Outcomes of lung disease-associated pulmonary hypertension and impact of elevated pulmonary vascular resistance

Abstract

Background: The clinical characteristics, hemodynamic changes and outcomes of lung disease-associated pulmonary hypertension (LD-PH) are poorly defined. Methods: A prospective cohort of PH patients undergoing initial hemodynamic assessment was collected, from which 51 patients with LD-PH were identified. Baseline characteristics and long-term survival were compared with 83 patients with idiopathic pulmonary arterial hypertension (iPAH). Results: Mean age ( ± standard deviation) of LD-PH patients was 64 ± 10 years, 30% were female and 78% were New York Heart Association class III-IV. The LD-PH group was older than the iPAH group (64 ± 10 vs 56 ± 18 years, respectively, P = 0.003) with a lower percentage of women (30% vs 70%, P = 0.007). LD-PH patients had smaller right ventricular sizes (P = 0.02) and less tricuspid regurgitation (P = 0.03) by echocardiogram, and lower mean pulmonary arterial pressures (mPAP) (P = 0.01) and pulmonary vascular resistance (PVR) (P = 0.001) at catheterization. Despite these findings, mortality was equally high in both groups (P = 0.16). 5-year survival was lower in patients with interstitial lung disease compared to those with obstructive pulmonary disease (P = 0.05). Among the LD-PH population, those with mild to moderately elevated mPAP and those with PVR < 7 Wood units demonstrated significantly improved survival (P = 0.04 and P = 0.001, respectively). Vasoreactivity was not associated with improved survival (P = 0.64). A PVR ≥7 Wood units was associated with increased risk of mortality (hazard ratio (95% confidence interval), 3.59 (1.27-10.19), P = 0.02). Conclusions: Despite less severe PH and less right heart sequelae, LD-PH has an equally poor clinical outcome when compared to iPAH. A PVR ≥7 Wood units in LD-PH patients was associated with 3-fold higher mortality.

ERS International Congress, Madrid, 2019: highlights from the Pulmonary Vascular Diseases Assembly

“…This reinforces the study by A werbach et al [ 24 ], who showed in interstitial lung disease (ILD)-related PH that PVR >7 WU was associated with three-fold higher mortality than PVR <7. A werbach et al [ 24 ] also demonstrated that ILD-related PH patients, at right heart catheterisation, had lower mPAP and PVR when compared to their idiopathic pulmonary arterial hypertension (PAH) cohort; despite these findings, mortality was high in both groups.…”

“…This reinforces the study by Awerbach et al, who showed in ILD related PH, PVR > 7WU was associated with 3-fold higher mortality than PVR <7. Awerbach et al also demonstrated that ILD related PH patients at right heart catheterization (RHC), had lower mean pulmonary arterial pressures (mPAP) and pulmonary vascular resistance (PVR) when compared to their idiopathic PAH cohort, despite these findings, mortality was high in both groups (24).…”

Pulmonary Hypertension in COPD: A Case Study and Review of the Literature

“…Rates of mortality were noted to be lower at one, two, and three years in PH-COPD compared to PH-ILD. A 2019 study comparing 51 patients with PH due to chronic lung disease to 83 patients with IPAH demonstrated that the former patient group had equally poor outcomes despite lower mean pulmonary artery pressure and pulmonary vascular resistance [31]. A PVR of seven Wood units or greater was noted to confer a 3-fold higher risk of mortality and was more strongly associated with mortality than mPAP.…”

Acute Respiratory Failure in Interstitial Lung Disease Complicated by Pulmonary Hypertension

“…Although RHC is not without risk, early RHC has demonstrated improved survival when findings have influenced management [62]. Additionally, a PVR of greater than �7 Wood Unit was found to have three times the risk of mortality in Group 3 PH patients, highlighting the prognostic value of RHC [102]. Despite common reservation, RHC should always be performed when an ILD patient is suspected to have PH and PH specific therapy is being considered.…”