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Exercise with vEDS Siddharth Prakash, MD, PhD Associate Professor Department of Internal Medicine Division of Cardiology

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Exercise with vEDS

Siddharth Prakash, MD, PhD Associate Professor

Department of Internal Medicine Division of Cardiology

McGovern Medical School

• I have an aortic aneurysm. How can I remain active and live a productive and healthy life?

• What exercises do you recommend for people who are at risk for aortic dissections?

• After surgical repair of the aorta, what activity levels are appropriate?

Some Questions About Exercise

McGovern Medical School

What is my risk to have a vascular dissection?

0

Risk Factors: Hypertension Type of mutation Previous Aortic Surgery Pregnancy

1 2 3+

All vEDS patients are at increased risk, but the risk is not proportionately distributed

McGovern Medical School

How safe is exercise for patients who have aortic disease?

• Safety of moderate exercise was validated for patients with abdominal aortic aneurysms and may even decrease their rate of expansion

• Cardiac rehabilitation exercises after aortic repair appear to be safe (France, US, Swiss)

• In mice with Marfan syndrome gene mutations, moderate exercise slows down aortic disease

McGovern Medical School

• Interviews of dissection survivors showed that 25% were engaged in acute “vigorous physical activity” immediately prior to dissection

• The intensity, acuity and dynamic component of exertion differentiates beneficial exercise from harmful exertion that triggers dissections

• Uncontrolled hypertension is the number one risk factor for aortic dissection

• Being on prophylactic medical therapies lowers risk

How safe is exercise for patients who have aortic disease?

McGovern Medical School

Blood Pressure and ExercisePulse Pounding During Exercise is Distension of

Blood Vessels

Risk for Rupture: Aneurysm Thin Aortic Wall Hypertension

Risk markers: Type of mutation Vascular stiffness Previous Surgery

McGovern Medical School

Before starting exercise, control your blood pressure

McGovern Medical School

Lifelong follow up: Know your factors and disease status

All children and adults with vEDS require lifelong screening for cardiovascular disease

• Annual check up with primary care physician • Annual blood pressure checks and medication adjustments • Detailed imaging of blood vessels by cardiologist

• Computed tomography (CT) or Magnetic Resonance (MR)

• Every 6 months – 3 years, depending on disease status and location

McGovern Medical School

Taking a beta blocker can reduce your risk

Harleen Singh, Ph.D. http://slideplayer.com/slide/4850376/

Angiotensin Receptor Blocker – Mechanism

Focus: Blood vesselsFocus: Heart, Nervous System

Most people who had dissections were not taking adequate doses of blood pressure medication

McGovern Medical School

Risk of Exercise is Associated with Exercise Type

https://doi.org/10.1016/j.ejvs.2006.06.023 https://slideplayer.com/slide/4450334/

McGovern Medical School

1. Isometric and isotonic exercises i. Systolic blood pressure increases in proportion to the % of body

weight lifted ii. Increase is sudden and short-lived iii. SBP can not be readily measured during isometric exercise and

depends on technique and breath holds iv. Limited opportunity to adjust intensity of exercise before threshold

SBP is reached 2. Aerobic exercises

i. Systolic blood pressure increase is more gradual but is more prolonged

ii. Increase is not predictable from baseline blood pressure iii. SBP can be readily measured during aerobic exercise iv. Provides opportunity to adjust intensity of exercise before

threshold SBP is reached

Risk of Exercise is Associated with Exercise Type

McGovern Medical School

3 METS

walking hiking4-6 METS

sex

Check your METS: https://epi.grants.cancer.gov/atus-met/met.php Metabolic Equivalent (MET) Values for Activities in American Time Use Survey

4-6 METScycling

Risk of Exercise is Associated with Exercise Intensity

STATIC

DYNAMIC

McGovern Medical School

McGovern Medical School

“Systolic BP during exercise remained <150 mm Hg in 25% of the patients, was between 150 and 160 mm Hg in half of the patients, and averaged 160 to 170 mm Hg in the remaining (25% of) patients.”

Risk is Associated with Exercise Systolic Blood Pressure

McGovern Medical School

Prolonged hypertension after exercise

Risk is Associated with Pattern of Blood Pressure Rise During Exercise

120 140 165 180 210 170

SBP

Exercise-induced hypertension

30

Recovery

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Risk is Associated with Vascular Stiffness

Inflatable brachial cuff Adds in wave reflection from periphery

Calculates central aortic pressure

“Arterial Waveform Analysis”

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Risk is Associated with Vascular Stiffness

Minimal augmentation: normal stiffness

53 year old man with vEDS and no dissections Brachial pressure 116/76

McGovern Medical School

Risk is Associated with Vascular Stiffness26 year old woman with vEDS and previous dissections Brachial pressure 105/62

Increased augmentation: increased stiffness

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Components of Hypertension

Vascular Stiffness

Ambulatory Hypertension

Exertional Hypertension

Total Risk Due to Hypertension

Measure and treat all three components to improve outcomes in high risk patients with vascular disease

McGovern Medical School

VEDS Exercise Issues

• Avoid chiropractors for neck and upper back pain • Use caution when cycling or squatting, especially with

iliac artery aneurysms or dissections • Use stool softeners and avoid straining with bowel

movements

McGovern Medical School

• You can lift whatever weight you can comfortably lift without having to "bear down." Additionally, isometric exercises are not recommended. Isometric exercises are those where one contracts muscles but does not move joints. These actions increase pressure on the aorta and may trigger an acute dissection. Your physician can provide a letter explaining these restrictions if needed. • We recommend daily light to moderate aerobic exercise • What about more intense exercise regimens (marathon) or

patients with persistent hypertension who want to exercise?

Common Exercise Issues

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Treadmill exercise test

Echocardiogram/CT/MR

History/Physical

Targeted Antihypertensive Therapies Exercise Prescription

Exercise Hypertension Activity Thresholds

Assessment of Hypertensive or Highly Active Thoracic Aortic Disease Patients

Mutation Type/Family History Contraindications to Exercise

Vascular Stiffness Cardiac disease

Baseline Hypertension

Extent of Dissection Estimate aortic quality Assess disease pattern

24hr ambulatory BP Timing of exercise Timing/dose of medications

Genetic Evaluation

ECG and Waveform analysis