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CASE REPORT
Year : 2013  |  Volume : 40  |  Issue : 3  |  Page : 183-185

Multiple intra cardiac rhabdomyomas in neonate


1 Department of Pediatric Cardiology, KLES Dr. P. K. Hospital and MRC, Belgaum, Karnataka, India
2 Consultant Pediatric Cardiac Intensivist, KLE Heart Foundation, Dr. P. K. Hospital and Research centre, Belgaum, Karnataka, India
3 Consultant Echocardiography, KLE Heart Foundation, Belgaum, Karnataka, India
4 Department of Surgery, JNMC, Belgaum, Karnataka, India

Date of Web Publication19-Oct-2013

Correspondence Address:
Veeresh F Manvi
Senior Consultant Pediatric Cardiology, Department of Pediatric Cardiology, KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum - 590 010,, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.120064

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  Abstract 

An 18 day old neonate presented with tachypnea. Congenital heart disease was suspected and referred for Pediatric Cardiology opinion. 2D Echocardiography revealed multiple, intra cardiac, highly echogenic, well circumscribed, intramural and intra cavitary rhabdomyomas. There were no systemic manifestations of associated tuberous sclerosis. At present, surgical excision of rhabdomyoma was not indicated and hence child was advised regular follow up with echocardiography evaluation.

Keywords: Multiple intracardiac rhabdomyomas, neonate, tuberous sclerosis


How to cite this article:
Manvi VF, Manvi NG, Shavi N, Pattanshetti VM. Multiple intra cardiac rhabdomyomas in neonate. J Sci Soc 2013;40:183-5

How to cite this URL:
Manvi VF, Manvi NG, Shavi N, Pattanshetti VM. Multiple intra cardiac rhabdomyomas in neonate. J Sci Soc [serial online] 2013 [cited 2019 May 26];40:183-5. Available from: http://www.jscisociety.com/text.asp?2013/40/3/183/120064


  Case Report Top


An 18 days old male baby was born to a primigravida mother by full term normal vaginal delivery. Baby was apparently normal till ten days of life, when he developed hurried breathing. There were no feeding issues or excessive perspiration. Baby was evaluated by a pediatrician who detected a soft systolic murmur and referred to our centre for Pediatric Cardiology opinion. On examination child had normal heart rate of 112/minutes and respiratory rate of 38/minutes. SPO2 was normal. There was no palpable liver and jugular venous pressure was normal. Precordial examination was not unusual. On auscultation heart sounds were normal. There was a grade 3/6 ejection systolic murmur in pulmonary area. 2D echocardiography and Doppler evaluation was done. It showed multiple (four) highly echogenic well circumscribed intra mural and intracavitary nodules. They had homogenous echo bright and finely speckled pattern. First lesion was intracavitary attached to the apex of left ventricle [Figure 1] measuring 8 mm × 4 mm in size. Second lesion was intramural and embedded in the interventricular septum. Third lesion was in right ventricular outflow tract and caused moderate obstruction to the flow of blood [Figure 2] and [Figure 3]. Fourth lesion was in right atrium and was 3 mm × 5 mm in size [Figure 4]. Doppler evaluation showed right ventricular outflow tract peak systolic gradient of 30 mmHg. Colour Doppler showed turbulence in flow of blood. Systemic examination done did not reveal manifestations of tuberous sclerosis such as Shagreen patch or adenoma sebaceum. Since, baby did not have any evidence of congestive cardiac failure and right ventricular outflow tract stenosis was of mild severity, patient was advised regular medical follow up.
Figure 1: Large rhabdomyoma in left ventricle

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Figure 2: Rhabdomyoma in right ventricular outflow tract

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Figure 3: Rhabdomyoma in right ventricular outflow tract as depicted by colour flow mapping

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Figure 4: Rhabdomyoma in right atrium

