scholarly journals Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta

2020 ◽  
Vol 21 (7) ◽  
pp. 2509 ◽  
Author(s):  
Jorge Nuche ◽  
Julián Palomino-Doza ◽  
Fernando Arribas Ynsaurriaga ◽  
Juan F. Delgado ◽  
Borja Ibáñez ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Molecular Mechanisms ◽  
Artery Aneurysm ◽  
Artery Dissection ◽  
Molecular Pathways ◽  
Pulmonary Artery Aneurysm ◽  
Increased Risk ◽  
Pulmonary Arterial ◽  
Aneurysm Development ◽  
New Research

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.

Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development

2020 ◽  
Vol 35 (9) ◽  
pp. 1290-1298
Author(s):  
Jorge Nuche ◽  
José Manuel Montero-Cabezas ◽  
Ana Lareo ◽  
Sergio Huertas ◽  
Carmen Jiménez López-Guarch ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Artery Aneurysm ◽  
Pulmonary Artery Aneurysm ◽  
Pulmonary Arterial ◽  
Aneurysm Development

Pulmonary artery aneurysm mimicking pulmonary artery dissection detected by multimodality imaging

2013 ◽  
pp. 1180-1182
Author(s):  
Murat Unlu ◽  
Sait Demirkol ◽  
Sevket Balta ◽  
Ugur Bozlar ◽  
Ugur Kucuk
Keyword(s):  
Pulmonary Artery ◽  
Multimodality Imaging ◽  
Artery Aneurysm ◽  
Artery Dissection ◽  
Pulmonary Artery Aneurysm

scholarly journals Usefulness of transthoracic echocardiography for pulmonary artery aneurysm screening in patients with pulmonary arterial hypertension

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M Guisasola Cienfuegos ◽  
J Nuche ◽  
A Lareo ◽  
S Alonso ◽  
F Arribas-Ynsaurriaga ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Transthoracic Echocardiography ◽  
Area Under The Curve ◽  
Artery Aneurysm ◽  
Pulmonary Artery Aneurysm ◽  
Diameter Measurement ◽  
Pulmonary Arterial

Abstract Background/Introduction Pulmonary artery aneurysm (PAA), defined as a pulmonary artery (PA) diameter >40 mm, is a common finding among pulmonary arterial hypertension (PAH) patients. Although often asymptomatic, PAA may lead to life-threatening complications such as left main coronary artery compression or PA dissection. Transthoracic echocardiography (TTE) is regularly employed for risk assessment in PAH patients. However, TTE accuracy for PA measurement has not been evaluated, and current practice guidelines lack formal recommendations for PAA screening and follow-up. We aim to determine whether TTE is an appropriate tool for PA diameter measurement and determine an optimal cut-off point to diagnose a PAA through TTE. Methods We retrospectively analyzed a cohort of 657 PAH patients followed up at a national referral centre. For this analysis, we selected those patients who had undergone at least one TTE and one computed tomography (CT) or magnetic resonance (MR) within six months before or after the TTE. We performed an agreement analysis between CT/MR-based and TTE-based PA diameter using the Passing–Bablok method. Furthermore, we calculated the area under the curve for the identification of a PAA with a TTE (compared to CT/MR). Results We analyzed 281 simultaneous CT/MR and TTE of a total of 178 PAH patients (71% women). Median age at diagnosis was 42.1 (32.2–58.0) years. PAH etiology was idiopathic or familial in 67 (38%), associated with congenital heart disease in 28 (16%) and associated with connective tissue disease in 36 (20%) patients. In 46 (26%) patients PAH was associated with other entities, such as human immunodeficiency virus, pulmonary veno-occlusive disease, drugs or portal hypertension. We found a significant correlation between PA diameter measured in TTE and CT/CMR (Lin's concordance correlation coefficient = 0.851) (Figure 1). The area under the curve for the detection of PAA was 0.91 (95% CI 0.88–0.95, p=0.018) (Figure 2). We selected a TTE-based PA diameter 37 mm as the optimal cut-off point for PAA identification. This diameter correctly classified 85.4% of measurements with a sensitivity and a specificity of 83.2% and 87.2%, respectively. Conclusion Our study demonstrates that TTE is an adequate tool for PA diameter quantification with a strong correlation with CT/MR. This good correlation makes TTE an excellent tool for PAA screening among PAH patients, avoiding unnecessary CT or MR scan and helping to identify those patients in whom close follow-up is advisable. Based on these results, we recommend the inclusion of PA diameter measurement in TTE acquisition protocols for PAH patients. FUNDunding Acknowledgement Type of funding sources: None. Figure 1. Passing–Bablok regression line Figure 2. ROC curve for PAA detection with TTE

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