Prospective Validation of the Novel PVD-B65 Risk Score in Patients With Chronic Lung Disease and Pulmonary Hypertension

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

• Composite endpoint of clinical worsening defined as hospitalization for respiratory failure, worsening oxygen requirement, intensification of pulmonary vasodilator therapy from initial treatment
• 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)
• Change in lung function (FEV1, FVC), 6MWD, and DLCO
• Change in PVD-B65 score and associated risk severity through duration of study

Inclusion criteria

• Eligibility criteria

• Adult patients ≥ 18 years of age with chronic lung disease diagnosed via CT chest and/or PFT data and pre-capillary pulmonary hypertension diagnosed via right-heart catheterization (RHC, mPAP > 20 mmHg, PVR > 2 WU, and PCWP ≤ 15 mmHg)

• Chronic lung disease diagnoses will include: COPD, IPF, other pulmonary fibrosis, non-fibrotic ILD, combined pulmonary fibrosis and emphysema, and advanced pulmonary sarcoidosis with parenchymal involvement
• PFT criteria will include an FEV1/FVC < 0.70 for the diagnosis of COPD
• Willingness to make return visits and be available by telephone for the duration of the study.
• Ability to participate in necessary testing, including ambulatory testing

Exclusion criteria

• Exclusion criteria

• Patients with pulmonary hypertension but without associated chronic lung disease (i.e. idiopathic or group 1 PAH, PH with post-capillary component defined as PCWP > 15 mmHg or group 2 PAH, CTEPH or group 4 PH, group 5 PH aside from sarcoidosis with parenchymal involvement)
• Patients with uncontrolled severe systemic disease that could influence life expectancy (i.e. uncontrolled cardiovascular disease, active malignancy, etc.)
• Prior lung and/or heart transplantation