August 26, 2025
4 min read
Key takeaways:
- The PVD-B65 risk assessment tool includes five factors that each significantly raise the risk for 1-year mortality in those with chronic lung disease and pulmonary hypertension.
- Internal validation was achieved.
A risk assessment tool including five factors identified whether patients with chronic lung disease and pulmonary hypertension face a high, intermediate or low risk for 1-year mortality, according to study results.
These findings were published in BMC Pulmonary Medicine.
“For frontline clinicians, the score offers several practical benefits: improved prognostication at the point of diagnosis; a framework to guide decisions regarding referral for lung transplantation, palliative care discussions or therapeutic escalation; and enhanced clarity in shared decision-making with patients and caregivers,” Mario Naranjo-Tovar, MD, MHS, assistant professor of clinical thoracic medicine and surgery at the Lewis Katz School of Medicine at Temple University, told Healio.
“Because the score relies exclusively on data routinely collected in clinical practice, it is both feasible and scalable for real-world implementation,” Naranjo-Tovar said.
In a retrospective cohort study, Naranjo-Tovar and colleagues evaluated 793 patients (mean age, 63.3 years; 50.8% men; 49.9% non-Hispanic white) with chronic lung disease and pulmonary hypertension (PH) to determine predictors of 1-year mortality from time of PH diagnosis, create a risk assessment tool using this information and validate it.
“Patients with chronic lung diseases — like pulmonary fibrosis or COPD — often go on to develop PH, a condition that dramatically worsens survival,” Naranjo-Tovar told Healio. “However, clinicians have lacked a validated, disease-specific tool to predict mortality in this high-risk group.
“Existing risk scores are designed for pulmonary arterial hypertension and don’t adequately account for the unique pathophysiology seen in patients whose PH arises from underlying lung disease,” he added.
The most common underlying lung disease in this population was COPD (41.6%), followed by pulmonary fibrosis without emphysema (31.7%), combined pulmonary fibrosis and emphysema (13.2%), advanced pulmonary sarcoidosis (8.8%) and non-fibrotic interstitial lung disease (4.7%).
Using multivariable Cox regression, researchers found five factors that significantly raised the risk for 1-year mortality and were deemed predictors:
- pulmonary fibrosis without emphysema (HR = 4.42; 95% CI, 1.41-13.87);
- age older than 65 years (HR = 3.3; 95% CI, 1.36-8.02);
- B-type natriuretic peptide (BNP) greater than 200 pg/dL (HR = 2.77; 95% CI, 1.18-6.51);
- 6-minute walk distance (6MWD) less than 150 m (HR = 2.5; 95% CI, 1.41-8.45); and
- pulmonary vascular resistance (PVR) greater than 5 WU (HR = 2.17; 95% CI, 1.53-3.57).
“Notably, mean pulmonary artery pressure — a cornerstone metric for diagnosing PH — did not emerge as an independent predictor of 1-year mortality,” Naranjo-Tovar told Healio. “This suggests that traditional hemodynamic severity may be less prognostically relevant in chronic lung disease-associated PH than previously assumed.
“These insights reinforced the notion that functional capacity (eg, 6-minute walk distance) and biomarkers of cardiac strain (eg, BNP) may offer superior predictive value over isolated hemodynamic metrics in this population,” he continued.
The study reported that each predictor was given a point value based on its hazard ratio, meaning that pulmonary fibrosis without emphysema had the highest point value (4 points), age older than 65 and BNP greater than 200 pg/dL had the second highest point value (each 3 points) and 6MWD less than 150 m and PVR greater than 5 WU had the lowest point value (each 2 points).
Researchers outlined that a total score of 0 to 3 signaled low risk, a score of 4 to 10 signaled intermediate risk and a score of 11 to 14 signaled high risk. Using these ranges in the 407 patients with full data to calculate a risk score, 155 had low risk, 212 had intermediate risk and 40 had high risk.
Aligning with their calculated PVD-B65 tool risk score, the high-risk group had the highest proportion of patients who died by 1 year at 17.5%, according to the study. Following suit, the intermediate-risk group had the second highest proportion (9.4%) and the low-risk group had the lowest proportion (3.9%).
Researchers also found that the risk for 1-year mortality was significantly elevated among those with high vs. low risk (HR = 6.2; 95% CI, 2.08-18.48) and those with intermediate vs. low risk (HR = 2.59; 95% CI, 1.04-4.65) in Cox regression analysis.
Additionally, 1-year survival probability significantly differed between those with high vs. intermediate risk and those with intermediate vs. low risk in Kaplan-Meier survival analysis, according to the study.
Lastly, researchers reported that the PVD-B65 risk score calculator achieved internal validation in Cox regression with bootstrapping, noting that the findings were comparable to those observed above in the original Cox regression analysis.
“The development of the PVD-B65 score represents a significant advancement in risk stratification for chronic lung disease-PH patients,” Naranjo-Tovar told Healio. “By integrating five binary clinical variables, the model provides an intuitive, evidence-based method for estimating 1-year mortality risk.”
Moving forward, Naranjo-Tovar said external validation is needed.
“Future investigations will focus on external validation across diverse patient cohorts and health care systems to assess the model’s generalizability,” Naranjo-Tovar told Healio.
“The PVD-B65 score fills a longstanding gap in pulmonary vascular medicine: a validated, lung disease-specific tool to predict 1-year mortality in patients with PH,” he said. “It’s clinically accessible, prognostically robust and offers a new lens through which clinicians can individualize care for a complex and vulnerable population.”
For more information:
Mario Naranjo-Tovar, MD, MHS, can be reached at mario.naranjo-tovar@tuhs.temple.edu.
Perspective
PH in the context of chronic lung disease remains a formidable clinical challenge, often portending poor outcomes and limited therapeutic clarity. In this study, Gayen et al introduce the PVD-B65 risk score — a novel, internally validated tool designed to predict 1-year mortality in patients with PH in the setting of chronic lung disease.
The retrospective cohort included 793 patients diagnosed with severe PH diagnosed via right heart catheterization (mPAP > 35 mmHg or mPAP 25 mmHg with reduced cardiac index in accordance with the 2015 guidelines, or PV > 5 WU in accordance with 2022 guidelines) between 2011 and 2023. Underlying lung diseases included COPD (42%), pulmonary fibrosis without emphysema (32%), CPFE (13%), sarcoidosis (9%) and non-fibrotic ILD (5%). The score integrates five variables: pulmonary fibrosis without emphysema, pulmonary vascular resistance (PVR) greater than 5 WU, 6MWD less than 150 m, BNP greater than 200 pg/mL and age greater than 65 years. The score stratifies patients into low, intermediate and high-risk categories with statistically significant survival differences.
Clinically, this tool has the potential to address an important gap. Existing risk models, largely derived from PAH cohorts, fail to account for the heterogeneity and complexity of PH in chronic lung disease. Further, the components of the risk score (PVR, 6MWD, BNP, type of chronic lung disease) are already widely used in clinical practice, increasing its appeal for “real world” applications. When a patient scores in the intermediate or high-risk categories, for example, clinicians may be prompted to augment therapy (when feasible) or refer for consideration of lung transplant, if appropriate.
External validation of the tool is certainly warranted and will likely be quite informative. Going forward, risk stratification tools tailored toward specific types of chronic lung disease — which are both clinically and physiologically heterogeneous — would be invaluable, particularly as treatment options continue to evolve. Nonetheless, the PVD-B65 risk score’s simplicity and applicability make it a promising candidate for integration into routine practice.
Kathryn T. del Valle, MD
Disclosures: Del Valle reports no relevant financial disclosures.
