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 ob...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2014-10, Vol.134 (4), p.e1199-e1202
Main Authors: Breathnach, Colm, Pears, Jane, Franklin, Orla, Webb, David, McMahon, Colin J
Format: Article
Language:English
Subjects:
Antibiotics, Antineoplastic - therapeutic use
Cardiovascular disease
Heart Neoplasms - diagnostic imaging
Heart Neoplasms - drug therapy
Heart Ventricles - diagnostic imaging
Humans
Infant, Newborn
Male
Newborn babies
Pediatrics
Remission Induction
Rhabdomyoma - diagnostic imaging
Rhabdomyoma - drug therapy
Sirolimus - therapeutic use
Tumors
Ultrasonography
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Summary: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.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2013-3293