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

Respir Med. 2019 Apr;150:126-130. doi: 10.1016/j.rmed.2019.03.004. Epub 2019 Mar 15.

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.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Cardiac Catheterization / methods
  • Echocardiography / methods
  • Familial Primary Pulmonary Hypertension / epidemiology
  • Familial Primary Pulmonary Hypertension / mortality*
  • Familial Primary Pulmonary Hypertension / physiopathology
  • Female
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Hemodynamics / physiology
  • Humans
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / physiopathology
  • Lung / blood supply*
  • Lung / physiopathology
  • Lung Diseases, Interstitial / epidemiology
  • Lung Diseases, Interstitial / mortality
  • Lung Diseases, Interstitial / physiopathology
  • Lung Diseases, Obstructive / epidemiology
  • Lung Diseases, Obstructive / mortality
  • Lung Diseases, Obstructive / physiopathology
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulmonary Artery / physiopathology
  • Survival Analysis
  • Tricuspid Valve Insufficiency / diagnostic imaging
  • Tricuspid Valve Insufficiency / epidemiology
  • Vascular Resistance / physiology*