A Huge Aortic Aneurysm in a boy with Marfan Syndrome-Juniper Publishers
JUNIPER PUBLISHERS-OPEN ACCESS JOURNAL OF CARDIOLOGY &
CARDIOVASCULAR THERAPY
Abstract
One of the most serious complications in Marfan’s
syndrome is dilation of aortic root and dissection of aorta and
regularly physical examination of these patients is necessary.
Dilatation of the ascending aorta is a rare finding in children with
this syndrome. We present a 9.5-year-old boy; 22 kg weight was referred
to our center because of systolic murmur during the routine examination
for pre-operation of inguinal hernia. Transthoracic echocardiography and
CT angiography showed a huge ascending aortic aneurysm. The patient
underwent composite graft replacement of the aortic root and ascending
aorta.
Keywords: Marfan Syndrome; Aortic Aneurysm
Abbreviations: CT: Computed Tomography; MFS: Marfan Syndrome
Introduction
In patients with Marfan’s syndrome one of the most
serious complications is dilation of aortic root and dissection of
aorta. In these patients regularly physical examination and estimation
and comparison of aortic root to previous examination is necessary for
prevention of this complication.
In childhood dilatation of the ascending aorta is
rare finding. It is usually associated with predisposing disease such as
Marfan’s syndrome (MFS). MFS is a genetic connective tissue disorder
that its incidence is about 2-3 per 10000 individuals [1]. This disorder
is a multisystem disorder involving many systems such as the
cardiovascular and skeletal systems [2]. Aortic aneurysm and dissection
of aorta remain the most life-threatening manifestations of patients
with MFS. This finding is mostly age dependent, prompting life-long
monitoring by echocardiography or other imaging modalities such as
computed tomography angiography. Some unequivocally patients never reach
an aortic size that needs surgical approach. Normal aortic diameter
varies with both age and body size. The two most important risk factors
that determinants dissection of the aorta is the family history of
dissection and maximal dimension of aorta. When greatest diameter of
aorta reaches about 50 mm, surgical repair is recommended for prevention
of dissection. In childhood there are no definitive describe to guide
the timing of surgery. Early surgery is often undertaken given the
emergence of significant aortic regurgitation or a rapid rate of growth
(>1 cm in a year).
Case Report
A 9.5-year-old boy, 22 kg weight was referred to our center
because of systolic murmur grade II/VI and mild chest deformity
during the routine examination for pre-operation of inguinal
hernia. He had some of physical features of the MFS including
myopia >3 diopters, facial features, increased arm/height, hind
foot deformity, deformity of the thoracic cage and inguinal
hernia. Physical examination revealed chest deformity and
a systolic murmur grade I-II/VI at the aortic area. Chest X-ray
showed significant widened upper mediastinum (Figure 1).
Transthoracic echocardiography showed a huge ascending
aortic aneurysm, that extending from sinotubular junction to
aortic arch (70*70* 110 mm) and mild aortic regurgitation.
CT angiography showed huge oval shape ascending aorta
aneurysm (75*75*116mm) without dissection of aorta and
normal aortic arch size. The patient underwent composite graft
replacement of the aortic root and ascending aorta, without
replacement of aortic valve or reimplantation of the coronary
arteries into the graft.
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