Professional Documents
Culture Documents
doi: 10.4183/aeb.2013.623
Abstract
Background. Aortic dissection is
a life-threatening disorder and up to 20%
of patients die before receiving medical
care. Marfan syndrome is noted in 59% of
individuals who suffer from aortic dissection.
Case presentation. We present
the case of a 53 years old woman, with
undiagnosed Marfan syndrome, addressed to
our clinic complaining about thoracolumbar
pain appeared 4 days ago, after a trauma.
According to the revised Ghent criteria for
the diagnostic of Marfan syndrome she had
a positive family history and more than 7
points of systemic findings. She was also
diagnosed with extensive aortic dissection
and right pneumothorax. Because of the
cachexia and important scoliosis, the
operative and post operative risk was high
and we decided a medical management. She
remained haemodynamically stable, with
a false lumen partially trombosed, and was
discharged home after 23 days.
Discussion. The particularity of
our case represent the diagnostic of Marfan
syndrome after the appearance of a vital risk
vascular complication aortic dissection,
the emergency surgical intervention being
limited by the clinical and prognostical
particularities of these two comorbidities.
Conclusion. Aortic dissection in
Introduction
Marfan syndrome, a variable,
autosomal dominant disorder of the
connective tissue caused by mutations in
the extracellular matrix protein fibrillin
1, is noted in 59% of individuals who
suffer from aortic dissection (1). The
definition of the syndrome has evolved
throughout the 20th century, illustrating
the difficulty in diagnosis (2). It is one of
the major inherited disorders that weaken
the aortic wall, lead to higher wall stress,
which can induce aortic dilatation
and aneurysm formation, eventually
resulting in aortic dissection or rupture
(3, 4). Aortic dissection occurs as the
result of a tear within the intimal lining
of the aorta, leading to exposure of the
intima to elevated aortic pressures with
resultant separation of the intima from
*Correspondence to: Larisa Anghel MD, Cardiovascular Diseases Institute, Prof. Dr. George I. M.
Georgescu, 50 Carol I Avenue, Iasi, Romania, E-mail: larisa_med86@yahoo.com.
Acta Endocrinologica (Buc), vol. IX, no. 4, p. 623-632, 2013
623
L. Macovei et al.
Figure 1. Arachnodactyly.
skin striae - 1;
myopia > 3 diopters - 1;
presence of family history of
Marfan syndrome (her sister).
Because of a positive family
history of Marfan syndrome and more
than 7 points of systemic findings, our
patient was diagnosed with Marfan
syndrome.
Electrocardiogram
showed
normal sinus rhythm and her blood results
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L. Macovei et al.
A transthoracic echocardiogram
demonstrated normal left ventricular
function with ejection fraction of
60%, mild aortic, mitral and tricuspid
Figure 4. Two-dimensional parasternal short-axis (A) and long axis (B) echocardiographic view of the
heart with a flap in the ascending aorta.
626
Figure 5. Aortography with intimal flap in abdominal aorta (A) and bifurcation of the both common
iliac arteries (B).
L. Macovei et al.
acute aortic dissection are initially diagnosed as having other conditions (15).
Approximately two thirds of
patients with acute aortic dissection
are male and mean age at presentation
ranges between 62 and 67 years (16).
Among patients less than 40 years of age
with aortic dissection, risk factors such
as Marfan syndrome, previous aortic
aneurysm, and bicuspid aortic valve are
common (17). The presence of Marfan
syndrome has been associated with
adverse outcome among survivors of
type B dissection (18).
This is a rare case of extensive
aortic dissection involving the left
common carotid artery, the ascending,
thoracic, abdominal aorta and bifurcation
of the both common iliac arteries. The
risk of death is high in untreated aortic
dissection. While the risk is very high
in the first 24 hours of the event, those
that survive the initial event still have
an elevated mortality compared to ageand sex-matched controls. It is estimated
that up to 20% of patients with aortic
dissection die before receiving medical
care, and mortality estimates within the
first 48 hours of treatment have been as
high as 1% to 1.4% per hour (19, 20).
This is not surprising considering the
fact that type A of aortic dissection, by
definition, must involve the ascending
aorta and are therefore more prone
to catastrophic complications such
as cardiac tamponade, myocardial
infarction, and aortic regurgitation (21).
Among patients with aortic dissection,
the majority of deaths occur within the
first 7 days of presentation, if not within
the first 48 hours (1).
A few case reports of dissection
628
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