Clinical characteristics of 195 Chinese patients with WHO Class Ⅰ pulmonary hypertension
10.3760/cma.j.issn.0253-3758.2014.12.006
- VernacularTitle:世界卫生组织第Ⅰ类肺动脉高压患者临床特征分析
- Author:
Gangcheng ZHANG
1
;
Xiaoke SHANG
;
Xiaoxian DENG
;
Hongmei ZHOU
Author Information
1. 430022,武汉亚洲心脏病医院先天性心脏病中心
- Keywords:
Hypertension,pulmonary;
Disease attributes
- From:
Chinese Journal of Cardiology
2014;42(12):1001-1005
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics of WHO Class Ⅰ pulmonary hypertension (PAH) in central China.Methods Data was collected as a part of prospective registry of PAH through Jan.2009 to Oct.2013 in Wuhan Asia Heart Hospital.A total of 195 patients were recruited including 144 cases with congenital heart disease with pulmonary hypertension (CHD-PAH) and 51 cases with idiopathic pulmonary hypertension(IPAH).Results The age of all patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years),129 cases were female(66.2％).WHO Class Ⅰ PAH accounted for 91.1％,CHD-PAH 67.3％,IPAH 23.8％,and other 8.9％.WHO function class Ⅲ/Ⅳ in newly diagnosed PAH accounted for 32.3％,the mean 6MWD was (397 ±74) m.For patients with IPAH,the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months.The mean pulmonary pressure,pulmonary vascular resistance index,cardiac index of patients with IPAH and CHDPAH were measured by the right heart catheterization and there was no difference between the two groups.Acute pulmonary vasodilator testing was negative in all patients in this cohort.Cardiac function was improved in the 121 cases who received the targeted drug treatment and 1 patient died out of these 121 patients while 5 cases died out of patients receiving conventional therapy.Conclusion In Central China,Class Ⅰ pulmonary hypertension is the most predominant type of PAH,the cardiac function and hemodynamic indexes of these patients were significantly impaired at the time of first PAH diagnosis.Most of the patients accepted targeted drug treatment of pulmonary hypertension,but the drug dose used for the targeted drug treatment is not effective enough in these patients.
