Pulmonary Hypertension in Patient with Neurofibromatosis Type 1.
10.7180/kmj.2017.32.2.227
- Author:
Yeong min LEE
1
;
Tae Hyun YANG
;
HeeSoo JUNG
;
Tae Seon OH
;
Jeong Hoon SONG
;
Seung Jung YU
Author Information
1. Department of Internal Medicine, Inje University, Haeundae Paik Hospital, Busan, Korea. yangthmd@naver.com
- Publication Type:Case Report
- Keywords:
Bosentan;
Cardiac catheterization;
Hypertension pulmonary;
Neurofibromatosis 1
- MeSH:
Aged;
Cardiac Catheterization;
Cardiac Catheters;
Dyspnea;
Female;
Heart;
Humans;
Hydrogen-Ion Concentration;
Hypertension, Pulmonary*;
Neurofibromatoses*;
Neurofibromatosis 1*;
Receptors, Endothelin
- From:Kosin Medical Journal
2017;32(2):227-232
- CountryRepublic of Korea
- Language:English
-
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.
