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HUGE SPORADIC AND COMPLEX CARDIAC RHABDOMYOMA WITH HYPOPLASTIC LEFT VENTRICLE AND LEFT LUNG
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  • Türkan Tansel,
  • İbrahim Demir,
  • Nilgün Yazıksız,
  • Ümrah Aydoğan,
  • İbrahim Kalelioğlu,
  • Recep Has,
  • Emine Yılmazbayhan
Türkan Tansel
Istanbul University Istanbul Faculty of Medicine
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İbrahim Demir
Istanbul University
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Nilgün Yazıksız
Istanbul University Istanbul Faculty of Medicine
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Ümrah Aydoğan
Istanbul University Istanbul Faculty of Medicine
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İbrahim Kalelioğlu
Istanbul University Istanbul Faculty of Medicine
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Recep Has
Istanbul University Istanbul Faculty of Medicine
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Emine Yılmazbayhan
Istanbul University Istanbul Faculty of Medicine
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Abstract

Cardiac rhabdomyomas are the most common fetal cardiac tumors and can be diagnosed antenatally and postnatally by echocardiography. They are usually asymptomatic and spontaneously regress within the first three years of life and often associated with tuberous sclerosis (TS). The prognosis of patients with rhabdomyomas is mostly determined by the size and location of the lesion. Rhabdomyomas may cause hemodynamic disturbances or arrhythmias due to obstructing the inflow or ventricular outflow tracts or alter valve function, leading to poor prognosis. Although it may regress spontaneously, surgical excision is usually curative, especially in sporadic and single tumors. We report an unique case of antenatally diagnosed as a huge cardiac rhabdomyoma accompanied by hypoplastic left lung and left ventricle, mimicking significant obstruction of the left ventricular and left atrial chamber. The patient underwent urgent surgery, because of severe hemodynamic compromise on the first day of life due to signs of severe obstruction of the left ventricle and left atrium. The large, lobulated epicardial cardiac rhabdomyoma located anterolateral and inferior aspect of the heart; involved the whole left ventricle through the left atrium excised.