Pulmonary hypertension had been recognized as an intractable disease with poor prognosis. In Japan, oral pulmonary vasodilators have been approved one after another since 2005, resulting in drastic improvement of treatment outcomes. In addition, based on 6th World Symposium held in Nice 2018, our knowledge of mechanisms responsible for development and progression of pulmonary arterial hypertension has been significantly increased. However, underlying mechanisms of pulmonary arterial hypertension are heterogeneous, and some patients are still difficult to diagnose correctly.
The “multiple-hits theory” is proposed as an underlying mechanism for development of pulmonary arterial hypertension, but the genetic, molecular, pathological and environmental mechanisms responsible for the development of pulmonary arterial hypertension have not been fully elucidated. In this symposium, we would like to discuss factors critical for the development and progression of pulmonary vascular remodeling, as well as potential future treatment targets based on the pathogenesis of pulmonary arterial hypertension.