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Medical Ignorance Contributes to Toll From Aortic Illness

Many Doctors Don't Realize Aneurysms Are Treatable;
A Paucity of Specialists

Updated November 4, 2003

By KEVIN HELLIKER and THOMAS M. BURTON
Staff Reporters of THE WALL STREET JOURNAL

After aortic aneurysms struck her ex-husband and his brother, Debra McMillan learned that the often-fatal condition can be hereditary. So last autumn, when pain suddenly ripped through the chest of her 19-year-old son, she rushed him to an emergency room in Omaha, Neb., and told the doctor about the family's medical history.

But she says the ER doctor at Nebraska Methodist Hospital dismissed her talk of aneurysms, which are bulges that can lead to lethal ruptures in the aorta, the body's largest artery. Instead, the doctor diagnosed Ms. McMillan's son, Tyler Kahle, with a minor lung infection and sent him home, she says. In following days, the young man and his mother visited their family physician and returned to Nebraska Methodist. During each visit, she asked whether her son's continuing chest pain could be connected to the family history of aneurysms. Each doctor answered no -- but without ever examining Mr. Kahle's aorta, she says.

Eight days after his symptoms began, the young man died of a ruptured aorta. Ms. McMillan has filed a malpractice suit against Nebraska Methodist in state court in Douglas County, Neb. The hospital has denied any liability, saying it provided adequate care.

The Kahle case illustrates a deadly discrepancy between the available medical knowledge about aortic aneurysms and the ignorance of many front-line physicians. The discrepancy contributes to what may be thousands of unnecessary deaths each year.

A common misconception among physicians is that aortic disease is rare, when in fact it kills an estimated 25,000 Americans a year. That is a larger toll than that of AIDS and most kinds of cancer. Another misconception is that little can be done about aortic aneurysms. The reality is that improvements in diagnostic-scanning methods introduced since the 1980s, and greater experience with aortic surgery, have vastly enhanced the ability to detect and repair aneurysms.

Behind these misconceptions is an anomaly in the medical profession's structure: There is no medical specialty devoted to treating or educating others about the aorta.

Aortic disease "falls squarely between about four different specialties," says Eric Isselbacher, a cardiologist at Harvard Medical School. "There's an education gap among physicians about aortic disease, and this gap isn't small. It's huge."

The depth of medical unfamiliarity with this illness became clear in September, following the sudden death of Hollywood star John Ritter, 54. He suffered an aortic dissection, a tear in the weakened wall of an aneurysm. Although dissection kills quickly by disrupting blood flow to major organs, the aneurysm that typically causes this event takes years to grow, during which time it can be detected and removed. Yet in media interviews after Mr. Ritter's death, doctor after doctor described dissection as rare and undetectable.
Such comments disheartened aortic experts. "I've been distressed by the series of physicians getting on the screen and calling this kind of death unpreventable," says Dianna Milewicz, director of medical genetics at the University of Texas Health Sciences Center in Houston. "The message should have been that John Ritter's children should be screened for this," adds John Elefteriades, chief of cardiovascular surgery at Yale-New Haven Medical Center. It hasn't been disclosed whether Mr. Ritter ever was checked for aortic problems.

Despite the diagnostic and surgical breakthroughs, recent academic studies suggest that there has been little or no improvement in a longstanding misdiagnosis rate of about 35% for aortic dissections, compared with about 5% for heart attack. Even with rupturing abdominal aortic aneurysms -- a variety that tends to strike a highly identifiable group, men over 60 with a history of smoking and arteriosclerosis -- studies have found a misdiagnosis rate of about 30%. The in-hospital mortality rate from aortic dissection hasn't declined in decades.

"No physician can diagnose a condition he never thinks about," observes Michael DeBakey, an inventor of aortic aneurysm-replacement surgery in the 1950s and, at 95, still on staff at Methodist Hospital of Houston.

Medical News

This isn't the first time doctors have been slow to absorb news of medical advances. Hungarian physician Ignac Semmelweis discovered in 1847 that merely by washing their hands, physicians could avoid spreading infection. But medical leaders resisted his teachings, and hand-washing didn't gain wide acceptance for years. In the 1840s, rudimentary communication slowed the spread of knowledge. Today, many physicians complain they are so swamped by information -- from journals, drug and device companies, and continuing-education courses -- that they can't absorb all of the latest news.
In the case of aortic disease, this problem is heightened by the lack of blood-vessel specialists dedicated to getting out the word about aneurysms. Every other significant body part -- brain, heart, lungs, bones and so on -- boasts its own specialty association. Fifteen-thousand podiatrists in the U.S. focus on feet.

