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Giant left ventricular rhabdomyoma treated successfully with everolimus: case report and review of literature

Published online by Cambridge University Press:  15 May 2018

Alfonso Martínez-García ,
Carolina Michel-Macías Open the ORCID record for Carolina Michel-Macías [Opens in a new window] ,
Guadalupe Cordero-González ,
Karla I. Escamilla-Sánchez ,
Mónica Aguinaga-Ríos ,
Alejandra Coronado-Zarco  and
Jorge A. Cardona-Pérez
Alfonso Martínez-García*
Affiliation:
Fetal and Pediatric Cardiology, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Carolina Michel-Macías
Affiliation:
Neonatal Intensive Care Unit, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Guadalupe Cordero-González
Affiliation:
Neonatal Intensive Care Unit, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Karla I. Escamilla-Sánchez
Affiliation:
Neonatal Intensive Care Unit, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Mónica Aguinaga-Ríos
Affiliation:
Perinatal Genetics, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Alejandra Coronado-Zarco
Affiliation:
Neonatal Intensive Care Unit, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
Jorge A. Cardona-Pérez
Affiliation:
Neonatal Intensive Care Unit, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Mexico City, Mexico
*
Author for correspondence: A. Martínez-García, MD, Fetal and Pediatric Cardiology, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, Calle Montes Urales 800, Lomas – Virreyes, Lomas de Chapultepec IV Secc, 11000 Ciudad de México, México. Tel: +52 1 55 1340 6716; Fax: 55400942; E-mail: alfonso.martinez@inper.gob.mx
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Abstract

Introduction

Intracardiac rhabdomyomas can cause severe ventricular dysfunction and outflow tract obstruction.

Case report

A term newborn infant with antenatal diagnosis of giant left ventricle rhabdomyoma presented with cardiac failure and duct-dependent systemic circulation after birth. She was treated successfully with everolimus, showing decrease in tumour size and improvement in left ventricular ejection fraction.

Discussion

Tumour regression rate was 0.32 cm2/day and improved to 0.80 cm2/day with the use of everolimus. Herein we report a newborn with inoperable giant left ventricular cardiac rhabdomyoma and significant regression of the tumour. To our knowledge, this is the largest left ventricular rhabdomyoma reported. A review of the literature was undertaken for comparison.

Conclusion

Everolimus has proven to be efficacious in size reduction of cardiac rhabdomyomas in cases when surgical resection is not possible.

Keywords

Rhabdomyomaeverolimusnewbornsirolimus
Type
Review Articles
Information
Cardiology in the Young , Volume 28 , Issue 7 , July 2018 , pp. 903 - 909
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Copyright
© Cambridge University Press 2018 

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