Management of Pulmonary Hypertension Due to Chronic Lung Disease

Abstract

Pulmonary hypertension (PH) is a known complication of chronic parenchymal lung diseases, including chronic obstructive lung disease, interstitial lung diseases, and more rare parenchymal lung diseases. Together, these diseases encompass two of the five clinical classifications of PH: group 3 (chronic lung disease [CLD] and/or hypoxia) and group 5 (unclear and/or multifactorial mechanisms). The principal management strategy in PH associated with CLD is optimization of the underlying lung disease. There has been increasing interest in therapies that treat pulmonary arterial hypertension (group 1, PAH), and although some studies have explored the use of these oral PAH-targeted therapies to treat PH associated with CLD , there is currently no evidence to support their routine use; in fact, some studies suggest harm. Inhaled therapies that target the pulmonary vasculature may avoid certain problems observed with oral PAH therapies. Recent studies suggest a promising role for inhaled PAH therapies in group 3 PH, but this requires further study. The objective of this article is to review the current treatment strategies for group 3 and group 5 PH.

Pulmonary Hypertension in Interstitial Lung Disease: Updates in Disease, Diagnosis, and Therapeutics

“…Hopefully, further study will shed more light as to the relative risks Beyond pulmonary vasodilators, management of PH-ILD continues to be anchored in the treatment of the patient's underlying lung disease and management of contributors to hypoxemia [6]. Patients with hypoxemia at rest, defined as a PaO 2 < 60 mmHg or SpO 2 < 92%, should be considered for continuous supplemental oxygen to reduce hypoxic vasoconstriction and improve PVR [56,57]. Additionally, patients should be screened for sleep-related oxygen desaturations and sleep disordered breathing, and be provided supplemental oxygen or ventilatory support as indicated [58,59].…”