The aorta is the River Nile of blood vessels. It rises from the heart nearly to the neck, then descends through the chest and abdomen, carrying blood for every organ and limb. But only about 300 nonsurgical doctors in the U.S. specialize in blood vessels. Cardiologists are responsible for the cardiovascular system, and typically are very knowledgeable about the tiny coronary arteries that channel blood from the aorta back into the heart. But their training leaves many of them in the dark about aortic disease.

A small corps of aortic experts from various specialties are beginning to focus more attention on aneurysms. But there is a long way to go, especially in medical schools, where the experts say aortic problems typically receive inadequate study.

During eight years as a medical student and resident at West Virginia University, obstetrician Devin Ciliberti says he rarely heard any mention of aortic illness. "If it ever came up, it was like, 'This goes at the bottom of your list' " of possible diagnoses, says the physician, who finished his residency in 2001. In particular, research suggesting that pregnancy heightens the risk of dissection never came up, Dr. Ciliberti adds.

This all became relevant when 25-year-old Julie Neal Lee came to The Women's Hospital of Greensboro, N.C., last November. She was 37 weeks pregnant and in extreme distress, but clearly not in labor, Dr. Ciliberti says. He says he ordered tests for kidney stones, a pregnancy-related high-blood-pressure condition called pre-eclampsia and anything else he could think of. All proved negative. Hours passed, and the young woman was frantic with pain.

Finally, Dr. Ciliberti ordered a computerized-tomography, or CT, scan of Ms. Lee. "Even then, I wasn't thinking about aortic dissection," he says. The scan, taken more than seven hours after she arrived, showed an aortic dissection. Dr. Ciliberti performed an emergency Caesarean, saving the baby. But Ms. Lee died after aortic-repair surgery by another doctor.

Her parents, Harold and Robin Lee, say they blame Dr. Ciliberti for failing to diagnose the problem sooner, but they haven't gone to court. Dr. Ciliberti says, "I don't think a quicker diagnosis would have saved her, but I don't know for sure." He attended Ms. Lee's wake and funeral and says he has spent much of the past year learning about aortic dissection. Women's Hospital declines to comment.

Driving Progress

Specialists drive most medical progress, educating generalists and promoting prevention. Twenty years ago, few Americans had heard of prostate cancer, but urologists have spurred screening and awareness campaigns, and U.S. deaths from that disease fell 21% between 1990 to 2000.

With no comparable campaign, unfamiliarity with aneurysms prevails in many emergency rooms and physicians' offices. Michael Giusti, 44, entered the ER at Methodist Medical Center in Peoria, Ill., one night in June 1998, complaining of chest pain and asking whether his aorta should be scanned, says his wife, Kathy Schwindenhammer, who accompanied him. For 13 years, he had been undergoing scans to monitor an aortic aneurysm that previously hadn't caused any symptoms and only now was approaching a dangerous size, she says. In the ER, two residents picked up a textbook and began flipping pages before concurring with the primary physician on duty that a scan wasn't needed, she says.

In fact, aortic experts say that any person with an aneurysm who experiences significant chest pain ought to have a scan done. But the doctors at Methodist Medical diagnosed Mr. Giusti with a pulled chest muscle and sent him home, his wife says. He died there that day of an aortic dissection.
The hospital has denied any negligence. But in 2002 it agreed to pay Ms. Schwindenhammer $850,000 to settle a suit she had filed, Illinois state-court records show.

MISSING THE SIGNS

The high death rate from aortic disease is partly due to physicians' unfamiliarity with the symptoms, the groups that face high risks, and available diagnostic and surgical procedures. Julie Neal Lee, 25, died November 2002, Greensboro, N.C.: Pregnancy is a huge risk factor for women with aortic disease. But her obstetrician says his medical training barely touched on the condition.

Eric Eshleman, 28, died September 2000, Atlanta: He entered the ER with sudden back pain. His lanky appearance signaled he might have Marfan's, a condition that makes the aorta prone to dissect.

