Pulmonary hypertensive vasculopathy in parenchymal lung diseases and/or hypoxia

摘要

Pulmonary hypertension (PH) with complicating chronic lung diseases and/or hypoxia falls into group 3 of the updated classification of PH. Patients with chronic obstructive lung disease (COPD); diffuse lung disease (such as idiopathic pulmonary fibrosis (IPF)) and with sleep disordered breathing are particularly exposed to the risk of developing PH. Although PH in such a context is usually mild; a minority of patients exhibit severe haemodynamic impairment; defined by a mean pulmonary arterial pressure (mPAP) of xe2x89xa535xe2x80x85mmHg or mPAP values ranging between 25xe2x80x85mmHg and 35xe2x80x85mmHg with a low cardiac index (<2xe2x80x85Lxc2xb7minxe2x88x921xc2xb7mxe2x88x922). The overlap between lung parenchymal disease and PH heavily affects life expectancy in such a patient population and complicates their therapeutic management. In this review we illustrate the pathological features and the underlying pathophysiological mechanisms of pulmonary circulation in chronic lung diseases; with an emphasis on COPD; IPF and obstructive sleep apnoea syndrome.