|
KMID : 1141320170320020227
|
|
Kosin Medical Journal
2017 Volume.32 No. 2 p.227 ~ p.232
|
|
|
Pulmonary Hypertension in Patient with Neurofibromatosis Type 1
|
|
Lee Yeong-Min
Yang Tae-Hyun
Jung Hee-Soo
Oh Tae-Seon
Song Jeong-Hoon
Yu Seung-Jung
|
|
|
Abstract
|
|
|
|
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.
|
|
|
KEYWORD
|
|
|
Bosentan, Cardiac catheterization, Hypertension pulmonary, Neurofibromatosis 1
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
 
|