primary cardiac tumors are quite rare , especially in the pediatric age group,13 and their atypical presentations often prevent a timely diagnosis.4 most primary cardiac tumors in the pediatric age group are benign ; autopsy studies in children have reported incidence rates ranging from 0.027% to 0.08%.4 one echocardiography database has reported an incidence of 0.17% , which suggests that one or two new primary cardiac tumors will be detected for every 1000 first - time pediatric echocardiograms.4 fibromas are generally reported as the second most common primary cardiac tumor in the pediatric age group.3 , 5 however , in a more recent review of boston children s hospital database from 1980 to 2005 , fibromas were the third most common tumor . to date , no distinct genetic inheritance or familial predisposition has been associated with cardiac fibromas .
these neoplasms are often intramural and involve the left ventricular free wall or the interventricular septum .
less frequently , they can be multiple and invade the right ventricular free wall , the atrial septum , or its free wall .
although rare , intracavitary fibromas have also been reported.4 , 6 it is very rare for primary intracardiac tumors to occur in the supravalvular pulmonic or aortic positions.7 a case of a seven - year - old boy with an intraluminal pulmonary artery fibroma was reported in the pediatric cardiology journal ( 21 : 480482 2000 ) from southwest texas methodist hospital .
we herein present the case of a girl with an intraluminal fibroma in her ascending aorta .
we present a 23-month - old girl admitted for the evaluation of a cardiac murmur with a history of one episode of syncope two months before admission . she had no positive history for syncope and no positive family history for congenital heart diseases or any kind of cardiac tumors . on physical examination ,
her growth and development were within normal limits ( height = 85 cm , weight = 11 kg ) .
, there was a grade 3/6 systolic ejection murmur at the second right inter - costal space with radiation to the neck .
transthoracic echocardiography demonstrated mild left ventricular hypertrophy and a large intraluminal mass in the ascending aorta , but its borders were not obvious .
doppler interrogation of the ascending aorta showed a turbulent , high - velocity ante grade flow with a 66 mmhg peak pressure gradient and a 46 mmhg mean pressure gradient . by color mapping ,
turbulency began at the level of the aortic valve . in order to confirm the transthoracic echocardiography findings
, transesophageal echocardiography was performed , which confirmed the presence of a large intraluminal mass in the ascending aorta .
the mass was elongated and occupied about 60% of the ascending aorta s area ( 3 cm  1 cm  1 cm ) .
the turbulent antegrade flow around the margins of the mass was obvious and there was no more adherence .
the distal end of the mass juxtaposed the initiation of the transverse aorta ( figure 1 ) .
the high likelihood of the mass being tumoral and its hazardous location precluded catheterization , and the child underwent surgery for mass resection .
given the position of the mass , cannulation via the femoral artery , inferior vena cava , and superior vena cava was done . with cerebral protection during cardiopulmonary bypass , the arteriotomy of the ascending aorta was preformed .
the mass was found to be entirely within the ascending aorta with firm adherence to the anterior leaflet of the bicuspid aortic valve ( figure 2 ) .
the tumor was excised subtotaly because it could not be detached from the aortic valve leaflet .
the arteriotomy was closed , and the child was weaned from the cardiopulmonary bypass machine without any difficulty .
repeat transthoracic echocardiograms following the operation did not reveal any evidence of residual supravalvular aortic stenosis and there was no aortic insufficiency . in gross pathology description ,
the mass was solid , firm , and creamy with a nodular appearance ( 3  1  1 cm ) .
microscopic sections showed a benign neoplasm composed of fascicles of fibroblasts with variable amounts of collagen and a scanty number of lymphocytes arranged in a focally myxomatous stroma without any evidence of malignancy such as increased mitotic figures or areas of necrosis ( figures 3a & 3b ) .
the most common tumors in newborns and infants are rhabdomyomas , fibromas , and intrapericardial teratomas ; whereas in older children and adolescents , myxomas , rhabdomyomas , and fibromas are prominent .
rhabdomyomas constitute 45% to 80% of all primary cardiac tumors in the pediatric age group .
these tumors can be diagnosed in the prenatal period but are most frequently diagnosed in the newborn infant . although fibromas have recently been reported in utero and in patients younger than 1 month of age , they are found much less commonly than rhabdomyomas in this age group . in a recent review at boston children
s hospital , fibromas were the second most common tumors ( 17% ) in patients diagnosed between 1 month and 1 year of age .
these primary tumors are rarely seen in older children , adolescents , or young adults.4 no known sex predilection is recognized , although the rarity of different benign cardiac tumors prevents an accurate determination of a male - to - female ratio.6 on two - dimensional echocardiography , cardiac fibromas are seen as a single , bright , intramural , echogenic mass .
ct scanning is often performed and might provide clues regarding tissue characterization , with central calcification suggestive of a cardiac fibroma.6 this case has two interesting aspects : presenting symptom of the tumor and its location .
our patient was previously healthy with a normal development , and she sought medical attention after a syncopal event and a murmur was noted on the auscultation of the heart .
fibromas are predominantly intramural tumors , and extensive intramural fibromas can encroach and obliterate the intracavitary space .
a similar case has been previously reported with an intraluminal fibroma in the proximal main pulmonary artery.8 although benign , fibromas may become life - threatening by causing arrhythmias or obstruction to blood flow.8 , 9 acquired supravalvular aortic stenosis and syncope due to the tumor were the presenting features of our patient .
although complete resection is preferable , our patient s tumor was not amenable to complete resection due to its firm adherence to the anterior cusp of her bicuspid aortic valve .
syncope can be the presenting symptom of the tumors of the intraluminal ascending aorta as was the case in the patient described here with an unusual location of fibroma .
transesophageal echocardiography is useful for the evaluation of anatomic details and can be of assistance for surgical planning .
 primary cardiac tumors are quite rare , especially in the pediatric age group , and their atypical presentations often prevent a timely diagnosis . 
 most primary cardiac tumors in the pediatric age group are benign . 
 fibromas are generally reported as the second most common primary cardiac tumors in the pediatric age group . these neoplasms are often intramural and involve the left ventricular free wall or the interventricular septum . 
 although benign , fibromas may become life - threatening by causing arrhythmias or obstruction to the blood flow . a case of supravalvular intraluminal ascending aorta fibroma in a 23-month - old girl , presenting with syncope , 
 is described here ; the location is rare and the presentation atypical for this type of tumor . 
 transesophageal echocardiography helped us to evaluate the anatomic details of the tumor and plan surgery . 
Introduction
Case Report
Discussion
Conclusion