Overview
Prospective observational study to determine if the PVD-B65 risk score for one-year mortality in patients with chronic lung disease and pulmonary hypertension (CLD-PH) can accurately risk stratify these patients and successfully predict one-year mortality from time of pulmonary hypertension diagnosis. PVD-B65 risk score was developed in a retrospective cohort of patients with CLD-PH, utilizing the presence of pulmonary fibrosis without emphysema, pulmonary vascular resistance (PVR) > 5 woods units (WU), 6-minute walk distance (6MWD) < 150 meters, B-natriuretic type peptide (BNP) > 200 pg/mL or N-terminal pro-natriuretic type peptide (NT-proBNP) > 300 pg/dL, and age > 65 years as the score components.
Description
- 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
Eligibility
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
- 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)
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