Pulmonary hypertension in COPD: results from the ASPIRE registry

Abstract

The phenotype and outcome of severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is described in small numbers, and predictors of survival are unknown. Data was retrieved for 101 consecutive, treatment-naïve cases of pulmonary hypertension in COPD.Mean¡SD follow-up was 2.3¡1.9 years. 59 patients with COPD and severe pulmonary hypertension, defined by catheter mean pulmonary artery pressure o40 mmHg, had significantly lower carbon monoxide diffusion, less severe airflow obstruction but not significantly different emphysema scores on computed tomography compared to 42 patients with mild-moderate pulmonary hypertension. 1-and 3-year survival for severe pulmonary hypertension, at 70% and 33%, respectively, was inferior to 83% and 55%, respectively, for mild-moderate pulmonary hypertension. Mixed venous oxygen saturation, carbon monoxide diffusion, World Health Organization functional class and age, but not severity of airflow obstruction, were independent predictors of outcome. Compassionate treatment with targeted therapies in 43 patients with severe pulmonary hypertension was not associated with a survival benefit, although improvement in functional class and/or fall in pulmonary vascular resistance .20% following treatment identified patients with improved survival.Standard prognostic markers in COPD have limited value in patients with pulmonary hypertension. This study identifies variables that predict outcome in this phenotype. Despite poor prognosis, our data suggest that further evaluation of targeted therapies is warranted.

Pulmonary Vascular and Ventricular Dysfunction in the Susceptible Patient (2015 Grover Conference Series)

“…Thus, further understanding of the basic mechanisms by which hypoxia regulates vascular remodeling, particularly fibrosis, is required to identify novel treatments that improve on elevated rates of morbidity and mortality in at-risk patients. [149][150][151] Endothelial-mesenchymal transition (EndMT) is a principal mechanism implicated in the development of perivascular myofibroblast proliferation that evinces collagen deposition in PAs. 152 Under hypoxic conditions or in the setting of hypoxia-independent upregulation of HIF signaling, as is the case in PAH, 153 EndMT induces a complex sequence of molecular events characterized by an acquired loss of cell-cell contact membrane proteins, such as vascular endothelial cadherin and PECAM-1 (platelet endothelial cell adhesion molecule), which permits dissociation of pulmonary artery endothelial cells (PAECs) and their migration inward toward the blood vessel lumen.…”

Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease and Pulmonary Fibrosis: Prevalence and Hemodynamic Differences in Lung Transplant Recipients at Transplant Center's Referral Time

“…Alterations in vessel structure are very common, and abnormalities in their function impair gas exchange and result in PH [5]. Hurdman et al, in a recent article [6], have studied the characteristics and outcomes, in particular mortality, P.S. and F.P.…”

Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease: A Review for Definition, Epidemiology, Pathology, and Diagnosis

“…A total of 101 COPD patients who received a new PHT diagnosis as a subgroup of the ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Center) registry group (n=1344 patients) were followed for an average of 2.3 years [26]. In patients with severe PHT, one and three-year survival rates were reported as 70% and 33%, respectively, while it were 83% and 55% in patients with mild-to-moderate PHT, respectively.…”