Pulmonary hypertension complicating interstitial lung disease and COPD.

[lymphangioleiomyomatosis]

Pulmonary hypertension (PH ) may complicate parenchymal lung disease , specifically interstitial lung diseases and chronic obstructive pulmonary disease , and uniformly increases the mortality risk . The epidemiology and degree of PH is variable and unique to the underlying lung disease . The clinician should exercise a high index of suspicion for PH complicating parenchymal lung disease especially given the nonspecific symptomatology and the limitations of echocardiography in this patient population . In general , PH-specific therapies in this setting have been poorly studied , with concern for increased shunting and /or ventilation /perfusion (V /Q ) mismatch and resultant hypoxemia . A better understanding of the mechanisms underlying PH related to parenchymal lung disease may lead to novel pharmacological targets to prevent or treat this serious complication .