Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis
Most cardiac rhabdomyomas with tuberous sclerosis (TS) are asymptomatic and spontaneously regress. However, some cases require surgical intervention due to arrhythmia and severe obstruction of cardiac inflow or outflow. We report herein a neonatal case of giant cardiac rhabdomyomas with TS and insuf...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Thieme Medical Publishers, Inc.
2019-07-01
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| Series: | American Journal of Perinatology Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1692198 |
| _version_ | 1819206396800925696 |
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| author | Yuka Shibata Hidehiko Maruyama Taiyu Hayashi Hiroshi Ono Yuka Wada Hideshi Fujinaga Shuhei Fujino Junko Nagasawa Shoichiro Amari Keiko Tsukamoto Yushi Ito |
| author_facet | Yuka Shibata Hidehiko Maruyama Taiyu Hayashi Hiroshi Ono Yuka Wada Hideshi Fujinaga Shuhei Fujino Junko Nagasawa Shoichiro Amari Keiko Tsukamoto Yushi Ito |
| author_sort | Yuka Shibata |
| collection | DOAJ |
| description | Most cardiac rhabdomyomas with tuberous sclerosis (TS) are asymptomatic and spontaneously regress. However, some cases require surgical intervention due to arrhythmia and severe obstruction of cardiac inflow or outflow. We report herein a neonatal case of giant cardiac rhabdomyomas with TS and insufficient pulmonary blood flow from the right ventricle. Lipoprostaglandin E1 was necessary to maintain patency of the ductus arteriosus. We used everolimus, a mammalian target of rapamycin inhibitor, to diminish the cardiac rhabdomyomas. After treatment, the rhabdomyomas shrank rapidly, but the serum concentration of everolimus increased sharply (maximum serum trough level: 76.1 ng/mL) and induced complications including pulmonary hemorrhage, liver dysfunction, and acne. After the everolimus level decreased, the complications resolved. Everolimus may be a viable treatment option for rhabdomyomas, but its concentration requires close monitoring to circumvent complications associated with its use. |
| first_indexed | 2024-12-23T05:06:56Z |
| format | Article |
| id | doaj.art-e7bd1d47fbce4548951ad5593f752496 |
| institution | Directory Open Access Journal |
| issn | 2157-6998 2157-7005 |
| language | English |
| last_indexed | 2024-12-23T05:06:56Z |
| publishDate | 2019-07-01 |
| publisher | Thieme Medical Publishers, Inc. |
| record_format | Article |
| series | American Journal of Perinatology Reports |
| spelling | doaj.art-e7bd1d47fbce4548951ad5593f7524962022-12-21T17:59:04ZengThieme Medical Publishers, Inc.American Journal of Perinatology Reports2157-69982157-70052019-07-010903e213e21710.1055/s-0039-1692198Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous SclerosisYuka Shibata0Hidehiko Maruyama1Taiyu Hayashi2Hiroshi Ono3Yuka Wada4Hideshi Fujinaga5Shuhei Fujino6Junko Nagasawa7Shoichiro Amari8Keiko Tsukamoto9Yushi Ito10Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Cardiology, National Center for Child Health and Development, Setagaya, Tokyo, JapanDivision of Cardiology, National Center for Child Health and Development, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Setagaya, Tokyo, JapanMost cardiac rhabdomyomas with tuberous sclerosis (TS) are asymptomatic and spontaneously regress. However, some cases require surgical intervention due to arrhythmia and severe obstruction of cardiac inflow or outflow. We report herein a neonatal case of giant cardiac rhabdomyomas with TS and insufficient pulmonary blood flow from the right ventricle. Lipoprostaglandin E1 was necessary to maintain patency of the ductus arteriosus. We used everolimus, a mammalian target of rapamycin inhibitor, to diminish the cardiac rhabdomyomas. After treatment, the rhabdomyomas shrank rapidly, but the serum concentration of everolimus increased sharply (maximum serum trough level: 76.1 ng/mL) and induced complications including pulmonary hemorrhage, liver dysfunction, and acne. After the everolimus level decreased, the complications resolved. Everolimus may be a viable treatment option for rhabdomyomas, but its concentration requires close monitoring to circumvent complications associated with its use.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1692198complicationeverolimusrhabdomyomatuberous sclerosis |
| spellingShingle | Yuka Shibata Hidehiko Maruyama Taiyu Hayashi Hiroshi Ono Yuka Wada Hideshi Fujinaga Shuhei Fujino Junko Nagasawa Shoichiro Amari Keiko Tsukamoto Yushi Ito Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis American Journal of Perinatology Reports complication everolimus rhabdomyoma tuberous sclerosis |
| title | Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis |
| title_full | Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis |
| title_fullStr | Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis |
| title_full_unstemmed | Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis |
| title_short | Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis |
| title_sort | effect and complications of everolimus use for giant cardiac rhabdomyomas with neonatal tuberous sclerosis |
| topic | complication everolimus rhabdomyoma tuberous sclerosis |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1692198 |
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