The danger with an aneurysm is two fold:

1. An enlarged aorta may compress the neighboring structures, and causing symptoms. For example tracheal compression causing difficulty in breathing and pain due to nerve compression or compression of the blood vessels and their blockage.

2. More importantly, with little warning, the aneurysm could rupture and cause excessive bleeding and shock. If not treated immediately, a ruptured thoracic aortic aneurysm is fatal. A person with an unruptured aneurysm might not feel any symptoms; other times, there is pain in the back and shoulders, hoarseness, and a dry cough. A ruptured aneurysm, however, brings on sharp, excruciating chest pain.

“Ideally, we’re able to identify an aneurysm before it ruptures through an echocardiogram, computed tomography or magnetic resonance imaging scans,
or chest X-ray so we can map a strategy for repair,” says Dr. Ahmad Rajaii Khorasani, a cardiac surgeon at University Hospital. “These are complex operations designed to eliminate the danger of rupture while ensuring the safe distribution of blood to the rest of the body. Once an anuerysm ruptures, it’s a true surgical emergency.”

In theory, aneurysm repair is simple: replace the damaged section of aorta with a graft of special surgical woven material. In practice, these open-heart operations are extremely complicated, depending on where the aneurysm is located. (See “Where the Aorta Travels.”) For example, a repair near the heart might also require replacement of the aortic valve. There’s also the possibility of complications. “The aorta is the trunk of the circulatory tree,” says Dr. Khorasani. “The techniques used in these operations have the potential to affect every organ system in the body.”

What’s important is that the surgeon is experienced in performing these very specialized procedures and knows when–and when not to–operate especially when the aneurysm is small. Sometimes it’s in the patient’s best interest to initially treat the condition with medicine, such as beta blockers, which can enable the heart to beat with less force, or antihypertensives to lower blood pressure.”

Dr. Rajaii Khorasani has the expertise, from his training, practice and years of innovative and problem oriented research in the area of prevention of stroke and paralysis, two disasterous complications associated with thoracic and thoracoabdominal aortic surgery. Since January of this year, he has managed about 10 cases, a high number considering that this condition is very uncommon. His innovative technique of perfusion of the brain and spinal cord blood vessels have been effective in avoiding these complications.

Another potentially fatal condition of the heart’s main artery is aortic dissection. “Aortic dissection can be compared to the treads of a tire peeling apart,” says Dr. Khorasani. “The inner layers of the aorta tear, leaving only an outermost, very thin layer. Blood forces its way between the layers, which can lead to heart attack or stroke.”

As with thoracic aortic aneurysm, the symptoms of aortic dissection can be gradual and subtle until the situation is severe; then, there is typically sharp pain in the back or below the breast bone. In some cases of dissection, early treatment with antihypertensives or beta blockers may be best. But in other cases, dissection requires emergency surgery.

Where the Aorta Travels Click here for a more detailed explanation of how the aorta works.

A thoracic aortic aneurysm can occur for a variety of reasons, says Dr. Khorasani, including atherosclerosis, a build up of plaque commonly referred to as hardening of the arteries, and more rarely, untreated syphilis. Hypertension, atherosclerosis, or an injury to the chest can all lead to aortic dissection; sometimes dissection occurs in an aortic aneurysm. Women who are pregnant or have Turner’s syndrome, a chromosomal disorder, are also predisposed to aortic dissection.

But one group of people at high risk for both thoracic aneurysm and dissection are those who have Marfan syndrome, a rare, inherited condition involving the body’s connective tissue.

People with Marfan syndrome are typically tall and thin; their arm span often exceeds their height. Abraham Lincoln, for example, is believed to have had the syndrome. They may also have scoliosis (curvature of the spine) and visual problems such as nearsightedness or detached retina. But the most severe ramifications of Marfan syndrome involve the heart.

“Because their connective tissue isn’t formed properly, the aorta in Marfan syndrome patients can progressively widen, making it more susceptible to becoming an aneurysm,” says Dr. Khorasani, who has treated many Marfan patients. “The layers within the aorta are also more fragile and subject to tear, resulting in aortic dissection.”

“It is quite possible for a person, seemingly in good health, to be unaware he or she has Marfan syndrome well into adulthood, until an aneurysm or dissection occurs,” he continues. “Obviously, our first priority is that patient, but we also are concerned about that person’s blood relatives, who might also unknowingly have Marfan syndrome. It is very important that these family members be examined and evaluated for the syndrome.”

When an aneurysm or dissection is diagnosed in the early stage, the better the possibility exists for a positive outcome. “Sometimes it is tempting for people to dismiss mild chest pain as nothing serious,” says Dr. Khorasani. “But especially for those with Marfan syndrome or a history of atherosclerosis, waiting too long to seek help can have serious consequences.”