4.10 Aneurysm of aorta or major branches, due to any cause (e.g., atherosclerosis, cystic medial necrosis, Marfan syndrome, trauma), demonstrated by appropriate medically acceptable imaging, with dissection not controlled by prescribed treatment (see 4.00H6).

An aneurysm is a bulging weakness in the wall of the aorta or one of its major arterial branches, and is considered “dissecting” when the inner lining of the artery begins to separate from the outer arterial wall. This tearing in the wall of the artery results in development of a blood clot (hematoma) within the wall of the artery that grows and blocks blood flow. Sometimes, the dissection results in complete rupture of the wall of the artery causing significant blood loss and possible death.

Trauma and atherosclerosis (formation of cholesterol plaque) can weaken walls of arteries resulting in an aneurysm and dissection. Cystic medial necrosis is a term used to described the pathologic changes that occur in the lining of the arterial wall and is classic for changes that occur in arterial dissection in Marfan Syndrome. See the end of this article for more information about Marfan Syndrome.

Appropriate medically acceptable imaging may include angiography, computed tomography (CT scan), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and heart ultrasound including transthoracic echocardiography (ECHO) and transesophageal echocardiography (TEE).

SSA considers the dissection “not controlled” when you have:

  • persistence of chest pain due to progression of the dissection
  • an increase in the size of the aneurysm
  • or compression of one or more branches of the aorta supplying the heart, kidneys, brain, or other organs

A dissecting aneurysm can cause heart failure, renal (kidney) failure, or neurological complications due to lack of blood flow (ischemia) to organs. Symptoms (sensations) of aortic dissection may include chest pain, jaw pain, neck pain, altered mental status, syncope (passing out), focal weakness or numbness, shortness of breath, and trouble swallowing.

If you have an aneurysm that does not meet the requirements of 4.10 but you have one or more associated organ damage, SSA will evaluate the condition(s) using the appropriate Listing for the affected body system.
Disease that affect the lining of the aorta and predispose it to dissection include hypertension, connective tissue disorders (like Marfan Syndrome), congenital aortic stenosis (narrowing of the aortic valve), and bicuspid (split) aortic valve.  

Marfan syndrome

Is a genetic connective tissue disorder that affects multiple body systems, including the skeleton, eyes, heart, blood vessels, nervous system, skin, and lungs. The diagnosis is made by medical history, including family history, physical examination, including an evaluation of the ratio of arm/leg size to trunk size, a slit lamp eye examination, and a heart test(s), such as an echocardiogram. In some cases, genetic testing may be useful, but such analyses may not provide any additional helpful information.

The effects of Marfan syndrome can range from mild to severe. In most cases, the disorder progresses as you age. Most individuals with Marfan syndrome have abnormalities associated with the heart and blood vessels. Your heart’s mitral valve may leak, causing a heart murmur. Small leaks may not cause symptoms, but larger ones may cause shortness of breath, fatigue, and palpitations. Another effect is that the wall of the aorta may be weakened and abnormally stretch (aortic dilation). This aortic dilation may tear, dissect, or rupture, causing serious heart problems or sometimes sudden death. SSA will evaluate the manifestations of your Marfan syndrome under the appropriate body system criteria, such as 4.10, or if necessary, consider the functional limitations imposed by your impairment.