Article Text
Abstract
Introduction Patients with interstitial lung disease (ILD) often have signs of pulmonary hypertension (PH) when assessed non-invasively. The presence of pulmonary hypertension (PH) at right heart catheter (RHC) is a negative prognostic factor; however, the impact of elevated pulmonary vascular biomarkers in the absence of PH is poorly understood. We hypothesised that pulmonary vascular disease biomarkers would predict mortality in patients without PH at RHC.
Methods Demographics, ILD subtype, PFTs, echocardiogram, and CTs were reviewed in consecutive patients undergoing right heart catheterisation (RHC) for suspected ILD-PH. Patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at RHC were studied. Predictors of prognosis were evaluated in their ability to predict mortality using Cox proportional hazard analysis.
Results Between 2005 and 2015, 68 patients (47% male) were evaluated that did not subsequently have PH (mPAP at RHC 19±4 mmHg; Pulmonary vascular resistance (PVR) 2.5±1.4 Wood units). On CT scanning main pulmonary artery diameter (MPAdiam) was 29.9±5 mm and main pulmonary artery to aorta ratio (MPAdiam:aa) was 0.97±0.1. Median brain natriuretic peptide (BNP) was 44[29–72] (normal <20 ng.L) and predicted right ventricular systolic pressure (RVSP) at echocardiogram was 48±13 mmHg. Forced vital capacity (FVC) was 62%±22% predicted. PVR as a continuous variable predicted mortality (hazard ratio (HR):1.35, p=0.02) per unit increase. A MPAdiam ≥32 mm was associated with mortality (hazard ratio (HR):3.02, p=0.001) as was MPAdiam:aa ratio ≥0.9 (HR:4.05, p=0.001). BNP ≥40 ng.L (HR:2.47, p=0.02) and a RVSP ≥40 mmHg (HR:2.74, p=0.02) also predicted mortality. MPAdiam and PVR (expressed as a continuous variable) remained independent predictors of mortality after adjusting for ILD diagnosis, forced vital capacity (% predicted) and age at RHC.
Conclusion Even in the absence of PH at RHC, elevated pulmonary vascular biomarkers are useful in risk stratification of patients suspected of having ILD associated PH.