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  Discussion Top


Rhabdomyomas are the most common benign cardiac neoplasms occurring in the fetus and neonate. [1],[2] Most of them are identified in first year of life. It is considered to be a hamartoma of developing cardiac myocytes with an inability of cells to undergo mitotic division. [3] Though the behavior of cardiac rhabdomyoma is benign, the positioning within critical areas in the heart can lead to lethal arrhythmias and chamber obstruction. They constitute about 45% to 80% of all primary cardiac tumors in the pediatric age group. [4] Studies have demonstrated incidence of cardiac rhabdomyoma as 0.002-0.25% at autopsy, 0.02-0.08% in live born infants and 0.12% in prenatal reviews. [5] Cardiac rhabdomyoma frequently occurs in association with tuberous sclerosis. [6] It is an autosomal dominant disorder characterized by wide spread lesions that involve brain, kidney, heart, skin, and other organs. About 50% of patients with tuberous sclerosis develop a cardiac rhabdomyoma. Similarly about 51-86% cases of cardiac rhabdomyoma have clinical or radiological evidence of tuberous sclerosis. [7] Histologically, rhabdomyoma contains large vacuolated cells filled with glycogen. Typical spider cells are seen with the eccentric nuclei, granular cytoplasm and thin cytoplasmic extensions projecting towards the cell membrance.

The differential diagnosis for rhabdomyoma includes fibroma, myxoma, teratoma and haemangioma. Fibromas do not demonstrate hemorrhage and necrosis. Classification is typically present, a feature that distinguishes them from rhabdomyoma. Myxomas are endocardium based lesions and do not infiltrate into the underlying tissue. Teratomas are extracardiac masses that are located in the pericardial cavity and associated with pericardial effusion. They are composed of both cystic and solid areas. In contrast to rhabdomyoma, haemangiomas have circumscribed echo lucent areas as a result of hemorrhage.

The natural history of cardiac rhabdomyoma comprises of complete or partial regression with consequent resolution of symptoms. The reported survival rates range from 81% to 92%. Tumors more than 20 mm in diameter are more likely to cause hemodynamic disturbance or arrhythmias, which are associated with an increased risk of death. Long term prognosis is affected by neurologic manifestations associated with tuberous sclerosis. [8],[9] Surgical intervention is reserved for patients who have symptoms of severe hemodynamic compromise or intractable arrhythmia.

 
  References Top

1.Becker AE. Primary heart tumors in the pediatric age group: A review of salient pathologic features relevant for clinicians. Pediatr Cardiol 2000;21:317-23.   Back to cited text no. 1
    
2.Elderkin RA, Radford DJ. Primary cardiac tumours in a paediatric population. J Paediatr Child Health 2002;38:173-7.   Back to cited text no. 2
    
3.Holley DG, Martin GR, Brenner JI, Fyfe DA, Huhta JC, Kleinman CS, et al. Diagnosis and management of fetal cardiac tumors: A multicenter experience and review of published reports. J Am Coll Cardiol 1995;26:516-20.   Back to cited text no. 3
    
4.Chan HS, Sonley MJ, Moës CA, Daneman A, Smith CR, Martin DJ. Primary and secondary tumors of childhood involving the heart, pericardium and great vessels: A report of 75 cases and review of the literature. Cancer 1985;56:825-36.   Back to cited text no. 4
    
5.Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004;25:252-73.   Back to cited text no. 5
    
6.Gomez MR. Varieties of expression of tuberous sclerosis. Neurofibromatosis 1988;1:330-8.  Back to cited text no. 6
    
7.Harding CO, Pagon RA. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am J Med Genet 1990;37:443-6.   Back to cited text no. 7
    
8.Yinon Y, Chitayat D, Blaser S, Seed M, Amsalem H, Yoo SJ, et al. Fetal cardiac tumors: A single-center experience of 40 cases. Prenat Diagn 2010;30:941-9.   Back to cited text no. 8
    
9.Degueldre SC, Chockalingam P, Mivelaz Y, Di Bernardo S, Pfammatter JP, Barrea C, et al. Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes. Cardiol Young 2010;20:18-24.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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