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A Huge Aortic Aneurysm in a boy with Marfan Syndrome
Ghaderian Mehdi* and Ghafri Ghafar
Department of pediatric cardiology, Esfahan University of Medical Sciences, Iran
Submission: October 08, 2016; Published: October 21, 2016
*Corresponding author: Ghaderian Mehdi, Assistant professor of pediatric cardiology, Department of pediatric cardiology, Emam Hosein Medical, Educational and Research Center, Esfahan University of Medical Sciences, Emam Khomeini St., P.O.Box 8195100000, Esfahan, Iran.
How to cite this article: Ghaderian M and Ghafri G. A Huge Aortic Aneurysm in a boy with Marfan Syndrome. J Cardiol & Cardiovasc Ther. 2016; 1(4): 555569. DOI: 10.19080/JOCCT.2016.01.555569
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.
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 . This disorder is a multisystem disorder involving many systems such as the cardiovascular and skeletal systems . 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).
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.