Objectives:
Primary objective: Prospectively validate the PVD-B65 risk score in patients newly diagnosed with or referred to our center with chronic lung disease and pulmonary hypertension by assigning a PVD-B65 score at time of diagnosis and/or referral and following one-year mortality outcome.
Secondary objectives:
* Assess whether the risk severity assigned from the PVD-B65 score in patients newly diagnosed with or referred to our center with chronic lung disease and pulmonary hypertension can predict clinical worsening.
* Assess whether optimal treatment in line with standard of care of both chronic lung disease and pulmonary hypertension can modify the initial risk severity assigned from the PVD-B65 score and in turn influence survival outcomes.
Primary endpoint: One-year outcome of mortality vs. lung transplantation vs. alive without lung transplantation
Secondary endpoints
1. Composite endpoint of clinical worsening defined as hospitalization for respiratory failure, worsening oxygen requirement, intensification of pulmonary vasodilator therapy from initial treatment
2. Change in PH markers (mPAP, PVR, cardiac output, cardiac index via RHC; PASP via TTE, pulmonary artery diameter and pulmonary artery to aorta ratio on CT chest)
3. Change in lung function (FEV1, FVC), 6MWD, and DLCO
4. Change in PVD-B65 score and associated risk severity through duration of study
