Chest
Volume 132, Issue 2, August 2007, Pages 373-379
Journal home page for Chest

Original Research
Pulmonary Hypertension
Registry and Survival Study in Chinese Patients With Idiopathic and Familial Pulmonary Arterial Hypertension

https://doi.org/10.1378/chest.06-2913Get rights and content

Background

To evaluate the clinical features and survival data of patients with idiopathic pulmonary arterial hypertension (PAH) and familial PAH in Chinese patients.

Methods

Seventy-two patients with idiopathic PAH and familial PAH were enrolled in the study from 1999 to 2004 and were classified into two groups according to World Health Organization (WHO) functional class (I/II and III/IV). Clinical and hemodynamic data were recorded.

Results

The mean age of the 72 patients was 35.9 years with female patient/male patient ratio of 2.4:1. A significant difference was identified in the clinical presentation between two WHO functional class groups at baseline. Echocardiography showed a mean pulmonary systolic pressure of 98 mm Hg. Left ventricular end-diastolic diameter was significantly smaller in the group of patients in WHO functional class III/IV than in those in class I/II group. After follow-up for a mean (± SD) duration of 40.1 ± 20.0 months, the survival rates at 1, 2, 3, and 5 years were 68.0%, 56.9%, 38.9%, and 20.8%, respectively. A significant difference was identified in survival rate between the class I/II and class III/IV groups (p = 0.02 [log rank test]).

Conclusions

The baseline characteristics and survival rates of our cohort study are close to those of the National Institutes of Health Registry in the 1980s, and the 1-year survival rate is obviously lower for patients in this registry than for those in the French registry between 2002 to 2003. Lack of effective treatment was the main cause of poor survival in this study. Our results support the need of an appropriate treatment strategy for this devastating disease in China.

Section snippets

Patients

This registry study was conducted at Fu Wai Hospital, Chinese Academy of Medical Sciences, and Peking Union Medical College from January 1999 to October 2004. A total of 72 consecutive patients in whom idiopathic and familial PAH had been diagnosed were enrolled into this study. Patients with suspected pulmonary hypertension were admitted to a hospital ward for further evaluation. PAH was defined as a mean PAP of > 25 mm Hg at rest by right heart catheterization (RHC)6 or systolic PAP exceeding

Demographic Characteristics and Comparison With NIH Registry and French Registry

Of the 72 patients enrolled in this study, 21 were men and 51 were women (Table 1). The female patient/male patient ratio was 2.4:1. The mean age was 35.9 ± 12.2 years (range, 9.7 to 73.8 years). According to different genders, there was no difference in patients' age when they received the first medical examination (p = 0.36). Of these 72 patients, 11.1% (8 patients) were < 20 years old, 56.9% (41 patients) were 20 to 39 years old, 27.8% (20 patients) were 40 to 59 years old, and 4.2% (3

Discussion

The present study is the first report from China on data concerning idiopathic and familial PAH. Dyspnea on exertion is the most common symptom of disease onset, occurring in 98.6% of Chinese patients with idiopathic and familial PAH, predominantly in women (women/men ratio, 2.4:1); idiopathic and familial PAH can occur at any age but most often occurred between the ages of 20 and 59 years. The survival rate decreased dramatically after 5 years. These results were similar to those in the report

References (17)

There are more references available in the full text version of this article.

Cited by (0)

This study was supported by research grant No. 2002BA711A08 (“National Key Technologies R&D Program”) from the National Ministry of Science and Technology, People's Republic of China.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

These authors contributed equally to the work.

View full text