ACA App
Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia
Home | About us | Editorial Board | Search | Ahead of print | Current Issue | Archives | Submission | Subscribe | Advertise | Contact | Login 
Users online: 604 Small font size Default font size Increase font size Print this article Email this article Bookmark this page
 


 

 
     
    Advanced search
 

 
 
     
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed2561    
    Printed117    
    Emailed0    
    PDF Downloaded328    
    Comments [Add]    
    Cited by others 1    

Recommend this journal

 


 
INTERESTING IMAGES Table of Contents   
Year : 2009  |  Volume : 12  |  Issue : 1  |  Page : 81-82
Large rhabdomyosarcoma of the right ventricle obstructing tricuspid valve, pulmonary valve and left ventricular outflow tract


Department of Cardiac Anaesthesia, Cardio Thoracic Sciences Centre, All India Institute of Medical Sciences, New Delhi - 110 029, India

Click here for correspondence address and email
 

How to cite this article:
Selvaraj T, Kapoor PM, Kiran U. Large rhabdomyosarcoma of the right ventricle obstructing tricuspid valve, pulmonary valve and left ventricular outflow tract. Ann Card Anaesth 2009;12:81-2

How to cite this URL:
Selvaraj T, Kapoor PM, Kiran U. Large rhabdomyosarcoma of the right ventricle obstructing tricuspid valve, pulmonary valve and left ventricular outflow tract. Ann Card Anaesth [serial online] 2009 [cited 2019 Aug 22];12:81-2. Available from: http://www.annals.in/text.asp?2009/12/1/81/45020


A 7-year-old female child presented to our hospital with fever, weight loss, and progressive increase in facial puffiness, paedal swelling, dyspnoea and abdominal pain for 3 months. On examination, she appeared pale, the jugular venous pressure was elevated and liver was palpable 5 cm below the right costal margin. There was an ejection systolic murmur at the left 2nd and 3rd intercostal area. Transthoracic echocardiography showed a large mass occupying the entire right ventricle (RV) causing severe obstruction to the inflow and outflow of RV. The attending cardiologist suggested an emergency intervention in view of the emergent clinical condition of the patient. The patient was immediately taken for emergency surgery. Under general anaesthesia, the surgery was performed. Transoesophageal echocardiography (TOE) showed a homogenous mass filling the whole of right ventricle, which extended from the margin of the tricuspid valve to the infundibulum of the right ventricular outflow tract [Figure 1]. Under cardiopulmonary bypass, the intra-cardiac mass was resected. RV and pericardium were filled with cold saline in order to provide echocardiographic window, and TOE was carried out. Complete resection of the tumour was carried out with such an echocardiographic guidance. The diagnosis of rhabdomyosarcoma was made by frozen section biopsy.

Primary tumours of the heart are extremely rare with a prevalence of 0.0017% to 0.28%.in post-mortem studies. [1] Rhabdomyosarcomas are the most frequently encountered cardiac tumour in children and the second most frequant primary Sarcoma of the heart in all age groups. [1] Rhabdomyosarcoma usually involves the ventricular myocardium and project into the cavity. As in the author's case, it may present with cardiac obstructive phenomenon [1] [Figure 2],[Figure 3] or by arrhythmias, pericardial effusion, ventricular pre-excitation and even sudden death. Unlike the author's case, rhabdomyosarcoma is commonly associated with tuberous sclerosis.[2] Tumour debulking [Figure 4],[Figure 5] may provide relief from symptoms when the haemodynamic performance is affected. [3] Cardiac transplantation has been utilised to completely remove inoperable cardiac tumours. [4],[5] TOE is a useful guide to check for adequacy of surgical resection.

 
   References Top

1.McAllister HA, Fenoglio JJ, Tumours of the cardiovascular system. In: Atlas of tumour pathology, 2 nd series, fascicle 15. Washington DC: Armed Forces Institute of Pathology; 1978.  Back to cited text no. 1    
2.Colucci WS, Schoen FJ. Primary tumours of the heart. In: Braunwald E, Zipes DP, Libby P, editors. Heart disease. 6th ed. Philadelphia: WB Saunders; 2001. p. 1807-19.  Back to cited text no. 2    
3.Martin G, Sutton J, Maniet AR. Atlas of multiplane transesophageal echocardiography. 1 st ed. London: EC4P 4EE, Taylor and Francis group plc; 2003. p. 675.  Back to cited text no. 3    
4.Demkow M, Sorensen K, Whitehead BF, Rees PG, Sullivan ID, Elliott MJ, et al . Heart transplantation in an infant with rhabdomyoma. Pediatr Cardiol 1995;16:204-6.  Back to cited text no. 4    
5.Reardon MJ, DeFelice CA, Sheinbaum R, Baldwin JC. Cardiac autotransplant for surgical treatment of a malignant neoplasm. Ann Thorac Surg 1999;67:1793-5.  Back to cited text no. 5    

Top
Correspondence Address:
Poonam M Kapoor
Department of Cardiac Anaesthesia, 7th floor, Cardiothoracic Sciences centre, All India Institute of Medical Sciences, New Delhi - 110 029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.45020

Clinical trial registration None

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

This article has been cited by
1 Large malignant cardiac tumor in a 7-year-old child
Lazea, C. and Manasia, R. and Oprita, S. and Denes, C.L. and Manole, S.
Paediatria Croatica. 2012; 56(1): 59-61
[Pubmed]



 

Top