Background: Pulmonary hypertension (PH) associated with interstitial lung disease (ILD) is associated with significant mortality. Aims: To describe the demographics and outcomes of patients with ILD referred to a National PH service. Methods: All patients with ILD referred for right heart catheterisation (RHC) between 2005 and 2015 were included. Patients were excluded if they had evidence of CTEPH or <10% severity of ILD at CT. The nearest echocardiogram, CT, BNP level and lung function to the RHC were used for comparison. Results: 310 patients were included (49% male). ILD diagnoses: IPF (72), CTD-ILD (108), sarcoid (56), CHP (26), NSIP (22) and ‘other ILD’ (26). At RHC 77% had a diagnosis of PH: mean PAP was 33 +/-11 mmHg, CO 4.2 +/- 1.3 L/min and PVR 6.2 +/- 4.1 Wood units. 42 % had severe PH (mean PAP≥ 35mmHg). Patients with PH had a higher BMI, were more likely to use LTOT but had no difference in ILD CT severity. They had lower FEV1 and TLCO but similar FVC. Median follow up time was 28 months. 74% died or had a transplant. ILD sub-type heavily influenced survival: CTD-ILD and sarcoid had the best survival; IPF the worst. However, patients with CTD-ILD and sarcoid were more likely to have received vasodilators. Conclusions: This well described cohort of patients demonstrates the poor prognosis of ILD associated PH and the importance of ILD diagnosis in refining risk stratification.
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Hartmann et al. (2002) studied this question.