Twenty years ago, diagnosing aneurysms was extremely difficult, and surgery to repair the condition had a high mortality rate. The fatalism that surrounded the ailment -- which can stretch a vessel normally the diameter of a garden hose to that of a soda can -- was captured by a comment a century ago by medical pioneer William Osler: "There is no condition more conducive to clinical humility than aneurysm of the aorta."

Today, this shouldn't be true. Aortic aneurysms don't show up well on X-rays. But the advent of high-tech scans -- such as CT; abdominal ultrasound; magnetic-resonance imaging, or MRI; and echocardiogram -- have made aneurysms relatively easy to catch. (The scans cost from $40 to $2,000, depending on the aneurysm's location.) Medical geneticists have identified high-risk groups in whom the condition ought to be suspected. And with experience, surgeons have improved to roughly 90% the success rate of replacing damaged sections of aorta with Dacron hose.

One obstacle to disseminating information on the aorta is corporate profit. Medical-device and drug companies, which are playing an increasingly large role in shaping continuing-education seminars, tend to focus on products they sell, such as coronary stents, which are used to prop open clogged coronary arteries. Industry hasn't developed a comparable product for repairing aneurysms that is inexpensive or effective enough to replace most surgery. For cardiologists trying to keep up with their field, "pharmaceutical and device development for the coronary arteries is where the money and glamour are," says Harvard's Dr. Isselbacher.

Some heart doctors don't even realize that action can be taken. When an echocardiogram -- a scan of the heart and surrounding vessels -- found a large aneurysm in the chest of Donald Kehe four years ago, his cardiologist in Las Vegas called a private meeting with Mr. Kehe's wife. "He took my hands in his hands, looked me in the eyes and said there was no hope -- that Donald should tell his loved ones goodbye," says Rowena Kehe.
Sandy Morris, 13, died July 1998, Columbus, Ohio: When her parents took her to the ER with severe chest pain, they knew enough to request a high-tech scan. But the hospital didn't scan her aorta; four hours later, she died.

Daniel Slaughter, 37, died May 2001, Indianapolis: He entered the hospital with symptoms of dissection and a classic consequence: blood in the sac around his heart. Yet he went undiagnosed for a week.

After a friend pointed Mr. Kehe, then 69, toward Cedars-Sinai Medical Center in Los Angeles, Sharo Raissi, that hospital's top cardiovascular surgeon, removed the aneurysm. A few months later, instead of telling his family goodbye, Mr. Kehe treated them to a Hawaiian vacation. Mr. Kehe, now 71, is alive and well today.

Classic Symptom

In some aneurysm cases, the knowledge gap is especially clear because multiple cardiac doctors miss danger signs. Daniel Slaughter, a 37-year-old father of four, entered Methodist Hospital of Indianapolis in May 2001, experiencing chest pain radiating into his neck. That is a typical symptom of aortic dissection. He was bleeding into the sac around the heart, a common consequence of aortic dissection. And an echocardiogram found that his aorta was 50% larger than normal, according to a hospital report.

Yet the cardiologist who signed the echocardiogram report noted in it that the heart and aorta looked normal. A second cardiologist and a cardiac surgeon never looked at the echocardiogram, according to subsequent written statements they made in administrative proceedings. A week after entering Methodist, Mr. Slaughter died. After learning in the autopsy room that the cause of death was aortic dissection, the cardiac surgeon called Mr. Slaughter's widow. "He said, 'This probably won't help you now, but I could have saved him,' " says Paige Slaughter. She has named the hospital and three doctors in a proceeding that Indiana requires before the filing of a malpractice suit. Methodist Hospital denies any negligence.

Dr. Elefteriades, the top aortic surgeon at Yale-New Haven, and Craig Miller, his counterpart at Stanford Medical Center, say they are each asked about twice a month by lawyers for plaintiffs and defendants to review cases alleging malpractice related to aortic disease. In only about half are the doctors' or hospitals' actions legally defensible, say the doctors, who are paid for their opinions but typically don't testify in court. Both physicians say that doctors' performance in heart-attack cases they review is defensible far more often.
Banding Together

At some hospitals, including Massachusetts General in Boston, cardiologists, surgeons and other physicians are banding together to form aortic centers that draw on a range of specialties. In June, the American Heart Association published in its journal, Circulation, an article on heredity and aortic aneurysms. Still, AHA President Augustus Grant says, "I don't think aortic dissection is analyzed with the frequency it should be" at cardiac conferences.

