What does Loeys-Dietz syndrome do to the body?
What does Loeys-Dietz syndrome do to the body?
Loeys-Dietz syndrome can affect the heart and blood vessels. It can enlarge the aorta, the main blood vessel that carries blood from the heart to the rest of the body. Aortic enlargement can lead to a bulge (aneurysm) or tear (dissection) in the aorta.
Is Loeys-Dietz syndrome serious?
The most serious complication of Loeys-Dietz syndrome is the rupture of an aneurysm. To manage aneurysms, your child’s doctor may recommend: medications, such as beta blockers and Losartan, to help slow heart rate and lower blood pressure in the arteries to reduce the risk of rupture.
What is the life expectancy of someone with Loeys-Dietz syndrome?
Loeys–Dietz syndrome (LDS) is characterised by a mutation in the transforming growth factor beta receptor, and is strongly associated with aortic aneurysms and rupture. Most cases of LDS present in the second decade of life, with the average life expectancy being 37 years.
Is loeys-Dietz a heart disease?
Loeys-Dietz Syndrome – A Rare and Complex Heart Disease.
How many types of LOEY Dietz syndrome are there?
The five types of Loeys-Dietz syndrome are distinguished by their genetic cause: TGFBR1 gene mutations cause type I, TGFBR2 gene mutations cause type II, SMAD3 gene mutations cause type III, TGFB2 gene mutations cause type IV, and TGFB3 gene mutations cause type V.
What is the difference between Marfan and Loeys-Dietz syndrome?
Typically in Marfan syndrome surgery is considered when the aorta is around 5 cm; however, in Loeys-Dietz syndrome it has been recognized that individuals with aortic root measurements of 4 cm have shown aortic root dissection (in teens/adults). Therefore surgery is recommended when the aorta approaches this dimension.
How do you test for LOEY Dietz?
Getting Diagnosed with Loeys-Dietz Syndrome
- Echocardiogram.
- Either a computerized tomography (CT) or magnetic resonance (MR) angiogram (study of the blood vessels) with 3-D reconstruction, from the top of the head to the top of the legs.
Can you have EDS and LDS?
LDS and Ehlers-Danlos syndrome Clubfoot may be observed in both disorders. The disorders are different in that individuals with LDS have physical findings typically not present in individuals with vascular type EDS, such as widely-spaced eyes and bifid uvula.
