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

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.