Advanced Diagnosis and Therapy for Pulmonary Arterial Hypertension

Abstract

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and progressive disease, which often leads to premature death. Pulmonary arterial hypertension (PAH, group 1 PH) comprises of diverse diseases that result in similar pathological changes, including the medium-sized pulmonary arteries and pulmonary arterioles characterized by vascular obliteration. The evaluation and diagnosis of PH and PAH were re-defined based on proceedings of the 6th World Symposia on Pulmonary Hypertension (WSPH). Accurate early diagnosis and subsequent therapy of PAH are necessary, as management of this disease is still challenging, and life expectancy is remaining suboptimal. This review task force reflects the multidisciplinary nature of PAH, including the definition, epidemiology, genetics, especially the advanced diagnosis and the therapy development in recent years.