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

Inclusion Criteria:

* Eligibility criteria

  1. 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)

     1. 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
     2. PFT criteria will include an FEV1/FVC \< 0.70 for the diagnosis of COPD
  2. Willingness to make return visits and be available by telephone for the duration of the study.
  3. Ability to participate in necessary testing, including ambulatory testing

Exclusion Criteria:

* Exclusion criteria

  1. 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)
  2. Patients with uncontrolled severe systemic disease that could influence life expectancy (i.e. uncontrolled cardiovascular disease, active malignancy, etc.)
  3. Prior lung and/or heart transplantation