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
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
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
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
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.
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
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
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.
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
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
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.
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
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.
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
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
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.
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
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
Write to Kevin Helliker at firstname.lastname@example.org
and Thomas M. Burton at email@example.com
< 美國企業新聞社 > [l] 2001-10-05
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
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
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
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.