Vascular Ehlers Danlos Syndrome (VEDS) is caused by pathogenic variants of the COL3A1
gene, resulting abnormal Type III collagen protein. This impacts the body's connective
tissue and makes people with VEDS at high risk of spontaneous aortic and arterial
rupture, pneumothorax, and hollow organ perforation across the age spectrum. Given this
risk and high potential for lethality, VEDS is considered the most severe type of
Ehlers-Danlos Syndrome. In addition, many patients experience chronic pain and fatigue,
sleep disturbances, and mental health challenges. As is the case for many patients with
chronic illness, stress, anxiety, and depression are often present over the course of the
disease. Despite the antecedent, stress and anxiety trigger a sympathetic nervous system
(SNS) response in the body, which, over a period of time, can have detrimental effects
both physiologically and psychologically for patients. For patients with already
compromised cardiovascular systems, the dysregulation of their nervous system can
potentially be detrimental to their mental and physiological health, as well as their
overall quality of life.
Recent studies have begun to use biofeedback techniques to teach patients
non-pharmacological strategies for managing their autonomic nervous system. One such
program, Heartmath®, has been successful in helping patients lower stress, anxiety, and
systolic blood pressure.
Our primary aim of this study is to evaluate the effects of an 8 week, virtually-based
heart coherence training program (HCTP) with the use of wearable, biofeedback technology.
The goal is to perform a randomized-controlled pilot study to assess effect estimates on
multiple outcomes in an effort to establish a foundation for a larger, longitudinal
trial.
The objective is to:
Randomize 20 patients with Vascular Ehlers-Danlos Syndrome from ages 12 -45 years to
current status and care (controls) versus a biofeedback intervention of additional
training and advancement on HeartMath techniques, then
Allow the control group subjects to then have access to materials related to the
techniques that were taught to the HMI group to allow for therapeutic equality. The
investigators will then compare outcomes between both the intervention and control
groups, and between the baseline and post-intervention states. Specific outcome
measures will include ambulatory blood pressure, heart rate variability, coherence
ratio, mean heart rate, quality of life/mental health assessment: health-related
quality of life, depression and anxiety screening scales.
Our hypothesis is that individuals with VEDS undergoing a supervised HCTP will
demonstrate improvements in ambulatory blood pressure, heart rate variability, and mental
health survey measures.