Severe pulmonary hypertension in lung disease: phenotypes and response to treatment

Abstract

Pulmonary hypertension (PH) due to lung disease (World Health Organization (WHO) group 3) is common, but severe PH, arbitrarily defined as mean pulmonary artery pressure ⩾35 mmHg is reported in only a small proportion. Whether these should be treated as patients in WHO group 1 (i.e. pulmonary arterial hypertension) with PH-targeted therapies is unknown.We compared the phenotypic characteristics and outcomes of 118 incident patients with severe PH and lung disease with 74 idiopathic pulmonary arterial hypertension (IPAH) patients, all treated with pulmonary vasodilators.Lung disease patients were older, more hypoxaemic, and had lower gas transfer, worse New York Heart Association functional class and lower 6-min walking distance (6MWD) than IPAH patients. Poorer survival in those with lung disease was driven by the interstitial lung disease (ILD) cohort.In contrast to IPAH, where significant improvements in 6MWD and N-terminal pro-brain natruiretic peptide (NT-proBNP) occurred, PH therapy in severe PH lung disease did not lead to improvement in 6MWD or functional class, but neither was deterioration seen. NT-proBNP decreased from 2200 to 1596 pg·mL −1 ( p=0.015). Response varied by lung disease phenotype, with poorer outcomes in patients with ILD and emphysema with preserved forced expiratory volume in 1 s. Further study is required to investigate whether vasodilator therapy may delay disease progression in severe PH with lung disease. @ERSpublications Treatment of severe pulmonary hypertension associated with lung disease can improve or stabilise noninvasive outcomes

Gaining insights into pulmonary hypertension in respiratory diseases

“…For this reason current guidelines do not recommend such treatment until more evidence on its efficacy is available [1,6]. Two articles in this issue of the European Respiratory Journal provide further insights into group 3 PH [14,15], particularly regarding its prevalence and response to treatment. RAGHU et al [14] report the characteristics and outcomes of patients with IPF and PH that participated in the ARTEMIS-IPF trial, a randomised, placebo-controlled study to evaluate the effectiveness and safety of ambrisentan in IPF.…”

“…In the second article, BREWIS et al [15] report the experience of a PH reference centre on the characteristics and response to treatment of patients with severe PH and coexistent respiratory disease, which were compared with idiopathic pulmonary arterial hypertension (PAH). Quite surprisingly, during the 14-year evaluation period, there were more incident cases with concomitant respiratory disease than patients with idiopathic PAH.…”

“…Sometimes, in patients with mild ventilatory impairment and severe PH, the distinction between group 3 and group 1 PH is elusive and represents a diagnostic dilemma [6]. In the series reported by BREWIS et al [15], patients with concomitant lung diseases were older, more frequently male and smokers, and had mild ventilatory impairment, severe hypoxaemia, and markedly reduced diffusing capacity of the lung for carbon monoxide, although with a lower haemodynamic impairment as compared with idiopathic PAH. This suggests that patients with PH presenting this phenotype should undergo a high-resolution computed tomography scan to rule out concomitant parenchymal lung disease.…”

“…Despite the absence of supportive evidence, patients with PH and concomitant respiratory disease often receive targeted PAH therapy [20,21]. In the study by BREWIS et al [15], almost all patients with PH and lung disease were treated with PAH drugs, although they did not show improvement in either functional class or 6-min walking distance, and only NT-pro-brain natriuretic peptide improved significantly. In contrast, patients with idiopathic PAH showed significant improvements in all three assessments after treatment.…”

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

“…On the other hand, a decrease in NT-proBNP blood concentration was noted at the time of clinical improvement during the first months of sildenafil therapy. The role of NT-proBNP in SAPH diagnosing and monitoring is still under debate [25], but papers published lately documented high NT-proBNP serum concentration in patients with severe PH in the course of other ILD [26,27] and a significant decrease of the marker in the course of PAH-specific treatment [22].…”