon Keating, proprieter of Pots with Purpose in Elmhust, Illinois. The business is dedicated to beautifying spaces, and she serves her community by donating a portion of each month's proceeds to a specific charity. Her motto is "Every little bit helps!" (That's one of our motto's too!) Check them out on Facebook!
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Take a Picture in your Halloween costume and tag @LOEYSDIETZ #LDSFHALLOWEEN
Check out our arterial system factsheet to familiarize yourself with vascular anatomy here. And then check out some interesting MRA/CTA findings that radiologists use and/or look for on imaging for patients with Loeys-Dietz syndrome and other aortic conditions.
Vertebral tortuosity index (VTI) has been described as a tool in patients with, or suspected to have, connective tissue disease, and has been shown to be associated with increasing severity of aortic disease (ie. the higher the VTI, the more aggressive the aneurysm development/progression). It is not routinely measured on all MRAs or CTAs, but high VTI is very obvious on imaging in some LDS patients.
2011 American Heart Association--Shaine A. Morris. Circulation. Increased Vertebral Artery Tortuosity Index Is Associated With Adverse Outcomes in Children and Young Adults With Connective Tissue Disorders, Volume: 124, Issue: 4, Pages: 388-396, DOI: (10.1161/CIRCULATIONAHA.110.990549)
There is also literature to suggest more carotid artery tortuosity is associated with aneurysm progression For example, you can see major tortuosities of the internal carotid and vertebral arteries in this 3 year old with Loeys-Dietz syndrome.
2012 American Heart Association-- Dhouib. Circulation. Imaging Findings in a Child With Loeys-Dietz Syndrome, Volume: 126, Issue: 4, Pages: 507-508, DOI: 10.1161/CIRCULATIONAHA.112.103192)
Most recently, Mayo Clinic investigators have reported on a “chalice sign”, that when seen bilaterally is very specific for LDS. This refers to a widened angle between the internal and external carotid arteries as they branch off the common carotids (Benson, J.C. & Brinjikji, W. Clin Neuroradiol (2019). https://doi.org/10.1007/s00062-019-00838-5)
Check out our friends at Loeys-Dietz Syndrome Foundation Canada. They recently participated in the Heritable Aortic Diseases Symposium in Montreal and have posted some interviews with worldwide experts on the future of aortic conditions.