Abstract
Pulmonary arterial hypertension (PAH) is a rare disease caused by pulmonary vascular remodeling. Current vasodilator treatments have substantially improved patients’ survival. This improved survival has led to the appearance of complications related to conditions previously underdiagnosed or even ignored, such as pulmonary artery aneurysm (PAA). The presence of a dilated pulmonary artery has been shown to be related to an increased risk of sudden cardiac death among PAH patients. This increased risk could be associated to the development of left main coronary artery compression or pulmonary artery dissection. Nevertheless, very little is currently known about the molecular mechanisms related to PAA. Thoracic aortic aneurysm (TAA) is a well-known condition with an increased risk of sudden death caused by acute aortic dissection. TAA may be secondary to chronic exposure to classic cardiovascular risk factors. In addition, a number of genetic variants have been shown to be related to a marked risk of TAA and dissection as part of multisystemic syndromes or isolated familial TAA. The molecular pathways implied in the development of TAA have been widely studied and described. Many of these molecular pathways are involved in the pathogenesis of PAH and could be involved in PAA. This review aims to describe all these common pathways to open new research lines that could help lead to a better understanding of the pathophysiology of PAH and PAA and their clinical implications.
Highlights
Pulmonary artery (PA) dilatation is a common finding among pulmonary arterial hypertension (PAH) patients [1]
In this review we aim to summarize these common molecular pathways to help researchers identify potential molecular targets that could be involved in the development of Pulmonary artery aneurysm (PAA) among PAH patients
The hereditary influence that causes or predisposes a person to systemic artery dilation can be divided among (i) those carrying a highly penetrant variant and frequently showing syndromic features (e.g., FBN and Marfan syndrome, transforming growth factor β (TGF-β) and Loeys–Dietz syndromes or collagen and Ehlers–Danlos syndrome (Table 1)); (ii) those with less penetrant and perhaps more frequent variants in whom environmental influence might be key to aneurysm development; and (iii) a group of patients not carrying a net hereditary risk charge for aneurysm development who need a heavy environmental influence for aneurysm development [23]
Summary
Pulmonary artery (PA) dilatation is a common finding among pulmonary arterial hypertension (PAH) patients [1]. Some studies relate PAA development to hemodynamic severity among PAH patients [5] This finding is controversial since other published works have shown that a worse hemodynamic profile does not necessarily lead to PAA development [1]. It seems that PA pressure reduction with pulmonary vasodilators is not able to stop PA enlargement in PAH patients [2,7]. There are several acquired conditions leading to this abnormal growth including classical cardiovascular risk factors, vasculitis, or trauma [15] In this regard, genetics plays an important role. In this review we aim to summarize these common molecular pathways to help researchers identify potential molecular targets that could be involved in the development of PAA among PAH patients
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