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Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome
We present a case of double‐chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation‐associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160468/ https://www.ncbi.nlm.nih.gov/pubmed/32078254 http://dx.doi.org/10.1002/ehf2.12650 |
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author | Yamamoto, Masahiro Takashio, Seiji Nakashima, Naoya Hanatani, Shinsuke Arima, Yuichiro Sakamoto, Kenji Yamamoto, Eiichiro Kaikita, Koichi Aoki, Yoko Tsujita, Kenichi |
author_facet | Yamamoto, Masahiro Takashio, Seiji Nakashima, Naoya Hanatani, Shinsuke Arima, Yuichiro Sakamoto, Kenji Yamamoto, Eiichiro Kaikita, Koichi Aoki, Yoko Tsujita, Kenichi |
author_sort | Yamamoto, Masahiro |
collection | PubMed |
description | We present a case of double‐chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation‐associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not surgically treated, may cause paralysis of the extremities. We decided to pursue pharmacological therapy to control biventricular obstructions and reduce the perioperative complication rate. We initiated treatment with cibenzoline and bisoprolol; the doses were titrated according to the response. After 2 weeks, the peak pressure gradient of the two RV chambers decreased from 101 to 68 mmHg, and the LV peak pressure gradient decreased from 109 to 14 mmHg. Class 1A antiarrhythmic drugs and β‐blockers decreased the severe pressure gradients of biventricular obstructions caused by DCRV and HOCM. The patient was able to undergo surgery to remove the intradural extramedullary tumour, which was diagnosed as schwannoma. |
format | Online Article Text |
id | pubmed-7160468 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-71604682020-04-20 Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome Yamamoto, Masahiro Takashio, Seiji Nakashima, Naoya Hanatani, Shinsuke Arima, Yuichiro Sakamoto, Kenji Yamamoto, Eiichiro Kaikita, Koichi Aoki, Yoko Tsujita, Kenichi ESC Heart Fail Case Report We present a case of double‐chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation‐associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not surgically treated, may cause paralysis of the extremities. We decided to pursue pharmacological therapy to control biventricular obstructions and reduce the perioperative complication rate. We initiated treatment with cibenzoline and bisoprolol; the doses were titrated according to the response. After 2 weeks, the peak pressure gradient of the two RV chambers decreased from 101 to 68 mmHg, and the LV peak pressure gradient decreased from 109 to 14 mmHg. Class 1A antiarrhythmic drugs and β‐blockers decreased the severe pressure gradients of biventricular obstructions caused by DCRV and HOCM. The patient was able to undergo surgery to remove the intradural extramedullary tumour, which was diagnosed as schwannoma. John Wiley and Sons Inc. 2020-02-20 /pmc/articles/PMC7160468/ /pubmed/32078254 http://dx.doi.org/10.1002/ehf2.12650 Text en © 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes. |
spellingShingle | Case Report Yamamoto, Masahiro Takashio, Seiji Nakashima, Naoya Hanatani, Shinsuke Arima, Yuichiro Sakamoto, Kenji Yamamoto, Eiichiro Kaikita, Koichi Aoki, Yoko Tsujita, Kenichi Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title | Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title_full | Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title_fullStr | Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title_full_unstemmed | Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title_short | Double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome |
title_sort | double‐chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as noonan syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160468/ https://www.ncbi.nlm.nih.gov/pubmed/32078254 http://dx.doi.org/10.1002/ehf2.12650 |
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