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National Marfan Foundation Leads Educational Efforts for Correct Diagnosis and Treatment of Aortic Dissection in Hospital Emergency Departments

Posted on: Monday, 11 February 2008, 15:00 CST

According to the National Marfan Foundation (NMF), patients with an aortic dissection (a tear in the large artery carrying blood away from the heart), can be saved if the correct diagnosis is made immediately and appropriate treatment is instituted. The NMF, which has been cited by the New York State Department of Health as the lead advocate in this country for people with aortic disease, has been instrumental in educating thousands of doctors and other emergency department personnel about how to recognize, diagnose and treat aortic dissection. Among the lead supporters of this initiative is the family of Tony Award-winning "RENT" playwright Jonathan Larson, who died in 1996. Larson was sent home from two different New York City hospitals -- one diagnosed him with the flu, the other food poisoning -- before he died alone in his apartment of an aortic dissection. He never got to see his hit play open on Broadway.

"It is incumbent upon emergency physicians to consider aortic dissection in people who present in the emergency department with chest pain, especially if other diagnostic possibilities cannot be confirmed," said Diane Sixsmith, MD, FACEP, Chair, Department of Emergency Medicine, New York Hospital Queens, and member of the NMF's Board of Directors.

Because of the catastrophic nature of an untreated aortic dissection, Sixsmith urges emergency physicians to rule out aortic dissection early in the evaluation process. Only with proper imaging -- an echocardiogram, MRI or CT scan -- can the aorta be fully observed. She asserts that a standard x-ray may not reveal a tear in the aorta.

Limited Suspicion of Aortic Dissection Leads to Deaths

Due to limited awareness or lack of suspicion of an aortic dissection among medical professionals, tragic cases continue to be reported to the NMF in which relatively young individuals have chest pains that are misdiagnosed by emergency physicians and, subsequently, die of an aortic dissection.

In some cases, such as that of Jonathan Larson, the patient exhibited many outward signs of Marfan syndrome, a genetic disorder that puts people at up to 250 times increased risk of aortic dissection. These physical signs include tall stature (or taller than other members of their family); disproportionately long arms, legs, fingers and toes; protruding or sunken chest bone; arm span greater than height; scoliosis; nearsightedness; and flat feet. About 200,000 people in the United States have Marfan syndrome or a related connective tissue disorder; about half of them are not diagnosed.

Other conditions that put people at increased risk for aortic dissection are:

 --  Bicuspid aortic valves --  High blood pressure --  Family history of aortic dissection, thoracic aortic aneurysms or     Loeys-Dietz syndrome --  Turner syndrome (a chromosomal disorder)      

"Ideally, people who are affected by Marfan syndrome or another disease that threatens their aorta have the vessel monitored yearly to detect aortic enlargement, the precursor to aortic dissection. They also take medications to slow the progression of enlargement. Surgery to repair the aorta when it reaches a critical size before it tears may also be required; this surgery has a greater than 98 percent success rate," said Dianna Milewicz, MD, PhD, professor and director of the Division of Medical Genetics at The University of Texas Medical School at Houston, and Chair of the NMF's Professional Advisory Board.

The NMF's Emergency Medicine Campaign

Preventing avoidable deaths from aortic dissection is the objective of the NMF's Emergency Medicine Campaign, which was developed with the support of the family of Jonathan Larson. The goal is to educate emergency department personnel about the risk factors for aortic dissection and how to correctly recognize, diagnose and treat a dissection.

The initiative features a continuing medical education (CME) video program about the emergency diagnosis and treatment of aortic dissection, accredited by Washington University School of Medicine in St. Louis; a booklet for emergency department nurses, "Aortic Dissection: Red Flags for the Emergency Triage Nurse"; an educational advisory for pre-hospital providers (devised by New York's State Emergency Medical Advisory Committee); and a four-color poster for non-patient areas in the hospital emergency department that was developed in conjunction with the New York State Department of Health.

Recognizing and Treating Aortic Dissection

Although aortic dissection is uncommon, it is not rare. Based on available medical information, there are approximately 5,000-10,000 dissections per year in the U.S. Experts believe that the number may be underreported because dissections that do not result in death or cardiovascular surgery may be recorded under a different diagnosis, such as chest or abdominal pain. In addition, because so few autopsies are conducted, many deaths are attributed to "heart attack" or "sudden death" when the cause is actually an aortic dissection.

An aortic dissection that doesn't stop tearing will ultimately become a fatal rupture. In fact, there is a fatality rate of more than 90 percent associated with acute aortic dissection originating near the heart without urgent surgical intervention by an experienced physician.

The primary symptom of an aortic dissection is severe pain, usually in the chest, but occasionally in the abdomen when the tear begins in the lower part of the aorta. Other signs are pallor, pulselessness, parathesiae and paralysis. An imaging study of the aorta -- a standard echocardiogram, magnetic resonance imaging (MRI), computed tomographic (CT) scan or transesophogeal echocardiogram -- can confirm or disprove the diagnosis. A standard chest x-ray cannot be considered conclusive in identifying an aortic dissection.

Upon diagnosis of a dissection, medications are prescribed to lower the blood pressure and slow the pulse. In a dissection that starts away from the heart, if the blood pressure stabilizes, the pain stops and there's no compromise of blood flow to the organs, then surgery can be avoided. However, symptomatic ascending aortic dissections or aneurysms do require emergency surgery.

According to a study published in the New England Journal of Medicine, while elective aortic surgery has an operative mortality rate of 1.5 percent, emergency aortic repair has an operative mortality rate of 12 percent.(1) Despite the increased risk during emergency surgery, lead investigator Vincent Gott, MD. Johns Hopkins Hospital, emphasized the need for emergency room physicians to consider Marfan syndrome when diagnosing very tall patients who come to the hospital complaining of chest pains.

The National Marfan Foundation

The NMF was founded in 1981 to provide accurate and timely information about the disorder to patients, family members and physicians; to serve as a resource for medical information and patient support; and to support and foster research.

For more information on Marfan syndrome and the NMF's Emergency Medicine Campaign, log on to www.marfan.org or call 800-8-MARFAN.

(1) Gott, et al, N Engl J Med 1999;340:1307-13.

 Contact: Eileen Masciale 631-665-2163 Email Contact

SOURCE: National Marfan Foundation

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