Ritter lawsuit once again raises concerns about proper diagnosis of Aortic Dissection

National Marfan Foundation is Lead Advocate for Education on
Emergency Diagnosis and Treatment of Aortic Dissection

PORT WASHINGTON, NY, September 10, 2004 – According to the National Marfan Foundation (NMF), patients with an aortic dissection (a tear in the large artery carrying blood away from the heart), such as actor John Ritter – whose family filed a wrongful death suit yesterday – can be saved if the correct diagnosis is made immediately and appropriate treatment is instituted.

The NMF has been leading the charge to educate emergency medicine health care providers about recognizing aortic dissection since 1996, when Tony Award-winning Rent playwright Jonathan Larson died alone in his apartment of an aortic dissection after being sent home from two different New York City hospitals (one diagnosed him with the flu, the other food poisoning). His hit play opened the day after he died.

Larson had undiagnosed Marfan syndrome, a connective tissue disorder that increases the risk of aortic dissection 100 times, as compared to the general population. Other groups at high risk of aortic dissection are those with high blood pressure, bicuspid aortic valves, Ehlers-Danlos syndrome and Turner syndrome (a chromosomal disorder that can affect women).

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 in a hospital by emergency physicians and subsequently die of an aortic dissection.

The NMF’s Emergency Medicine Campaign

Preventing these avoidable deaths 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 room personnel about the risk factors for aortic dissection and how to correctly recognize, diagnose and treat a dissection.

“We know that our Emergency Medicine Campaign is having an impact because of the positive feedback from leading physicians. In addition, a Seattle woman advised us that she survived an aortic dissection because the physicians at the hospital had just seen the NMF video on emergency diagnosis and treatment,” said Carolyn Levering, NMF President and CEO. “However, despite all of the national emergency medicine meetings attended, medical grand rounds presented and publicity that has been generated, there are still emergency medicine professionals who do not have aortic dissection on their radar when they see patients with chest pain. As a result, there are still tragedies that can be prevented.”

At the center of the Foundation’s campaign is 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. Accompanying the video are materials developed by the NMF in cooperation with the New York State Department of Health as part of the medical education initiative instituted following the State’s investigation into the death of Larson. These include a booklet for physicians, a four-color poster for non-patient areas in the hospital emergency department and an educational advisory for pre-hospital providers (i.e., emergency medical technicians), which was devised by New York’s State Emergency Medical Advisory Committee (SEMAC).

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., but 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. Despite the increased risk during emergency surgery, lead investigator Vincent Gott, M.D., Johns Hopkins Hospital, emphasized the need for emergency room physicians to consider Marfan syndrome when diagnosing patients with chest pains who exhibit outward signs of the disorder, such as extreme height.

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 the Marfan syndrome, contact the NMF at 800-8-MARFAN or www.marfan.org.