A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly
Quadricuspid aortic valve (QAV) is a congenital aortic valve malformation usually associated with aortic regurgitation. We encountered a patient with Noonan syndrome (NS) who had severe QAV stenosis caused by degenerative aortic cusps with an unprecedented spherical cyst-like morphology. The patient...
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| Language: | English |
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American College of Physicians
2023-08-01
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| Series: | Annals of Internal Medicine: Clinical Cases |
| Online Access: | https://www.acpjournals.org/doi/10.7326/aimcc.2022.1356 |
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| author | Chie Bujo Kensuke Tsushima Kan Nawata Ryo Inuzuka Aya Shinozaki-Ushiku Genri Numata Norifumi Takeda Masaru Hatano Hiroyuki Morita Minoru Ono Issei Komuro |
| author_facet | Chie Bujo Kensuke Tsushima Kan Nawata Ryo Inuzuka Aya Shinozaki-Ushiku Genri Numata Norifumi Takeda Masaru Hatano Hiroyuki Morita Minoru Ono Issei Komuro |
| author_sort | Chie Bujo |
| collection | DOAJ |
| description | Quadricuspid aortic valve (QAV) is a congenital aortic valve malformation usually associated with aortic regurgitation. We encountered a patient with Noonan syndrome (NS) who had severe QAV stenosis caused by degenerative aortic cusps with an unprecedented spherical cyst-like morphology. The patient had also developed adult-onset acromegaly and an elevated serum growth hormone (GH) level that might have caused myxomatous degeneration of the congenital QAV. Although recombinant human GH has been used extensively in NS patients with short stature, this patient's case suggests that it is reasonable to monitor valvular degeneration as a possible outcome of GH action and cardiac hypertrophy. |
| format | Article |
| id | doaj-art-b7013d142d584e5f996ef9e7698fd368 |
| institution | OA Journals |
| issn | 2767-7664 |
| language | English |
| publishDate | 2023-08-01 |
| publisher | American College of Physicians |
| record_format | Article |
| series | Annals of Internal Medicine: Clinical Cases |
| spelling | doaj-art-b7013d142d584e5f996ef9e7698fd3682025-08-20T02:27:22ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642023-08-012810.7326/aimcc.2022.1356A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and AcromegalyChie Bujo0Kensuke Tsushima1Kan Nawata2Ryo Inuzuka3Aya Shinozaki-Ushiku4Genri Numata5Norifumi Takeda6Masaru Hatano7Hiroyuki Morita8Minoru Ono9Issei Komuro101Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan2Department of Cardiovascular Surgery, St. Marianna University School of Medicine, Miyamae-ku, Kawasaki, Kanagawa, Japan3Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan4Department of Pathology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan5Department of Cardiac Surgery, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan1Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, JapanQuadricuspid aortic valve (QAV) is a congenital aortic valve malformation usually associated with aortic regurgitation. We encountered a patient with Noonan syndrome (NS) who had severe QAV stenosis caused by degenerative aortic cusps with an unprecedented spherical cyst-like morphology. The patient had also developed adult-onset acromegaly and an elevated serum growth hormone (GH) level that might have caused myxomatous degeneration of the congenital QAV. Although recombinant human GH has been used extensively in NS patients with short stature, this patient's case suggests that it is reasonable to monitor valvular degeneration as a possible outcome of GH action and cardiac hypertrophy.https://www.acpjournals.org/doi/10.7326/aimcc.2022.1356 |
| spellingShingle | Chie Bujo Kensuke Tsushima Kan Nawata Ryo Inuzuka Aya Shinozaki-Ushiku Genri Numata Norifumi Takeda Masaru Hatano Hiroyuki Morita Minoru Ono Issei Komuro A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly Annals of Internal Medicine: Clinical Cases |
| title | A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly |
| title_full | A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly |
| title_fullStr | A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly |
| title_full_unstemmed | A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly |
| title_short | A Case of Uncommon Quadricuspid Aortic Valve Stenosis Associated With Noonan Syndrome and Acromegaly |
| title_sort | case of uncommon quadricuspid aortic valve stenosis associated with noonan syndrome and acromegaly |
| url | https://www.acpjournals.org/doi/10.7326/aimcc.2022.1356 |
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