Chronic Pulmonary Artery Stenosis: Time to Think as a Disease Entity

Chronic pulmonary artery stenosis (CPAS) is characterized by a reduction or complete obstruction of the cross-sectional area of the pulmonary artery owing to various causes. The condition exhibits similar pathophysiological progress, leading to pulmonary hypertension (PH), reduced physical endurance, right heart failure, and death. Although CPAS is often regarded as a subgroup of PH, it can manifest independently for an extended duration before the onset of PH and can significantly impact patient quality of life. It may therefore be more appropriate to consider PH as pathophysiological progression of CPAS, thereby recognizing CPAS as a distinct disease entity.