摘要
Pulmonary hypertension is a pathophysiologic process characterized by progressive elevation of pulmonary vascular resistance and right heart failure, which is a common complication of many diseases. Pulmonary hypertension with no apparent causes （unknown etiology） is termed primary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension （IPAH）. Before the availability of disease-specific （targeted） therapy （through the mid-1980s） the median life expectancy from the time of diagnosis in patients with this disease was 2.8 years. Modem treatment has markedly improved physical function and has extended survival, and the 5-year mortality is 50%. Although there is already more than 100 years of research history, the mechanisms of this disease are still not very clear. Recently, with the development of cell biology and molecular genetics, further research into the mechanisms responsible for pulmonary hypertension have been possible, which has helped in its diagnosis and treatment. It is believed that the mechanisms of pulmonary hypertension can not only be described by pathophysiology but involve multiple factors （pathways） like cellular, humoral and molecular genetics, etc.
Pulmonary hypertension is a pathophysiologic process characterized by progressive elevation of pulmonary vascular resistance and right heart failure, which is a common complication of many diseases. Pulmonary hypertension with no apparent causes （unknown etiology） is termed primary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension （IPAH）. Before the availability of disease-specific （targeted） therapy （through the mid-1980s） the median life expectancy from the time of diagnosis in patients with this disease was 2.8 years. Modem treatment has markedly improved physical function and has extended survival, and the 5-year mortality is 50%. Although there is already more than 100 years of research history, the mechanisms of this disease are still not very clear. Recently, with the development of cell biology and molecular genetics, further research into the mechanisms responsible for pulmonary hypertension have been possible, which has helped in its diagnosis and treatment. It is believed that the mechanisms of pulmonary hypertension can not only be described by pathophysiology but involve multiple factors （pathways） like cellular, humoral and molecular genetics, etc.
基金
This work was supported by the grants from Changjiang Scholars Program （No. 985-2-087-111）, the National Science Foundation for Distinguished Young Scholars （No. 30425010）, the Major State Basic Research Development Program of China （No. 2006CB503807）, the Research Fund for the Doctoral Program of Ministry of Education of China （No. 20070001702 and No. 20070001770） and the National Natural Science Foundation of China （No. 30630031）.
