Title:Pulmonary Hypertension Associated with Chronic Lung Disease
Volume: 20
Issue: 2
Author(s): Yuanchen Wang, Rodolfo A. Estrada, Khushboo Goel, Rodney J. Folz and Sandeep Sahay*
Affiliation:
- Division of Pulmonary, Critical Care, and Sleep Medicine, Houston Methodist Hospital, USA
Keywords:
Pulmonary hypertension, pulmonary arterial hypertension, chronic obstructive pulmonary disease, interstitial lung disease, obstructive sleep apnea, pathophysiology, diagnosis, management.
Abstract: Patients with Chronic Lung Disease (CLD) are frequently burdened by pulmonary hypertension
(PH), which is associated with reduced functional capacity, poor quality of life, increased
oxygen requirements, and increased morbidity and mortality. The development of PH associated
with chronic lung disease (PH-CLD) is complex and multifactorial and varies between
different types of CLD. In this review, we provide an update on PH-CLD, with a particular focus
on Interstitial Lung Disease (ILD), chronic obstructive pulmonary disease (COPD), obstructive
sleep apnea (OSA), and obesity hypoventilation syndrome (OHS). We discussed epidemiology,
histopathology, pathophysiology, diagnostic evaluation, and treatment approaches. There are limited
data on the use of pulmonary arterial hypertension-specific treatments in PH-CLD, so it has
been proposed to phenotype patients based on their degree of pulmonary vascular disease to guide
individualized care. The heterogeneity within PH-CLD highlights the importance of identifying
novel molecular pathways unique to each subgroup to ultimately achieve precision medicine.