Additional Diseases of the Aorta

Marfan Syndrome

Life threatening genetic connective tissue d

Marfan Syndrome causes

no tensile strength/structural support, inc elasticity and an ineffective cross linking of collagen

Marfan Syndrome mostly affects the

AO (AO dilation/dissection and rupture)

Marfan Syndrome is caused by

75% have a hereditary link and 25% have a spontaneous mutation (first in the family to have it)

Marfan Syndrome Physical appearance

Slender/tall habitus, pectus excavatum (concave sternum) and long flexible extremities

Marfan Syndrome complications

tamponade and early death

Marfan Syndrome Echo Findings

AO Root dilation/dissection (most common), Abdominal Ao dilation/dissection (second most common)

Marfan Syndrome Diagnostic Testing

Genetic Testing, Eye Exam to detect if eye lenses are dislocated and CT/MRI to monitor AO diameter and dural ectasia

dural ectasia

widening or ballooning of the dural sac surrounding the spinal column

aortic root diameter

>5 cm needs an AO root replacement to save to AoV

Sinus of Valsalva Aneurysm

Rare saccular dilation that usually originates from the R coronary sinus (65-85%) or noncoronary sinus (10-30%)

Sinus of Valsalva Aneurysm Can mimic

ventricular tumor symptoms (ex RV mass)

two types of Sinus of Valsalva Aneurysm

Congenital (from localized weakness) or acquired (from compromised connective tissue)

Sinus of Valsalva Aneurysm complications

rupture

for Sinus of Valsalva Aneurysm diagnosis use

TEE/CT to confirm the diagnosis

Aortic Coarctation

Narrow AO usually near the AO isthmus (use ssn)