Aortic disease generally strikes two types of victims. The first are men typified by James Whitehead, a University of Arkansas professor who at 67 had a long history of smoking and high blood pressure. This August, he experienced sudden, intense pressure in his chest, radiating into his jaw. At Washington Regional Medical Center in Fayetteville, Ark., he tested negative for a heart attack but remained stricken by pain so intense that morphine failed to numb it, his family says. Eight hours after his arrival, doctors did the CT scan that revealed he had an aortic dissection, and by then, it was too late, his family says. He died before reaching the operating room. Washington Regional declines to comment.

The second type of aneurysm victims are young, fit people cursed with a genetic predisposition for aortic problems. Most people in this category don't know they have an aneurysm, although family history can provide a clue.
So can body type. Aortic experts say that especially tall, lanky people entering an emergency room suffering sudden and intense chest or back pain ought to be considered possible aneurysm victims. People with strikingly long limbs may have Marfan's syndrome, a connective-tissue disorder, and Marfan's sufferers statistically have a much-greater-than-average risk of dissection.

Eric Eshleman, 28 years old, 6-foot-8 and 190 pounds, entered Atlanta's Northside Hospital in September 2000 suffering sudden, severe back pain. His wife, Britt Eshleman, says it was the first time she had ever seen him cry. Neglecting to scan his aorta, the hospital prescribed painkillers and sent him home, his wife says. Seven days later, he died of an aortic dissection. The county autopsy report describes him as "marfanoid appearing."

Ms. Eshleman has sued Northside for malpractice in state court in Fulton County, Ga., alleging that based on her husband's body type, among other factors, the hospital should have tested more aggressively for aortic dissection. The hospital says the suit "is without merit."

The seriousness of aortic dissection is lost on many doctors. Sandy Morris, 13, arrived in July 1998 in the emergency room at Ohio's Columbus Children's Hospital, complaining of intense chest pains. Her parents knew their daughter had Marfan's, and they say they knew the pain might indicate an aortic dissection. They even knew enough to request an echocardiogram. But doctors failed to do one, testing Sandy instead for heart attack, the Morrises say. That test came back negative, because Sandy was having an aortic dissection, the parents say. Court records show that doctors scheduled an MRI scan but for the following morning, about eight hours after Sandy had arrived at 11 p.m. She didn't live that long. "Why don't they do something, Daddy?" were the last words Andrew Morris says he heard his daughter speak.

Children's Hospital has settled a malpractice suit filed by the parents in state court in Columbus on terms that weren't disclosed. The hospital declines to discuss the case. In 2000, Children's Hospital and the Ohio State University Medical Center, which share faculty, opened a Cardiovascular Connective Tissue Disorders Clinic. That unit serves patients with Marfan's and others who have a genetic predisposition to develop aortic disease.
Extreme Pain

Aortic dissection is one of few conditions that causes pain so severe it often isn't relieved by morphine, experts say. Even so, after doctors rule out heart attack, they sometimes neglect to test patients experiencing this level of chest or back pain for aortic problems.

Christopher Cole, 39, a manufacturing executive in Elyria, Ohio, once broke his leg in six places in an amateur motorcycle race. His foot ended up pointing backward, he says. On another occasion, the South Africa native was hit by shrapnel while serving in that country's military in the 1980s. The pain from his aortic dissection 14 months ago was far worse than from either of those injuries, he says. "When my heart would beat, it felt as if my skin was tearing," he says.

But it took doctors an alarmingly long time to conclude that anything was wrong with his aorta. When he arrived at Elyria Memorial Hospital, near Cleveland, in August 2002, doctors and nurses ran various tests, but not a scan that would have shown the dissection, he says. Mr. Cole stayed overnight at the hospital, and the next morning a cardiologist told him they couldn't find anything wrong and he could go home. Mr. Cole did, but his pain grew worse. It took two more visits to the ER the next day before doctors finally gave him a CT scan. When that showed a dissection, he was flown immediately by helicopter to the Cleveland Clinic. Lars G. Svensson, the clinic's chief aortic surgeon, performed successful emergency surgery.

