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Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 339-340
Cardiac rhabdomyoma - antenatal diagnosis


Dept of Radio diagnosis, Goa Medical College, Goa 403202, India

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Keywords: Cardiac Rhabdomyoma, Antenatal diagnosis

How to cite this article:
Dalvi R, Vernekar J, Godinho S, Kartha R. Cardiac rhabdomyoma - antenatal diagnosis. Indian J Radiol Imaging 2002;12:339-40

How to cite this URL:
Dalvi R, Vernekar J, Godinho S, Kartha R. Cardiac rhabdomyoma - antenatal diagnosis. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Jun 5];12:339-40. Available from: http://www.ijri.org/text.asp?2002/12/3/339/28475

   Introduction Top


Primary congenital cardiac tumors are rare. Rhabdomyoma is the common benign tumor of which 50-78% are associated with tuberous sclerosis. We would like to report a case of cardiac rhabdomyoma diagnosed antenatally. The outcome was unfavourable. At autopsy the diagnosis of cardiac rhabdomyoma was confirmed.


   Case Report Top


A 24 year old primigravida was referred for a routine antenatal ultrasound at 30 weeks of gestation. The fetus biometry corresponded to the period of gestation. Sonographic evaluation of the fetal thorax revealed cardiomegaly with two well defined echogenic masses measuring 10 and 8mm each, arising from the interventricular septum and the lateral wall of the left ventricle. There was no pericardial effusion. The fetal heart rate was 140 beats per minute, fetal echocardiography did not reveal any cardiac dysrhythmias. In addition the fetus had bilateral pleural effusions and ascitics suggestive of hydropic changes. No other abnormality was detected in the fetus on ultrasonography. The sonographic features were consistent with diagnosis of cardiac rhabdomyoma

There was no history or clinical signs of tuberous sclerosis in the mother or other members of the family. The patient was counseled regarding the outcome of pregnancy. A preterm stillborn baby was delivered by vaginal delivery at 32 weeks gestation A postmortem biopsy of the mass confirmed the diagnosis of rhabdomyoma. No other abnormality was detected in the baby


   Discussion Top


The incidence of cardiac tumors is 1 to 2 cases per 10,000 live births.1 90% of these are benign[3] out of which rhabdomyomas are the most common [58%]. Other rare cardiac tumors are teratoma [19%], myxomas, hemangiomas and mesotheliomas[2]. Rhabdomyomas also known as myocardial hamartoma are benign smooth muscle tumors of the myocardium consisting of immature myocytes. Histopathology reveals the typical spider cell. They occur with equal frequency in the left and right ventricle and the interventricular septum[4] almost always these tumours are multiple. In approximately half of affected patients at least one of the tumours is intracavitary and obstructive. Patients may present with cardiac dysrhythmias and non immune hydrops as in the present case. There is a strong association between cardiac rhabdomyoma and tuberous sclerosis [50-86%][5]

The prognosis depends on the site, size and number of tumours. The presence of cardiac dysrhythmias or non immune hydrops indicates a poor prognosis. Various studies have shown that rhabdomyomas are slow growing tumours in utero and show no or minimal postnatal growth. Some tumours are known to show regression after birth6

Multiple postnatal management options exist. Serial postnatal ultrasound studies are suggested to look for signs of congestive cardiac failure or cardiac dysrhythmias. If asymptomatic, a conservative management is advised. When cardiac outflow obstruction, persistent arrhythmias, cardiac failure or cardiogenic emboli are present surgical resection may be considered. Family screening for tuberous sclerosis is suggested.[6]

 
   References Top

1.Harding W, Pagon RA. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am j med genet 1992 march; 42 [5] 754-5  Back to cited text no. 1    
2.Smaltz A, Apitz J. primary heart tumours in infancy and childhood report of 4 cases and review of literature. Cardiology 67; 12-6 1981.  Back to cited text no. 2    
3.Mcallister H Jr. primary tumours of the heart and pericardium. Pathol annul 14; 325-30 1979  Back to cited text no. 3    
4.Bosi G, Linterman JP, Pellegrino PA. Acta pediatric 1996 aug 85 [8] 928-31. the natural history of cardiac rhabdomyoma with and without tuberous sclerosis  Back to cited text no. 4    
5.Bass JL, Breningstall GN, Swarman KF, Echocardiographic incidence of cardiac rhabdomyoma in tuberous sclerosis. Am J cardiology 55;1379-82 1985  Back to cited text no. 5    
6.Alkalay A, ferry DA, Lin B, Fink B. Spontaneous regression of cardiac tumor rhabdomyoma in tuberous sclerosis. Clinical pediatric 26, 532-5 1987.  Back to cited text no. 6    

Top
Correspondence Address:
R Dalvi
C/o Sharad Dalvi, Mahadev Prasad, Shantinagar, Ponda Goa 403401
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2]

This article has been cited by
1 Tuberous sclerosis in an antenatally diagnosed cardiac rhabdomyoma
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[Pubmed]



 

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    Introduction
    Case Report
    Discussion
    References
    Article Figures

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