Rapid Regression of Obstructive Cardiac Rhabdomyoma in a Preterm Neonate after Sirolimus Therapy
نویسندگان
چکیده
Cardiac rhabdomyoma can be subclinical or fatal depending on the onset age, involving site, and the size and degree of invasion. Although most rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction induces hemodynamic instability. Mammalian target of rapamycin (mTOR) inhibitors have been used to treat neonates and children with hemodynamically obstructive cardiac rhabdomyoma. Herein, we report a premature neonate at the gestational age of 30 + 4 weeks with severe left ventricular outflow tract obstructive cardiac rhabdomyoma who was successfully treated with the mTOR inhibitor sirolimus. To the best of our knowledge, this is the first recorded case of a premature neonate with obstructive cardiac rhabdomyoma who was successfully treated with an mTOR inhibitor. Therefore, sirolimus could be considered as an alternative medical option for managing premature neonates with obstructive cardiac rhabdomyoma. © 2017 The Author(s) Published by S. Karger AG, Basel
منابع مشابه
Regression of Cardiac Rhabdomyomas in a Neonate after Everolimus Treatment
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...
متن کاملRapid 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...
متن کاملRapid 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...
متن کاملConservative Management in a Ventilated Preterm Neonate with Pneumopericardium, Pleural Effusion, and Pulmonary Collapse: A Case Report
Background: Neonatal pneumopericardium (PPC) is a rare clinical condition usually associated with other air leak syndromes. It increases morbidity and mortality due to cardiac tamponade. Case report: A preterm male neonate weighing 1260 g was born with the gestational age of 28 weeks. Cardiopulmonary resuscitation was routinely performed without any medical therapy. The newborn was transferred ...
متن کاملSpontaneous Regression of Cardiac Rhabdomyoma Presenting as Severe Left Ventricular Inlet Obstruction in a Neonate with Tuberous Sclerosis
Cardiac rhabdomyoma can be subclinical or have a fatal presentation according to the onset age and involved site, size, and degree of invasion. Although most cardiac rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction has occurred. In this report, we describe the spontaneous regression of a large cardiac rhabdomyoma (20.5 × 15.6 mm) presenting as s...
متن کامل