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

The efficacy and safety of sildenafil in Chinese patients with pulmonary arterial hypertension

“…In historical PAH patients receiving only conventional therapy in China, survival at 1 year has been documented to be 68.0%. 6 In this study, the survival of patients who received sildenafil treatment was 94.7% at the end of 1 year. This result suggests that sildenafil treatment might improve the prognosis in Chinese PAH patients in a manner similar to the results that have been reported in Western patients.…”

“…4,5 According to the idiopathic and familial PAH registry study in China, in which patients received only conventional therapy, the survival at 1, 2, 3 and 5 years was found to be 68.0, 56.9, 38.9 and 20.8%, respectively. 6 Although iloprost and bosentan have been approved for the treatment of PAH in China, for economic reasons, few patients have been treated with these agents.…”

The fifth world symposium on pulmonary hypertension will REVEAL the impact of registries: Figure 1.

“…Thus, a number of contemporary registries have been initiated worldwide to complement and update information from the pioneering NIH registry [1][2][3][4][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22], and data generated by these registries have been extremely helpful for investigators. As examples of the practical interest of such research, developing countries have produced high-quality data demonstrating the rapid progress of disease management in China [19,20], and the specific causes and outcomes of PAH subsets in Brazil (where schistosomiasis remains a major burden) [21,22]. More recently, specific registries have studied other forms of pulmonary hypertension, such as chronic thromboembolic pulmonary hypertension, a major cause of pulmonary vascular disease that can be cured by pulmonary endarterectomy [23].…”

Trends in pulmonary arterial hypertension

“…These studies vary in terms of quality of data (some are single centred, others lack routine use of right heart catheterisation for the appropriate diagnosis of PAH), but we now have data available from France [17], Scotland (UK) [18], Switzerland [19], China [20], USA [21] and Brazil [22].…”

“…Although none of the studies was designed to properly evaluate first-line treatment, the USAbased registry from a single reference centre [21] and the Chinese registry [20] reported a high proportion of patients using calcium channel blockers, despite the relatively rare response to the acute vasodilator challenge. To what extent this directly reflects drug availability (especially in China), and the consequences of such conduct, are so far only matter of speculation.…”