Dr. Svensson says Mr. Cole probably wouldn't have survived more than another two hours without it. The surgeon estimates that every second or third aneurysm case he gets was originally misdiagnosed. An Elyria Hospital spokesman declines to comment.

Aortic dissection and rupture are fatal far more often than heart attack. As a result, some doctors are aggressive about testing for aortic disease. When Howard Carney entered St. Luke's Hospital in Kansas City, Mo., last year, complaining of sudden, intense chest pain, Dr. Lance Waldo immediately ordered a CT scan that showed an aortic dissection. Mr. Carney, 36, underwent emergency surgery and today is fine. "I'm paid to be a pessimist," says Dr. Waldo.

Not every case of aortic rupture or dissection can be diagnosed. Composer Jonathan Larson died of an aortic dissection in 1996 after two New York City hospitals misdiagnosed him. The 35-year-old's death drew widespread attention because it came after the final dress rehearsal of his show "Rent," the rock opera that went on to huge success. Yet Diane Sixsmith, one of the physicians charged by New York state medical authorities with investigating the case, concluded no negligence occurred. Mr. Larson had complained only of flu-like symptoms, and it would have been a huge leap to guess that he had a disintegrating aorta, says Dr. Sixsmith, chairman of emergency medicine at New York Hospital Queens Medical Center and a leader in efforts to educate physicians about aortic disease.
Pregnant Patient

Many aortic dissections and ruptures involve aneurysms that doctors spot but fail to treat. An echocardiogram picked up Lori Irving's aortic aneurysm in 1998, her mother, Patty Irving, says. But her cardiologist, who was employed by Kaiser Permanente, said nothing about it, the mother adds. The younger Ms. Irving, a psychology professor at Washington State University in Vancouver, Wash., was then 35. In mid-2000, she became pregnant. Aortic experts say that any woman of child-bearing age who has an aneurysm should be warned that pregnancy severely compounds the dangers. "We'd never have gotten pregnant if we'd known about the risk factor," says Mike Morgan, Lori's husband.

When intense chest pain sent Ms. Irving to the emergency room at Southwest Medical Center, a Kaiser Permanente hospital in Vancouver, during the last month of her pregnancy in April of 2001, she had no way of knowing the cause. Doctors didn't take an echocardiogram, her mother says. They diagnosed the 38-year-old patient with indigestion and sent her home, her mother says. That same day, Lori Irving and the unborn baby died.
Kaiser declines to comment, citing a settlement and confidentiality agreement with Lori Irving's husband.

Father and Son

Some physicians hope that the story of Tyler Kahle's family could help educate the profession about the dangers of aneurysms. An article scheduled to appear in the winter issue of the Annals of Emergency Medicine describes the failure of three sets of medical personnel in Omaha to scan the aorta of Mr. Kahle, the 19-year-old whose mother rushed him to the emergency room and told doctors about the family's medical history. "Scanning him very likely would have saved his life," says Dr. Milewicz, the University of Texas genetics expert who co-wrote the journal article.

In August 2001, about a year before Tyler's death, his uncle, Tom Kahle, had entered St. Luke's Hospital in Cedar Rapids, Iowa, complaining of chest pain. He told doctors about his family's history of aneurysm, relatives say. But the hospital discharged him without scanning his aorta, the relatives add. Two days later, Tom Kahle, 37, died of an aortic dissection. His family has filed a negligence suit against St. Luke's in state court in Linn County, Iowa. The hospital has denied any liability.

Terry Kahle, Tom's brother and Tyler's father, survived a dissection in 1998. After attending Tyler's funeral in Omaha last year, Terry Kahle returned to his home in Atlanta with his older son, Marcus, 23. Almost immediately, Marcus started complaining of chest pains. "I figured it was the power of suggestion, but I wasn't taking any chances," the father says.

Rushing his son to the emergency room at St. Joseph's Hospital in Atlanta, Mr. Kahle says he requested a scan of the young man's aorta -- only to be told that aortic disease didn't strike people that young. Mr. Kahle, an auto technician who says he had never stood up to a doctor before, did so then.

"There were tears in my eyes," he says. "I said, 'Listen, I just buried my 19-year-old son last week, and I buried my brother last year -- both of them aortic aneurysms. We're not leaving here until you scan my son.' "

After getting scanned, Marcus Kahle underwent emergency surgery to repair an aortic aneurysm. Today, he is alive and well in Atlanta.

