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Introduction The primary cardiac tumor (PCT) is a rare disease in
children, with a frequency between 0.03-0.32 %. Eighty percent are benign tumors. Usually they are
asymptomatic and discovered fortuitously.
Conclusion Children's cardiac tumors are usually benign.
The severity is related to functional consequences to which depends the support. Surgery is the best
treatment with an excellent prognosis.
Discussion
•Fetal primary cardiac tumors (FPCT) are uncommon and discovered by widely use of prenatal echocardiography[1]. • Benign tumors are the most frequent[2,3] in children as well as in fetuses. • cardiac rhabdomyomas, teratomas, and fibromas remained the three most common types.[4]
Rhabdomyoma is the most common PCT in fetal life and childhood. It involve the left and right ventricles and ventricular septum. They often grow into the intracavity of the cardiac chambers this is the case of patients 1 and 2. They are associated with tuberous sclerosis in up to 50% of cases [6,7] wich may be the case of youssef who showed convulsions type bending spasms. Usually PCT are asymptomatic and discovered fortuitously but they may cause intracardiac flow obstruction, heart valve insufficiency, arrhythmia [8], heart failure, and hydrops fetalis, or even sudden fetal death[4]. This joins our results since 2/4 patients are asymptomatic. Cardiac ultrasound is paramount in the prenatal diagnosis but there is misdiagnosis. Recalling the echogenic foci in the ventricles, particularly those in the papillary muscles, can be hypertrophic and may sometimes mimic rhabdomyomas, according to Yuan S-M.So ,cardiac MRI is an important complementary modality for characterization of the mass and effect on cardiac function [9]. Histological study of the tumor (biopsy or surgical specimen) remains the best way to confirm the Diagnosis[3]. Therapeutic management of PCT depends on its location, size,number, and complications. Surgery is the solution for tumors resonating on the haemodynamic state. A clinical follow-up may be prescribed in case of rhabdomyoma which may regress spontaneously [11]. Everolimus an mTOR inhibitors which proved its effectiveness for the treatment of different clinical manifestations of tuberosis sclerosis remains the therapeutic future in case of rabdomyoma[12].
Cardiac tumors in newborn: five case report Ayari F ,Bensmail T. , Boussetta A , Ksibi I ,Cheour M , Ben Amara M , Kacem S
Neonatology Care Unit Center of Tunis, Tunisia
Objectives To recall the clinical and sonographic features of
primitive and benign cardiac tumors in children To cite complications through observations of 5
newborns.
Observations Here is a table summarizing the clinical and ultrasound
characteristics of our patients:
Patient medical background
Prenatal ultrsound
Postnatal Ultrsound
resounding others
Leith (1)
-At term -by vaginal
route -eutrophic
-good Apgar
CT evoking a left
intraventricular
rhabdomyoma
In the aorta
which partially obstructed
Asymptomatic
Taher (2)
-At term -by vaginal
route -eutrophic
-good Apgar
Biventricular
Rhabdomyoma (Figure 1)
Multiple biventricular whose largest
was opposite to the large mitral
valve
Supraventricular tachycardia quick to 300
bpm (Figure 4)
Youssef (3)
-At term -by vaginal
route -eutrophic
-good Apgar
right
ventricular fibroma
(Figure 2)
In the
trabecular room of the
right ventricle
Asymptomatic
Bending spasms
evoking a tuberous sclerosis
Khaled (4)
-At term -by vaginal
route -eutrophic
-good Apgar
two hyperechoic spots 2 mm
each depends on the valvular
pillars in the left ventricle
Nothing
Eya (5)
-caesarean section
-intrauterine growth
retardation
tumor taking all the right
ventricle
Aspect of trabeculation
Death by refractory hypoxemia (Autopsy: figure 3)
total agenesis
of the corpus
callosum.
Figure 1:Three-dimensional echocardiograms using a matrix array
transducer: Large right and left ventricular rhabdomyomas
Figure 2 :Spin-echo MRI from a child with a large right ventricular fibroma
Figure 3: Microscopic features of the Tumor in the autopsy: spider cells
Figure 4: Supra Ventricular tachycardia in electrocardiogram
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2. Uzun Orhan, Wilson DG, Vujanic GM, Parsons JM, Giovanni JV. Cardiac tumours in children. Orph J of Rare Diseases 2007;2 (11):1-14. 3. Castro FJ, Escudero. Tumores cardíacos. Protocolos diagnósticos y terapêuticos en Cardiología Pediátrica. Available at:http://www.aepe.es/.
5. Carrilho MC, Tonni G, Araujo Ju´nior E. Fetal cardiac tumors: prenatal diagnosis and outcomes. Rev Bras Cir Cardiovasc 2015;30:VIeVII. 4. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004;25:252e73.
6. Yu K, Liu Y, Wang H, Hu S, Long C. Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact CardioVasc Thorac Surg 2007;6:636-9. 7. Cruz ER, Maldonado RMC. Cardiac Tumors. Available at: http://www.emedicine.com/.
8. Ka Myers, KK Wong, M tipple, S sanatani. Benign cardiac tumours, malignant arrhythmias. Can J Cardiol 2010;26(2):e58-e61. 9. Ghadmi Mahani M, Lu JC, Rigsby CK, Krishnamurthy R, Dorfman AL, Agarwal PP. MRI of pediatric cardiac masses. AJR Am J Roentgenol. 2014 May;202(5):971-81. doi:
10.2214/AJR.13.10680. 10.Yuan S-M, Fetal Primary Cardiac Tumors During Perinatal Period, Pediatrics and Neonatology (2016), http://dx.doi.org/10.1016/j.pedneo.2016.07.004.
11. Liu X, Hong H, Zhang H, Xu Z, Liu J, Qiu L. Treatment Strategies for Primary Tumors of the Heart in Children: A 10-Year Experience. Ann thorac surg. 2015 Nov;100(5):1744-9. doi: 10.1016/j.athoracsur.2015.06.030. Epub 2015 Sep 11.
12. Mohamed I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-205138.