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


Thiruvenkadam Selvaraj, Poonam M Kapoor, Usha Kiran 
 Department of Cardiac Anaesthesia, Cardio Thoracic Sciences Centre, All India Institute of Medical Sciences, New Delhi - 110 029, India

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




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;12:81-82


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 Dec 14 ];12:81-82
Available from: http://www.annals.in/text.asp?2009/12/1/81/45020


Full Text

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

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