Pulmonary Hypertension in Patient with Neurofibromatosis Type 1 | Zendy

Yeong min Lee | Zendy; TaeHyun Yang | Zendy; Heesoo Jung | Zendy; Tae-Seon Oh | Zendy; Song Jeong-Hoon | Zendy; Seung-Jung Yu | Zendy

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Pulmonary Hypertension in Patient with Neurofibromatosis Type 1

Author(s) -

Yeong min Lee,

TaeHyun Yang,

Heesoo Jung,

Tae-Seon Oh,

Song Jeong-Hoon,

Seung-Jung Yu

Publication year - 2017

Publication title -

kosin medical journal

Language(s) - English

Resource type - Journals

eISSN - 2586-7024

pISSN - 2005-9531

DOI - 10.7180/kmj.2017.32.2.227

Subject(s) - medicine , neurofibromatosis , pulmonary hypertension , exertion , right heart catheterization , complication , endothelin receptor antagonist , family history , cardiology , cardiac catheterization , pediatrics , antagonist , surgery , radiology , receptor

Neurofibromatosis type 1 (NF1) is a rare genetic disease. Precapillary pulmonary hypertension (PH) with NF1 is an extremely severe complication. A 65-year-old woman was admitted in our hospital with 3-year history of gradually worsening dyspnea on exertion (New York Heart Association functional class III-IV). Considering her clinical feature and examination findings, she could be diagnosed as PH associated with NF1. She was treated with endothelin receptor antagonist. However her dyspnea was not significantly improved. This is the first Korean case of NF1 patient with PH which confirmed with right heart catheterization.

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