Pulmonary Arterial Dilatation in Pulmonary Hypertension: Prevalence and Prognostic Relevance

Abstract

Objectives: Pulmonary arterial dilatation is considered a consequence of chronic pulmonary hypertension (PH), but despite its relatively common detection, its prevalence and prognostic impact have not yet been systematically investigated. The aim of the study was to investigate these factors in a relatively large cohort of severe PH patients. Methods: One hundred and forty-one consecutive patients diagnosed with PH were monitored for a mean of 957 days. Data including functional class, exercise capacity, invasive hemodynamics and pulmonary artery (PA) echo/CT scan measurement were performed and outcomes prospectively collected. Results: PA dilatation is a common feature, present in the 76.6% of cases in this cohort of severe PH patients. Survival at 1, 2 and 3 years was 83, 71 and 58%, respectively. On univariate analysis, the baseline variables associated with a poor outcome were related to pulmonary arterial hypertension associated with connective tissue disease (CDT-PAH), New York Heart Association (NYHA) functional class, 6-min walk test and right atrial pressure. On multivariate analysis only CDT-PAH and NYHA functional class remained independently associated with poor survival. Conclusions: PA dilatation is commonly detected in severe PH patients and is not associated with an increased risk of death.

Aneurysm of the Pulmonary Artery, a Systematic Review and Critical Analysis of Current Literature

“…Pulmonary hypertension (PHT) is described as an important cause. 3,4 Others claim that the presence of arteriovenous communications are a major cause of PA aneurysms. 5,6 Post-stenotic dilatation and PA wall structural abnormalities, determined by genetic factors or infectious disease are also possible aetiologies.…”

Clinical Impact of Main Pulmonary Artery Dilatation on Outcome in Pediatric Idiopathic and Heritable Pulmonary Arterial Hypertension

“…Marked MPA dilatation is related to fragmentation of the elastic fibers, leading to PA remodeling and weakening of the vascular wall, and is associated with lower pulmonary compliance. 8, 9 Pulmonary compliance reflects the distensibility of the PA tree and is a component of right ventricular (RV) workload. Although normal pulmonary vessels have the capacity to dilate, PA capacity in severe PAH is diminished, leading to increased PAP and RV hypertrophy.…”

“…Structural changes of elastin and collagen in pulmonary vasculature eventually lead to PA dilatation. 8, 9 Additionally, altered pulmonary flow under high wall shear stress in the MPA may be associated with progressive PA dilatation. PAH is a potential risk factor of disease progression.…”

Unfavourable Effect of Pulmonary Arterial Dilatation in Pulmonary Hypertension

“…With regard to the paper by Badagliacca et al [1] about the prevalence and prognostic relevance of pulmonary arterial dilatation in pulmonary hypertension, we have the following comments.…”