Rapid Regression of Left Ventricular Outflow Tract Rhabdomyoma After Sirolimus Therapy
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چکیده
The neonatal presentation of cardiac rhabdomyomas varies in severity from severe outflow tract obstruction to minimal cardiac dysfunction. The natural history for these lesions is spontaneous regression in the majority of cases. We describe a newborn boy with severe left ventricular outflow tract obstruction secondary to a large rhabdomyoma. The tumor infiltrated the paraaortic area and extended around the origin of the right coronary artery, making surgical resection challenging. Oral sirolimus therapy resulted in a rapid regression of the tumor and alleviation of outflow tract obstruction within 1 month of treatment. This is the first report of sirolimus therapy in alleviating critical left ventricular outflow tract obstruction in this condition. Pediatrics 2014;134: e1199–e1202 AUTHORS: Colm Breathnach, MB, BCh, MRCPI,a Jane Pears, FRCPCH,b Orla Franklin, MB, FRCPI,a David Webb, FRCPI,c and Colin J. McMahon, MB, FAAP, FRCPIa Departments of aPaediatric Cardiology, bOncology, and cNeurology, Our Lady’s Children’s Hospital, Crumlin, Dublin, Ireland
منابع مشابه
Rapid regression of left ventricular outflow tract rhabdomyoma after sirolimus therapy.
The neonatal presentation of cardiac rhabdomyomas varies in severity from severe outflow tract obstruction to minimal cardiac dysfunction. The natural history for these lesions is spontaneous regression in the majority of cases. We describe a newborn boy with severe left ventricular outflow tract obstruction secondary to a large rhabdomyoma. The tumor infiltrated the paraaortic area and extende...
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Cardiac rhabdomyoma often shows spontaneous regression and usually requires only close follow-up. However, patients with symptomatic inoperable rhabdomyomas may be candidates for everolimus treatment. Our patient had multiple inoperable cardiac rhabdomyomas causing serious left ventricle outflow-tract obstruction that showed a dramatic reduction in the size after everolimus therapy, a mammalian...
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Rhabdomyomata are probably the most common tumors that occur very rarely during infancy. In this paper, we report the case of a 49-day-old infant who was diagnosed by echocardiography examination with left ventricle outflow tract (LVOT) obstruction caused by rhabdomyoma. The infant underwent surgical approach, and her mass was shaved. Finally, she was discharged from hospital in good gener...
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