Outpatient Intravenous Dobutamine as Right Ventricular Support During Inhaled Prostacyclin Uptitration in Severe PH-ILD

Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival. 

Lung vasculature arises from both pulmonary and systemic (bronchial) circulations. Remodeling and structural changes in lung vasculature have been recognized in end-stage fibrotic lung diseases such as idiopathic pulmonary fibrosis (IPF) but have not been well characterized. 

Phenotypic transition of pulmonary artery smooth muscle cells (PASMCs) under hypoxic conditions, which in turn causes increased proliferation and migration capacity, is an important pathological process in Hypoxic pulmonary hypertension (HPH). Although research on the phenotypic transition of PASMCs has been ongoing, little is known about the specific molecular mechanisms underlying this process.

Prognostic markers of Group 3 pulmonary hypertension (PH) remain largely unknown. In this study, we evaluate clinical data to provide a comprehensive profile of patients with Group 3 PH and evaluate the potential use of vasoreactivity testing as a prognostic tool within this population.