Write to Kevin Helliker at kevin.helliker@wsj.com and Thomas M. Burton at tom.burton@wsj.com

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< 美國企業新聞社 > [l] 2001-10-05
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TV Tragedy on 'ER' Too Often Imitates Reality: National Marfan Foundation Working to Save Lives Otherwise Lost in Emergency Departments

PORT WASHINGTON, N.Y., Oct. 4 /PRNewswire/ -- 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 the character on tonight's episode of "ER" (NBC-TV) whose dissection is initially misdiagnosed and dies as a result, can be saved if the correct diagnosis is made immediately and appropriate treatment is instituted. For Lee Norton, the 21-year-old who portrays the dying patient on "ER," this message hits close to home because aortic dissection claimed the lives of his brother, his uncle and his aunt.

Both Lee and his mother -- like his other relatives -- are affected by the Marfan syndrome, a genetic disorder of connective tissue, which increases the risk of aortic dissection 100-fold. Other risk factors for aortic dissection are high blood pressure, bucuspid aortic valves and Turner syndrome (a chromosomal disorder that can affect women).

"In my family, it took the death of my 15-year-old brother for us to learn about the Marfan syndrome, but it's not too late for others," said Lee, currently a student at the University of Utah whose diagnosis led to his interest in becoming a cardiothoracic surgeon. "I hope viewers realize that someone with the Marfan syndrome can appear very 'normal' until disaster strikes. By recognizing the characteristics of the disorder, people can seek diagnosis and treatment, and doctors can recognize a potential crisis before it happens."

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 in a hospital by emergency physicians and subsequently die of an aortic dissection. The most high profile case was the death of Tony Award-winning Rent playwright, Jonathan Larson, who was sent home from two different New York City hospitals -- one diagnosed him with the flu, the other food poisoning -- only for him to die alone in his apartment of an aortic dissection before his hit play opened on Broadway.

Larson, like many people who have the Marfan syndrome, exhibited many outward signs of the disorder, which include tall stature (or taller than other members of their family); disproportionately long arms, legs, fingers and toes; protruding or sunken chestbone; armspan greater than height; scolosis; nearsightedness; and flat feet. Every year many people with the Marfan syndrome and previously unrecognized aortic aneurysms require emergency surgery for acute dissection or sustain fatal rupture before surgery can be performed. Not infrequently such patients had skeletal or ocular changes diagnostic of the Marfan syndrome that were not recognized by the patients' physicians(1).

"Ideally, people who are affected by the Marfan syndrome have their aorta monitored yearly to detect aortic enlargement, the precursor to aortic dissection, and take medications to slow the progression of enlargement. The problem is that tens of thousands of people in the U.S. who have the Marfan syndrome are not diagnosed. For them, an aortic dissection can happen at any time, without notice," said Richard Devereux, M.D., Professor of Medicine, Weill Cornell Medical College-New York Presbyterian Hospital. "It is incumbent upon the emergency physicians to consider aortic dissection in people who present in the emergency room with chest pain, especially if they exhibit the typical physical signs of the Marfan syndrome and other diagnostic possibilities cannot be confirmed."

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.

In conjunction with the "ER" episode, the Foundation is launching a new 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(2). 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 very tall patients who come to the hospital complaining of chest pains.

The Marfan Syndrome
The Marfan syndrome and related connective tissue disorders affect approximately 200,000 people in the U.S. The Marfan syndrome -- which is named for the French pediatrician, Dr. Antoine Marfan, who first identified the disorder in 1896 -- is inherited from an affected parent in 75 percent of cases. However, one-quarter of cases result from a spontaneous mutation at conception. Although the gene for the disorder has been found, there is no simple genetic test to make the diagnosis. A complete examination, including an echocardiogram of the heart by a cardiologist, measurement of body proportions by a geneticist or orthopedist and a slit lamp eye exam by an ophthalmologist, remains the most effective and reliable method for diagnosis.

Once the Marfan syndrome is confirmed, medication can lessen the strain on the aorta and proper monitoring can ensure that surgery is done early to prevent a fatal aortic rupture. With an early diagnosis, proper treatment and a modified lifestyle, most people with the Marfan syndrome can hope to live a normal life span.

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.