| Literature DB >> 30793562 |
A Radermecker1, L Stiennon1, A Leroux2, M Sooknunden1, B Duysinx3, J Guiot4, L Davin5, N Sakalihasan1, M A Radermecker6, J O Defraigne1.
Abstract
Pulmonary artery aneurysm is a rare and multiform pathology related to multiple etiologies and therefore different pathophysiological mechanisms. Delineating homogenous sub-groups is a pre-requisite to refine medico-surgical management. The case of a giant PAA without pulmonary hypertension but associated to a dysplastic pulmonary valve is reported. This association could be in some instances the result of a congenital anomaly in the development of both the pulmonary valve and the root creating the conditions for further development of a pulmonary artery aneurysm. Whilst minor forms are usually asymptomatic, they can lead to lethal complications in huge sizes and are frequently associated via pulmonary valve insufficiency to right ventricular dysfunction. This specific association is discussed and a diagnostic algorithm for nosologic classification and management is proposed.Entities:
Keywords: Aneurysm ; Dysplastic pulmonary valve; Pulmonary artery
Mesh:
Year: 2019 PMID: 30793562
Source DB: PubMed Journal: Rev Med Liege ISSN: 0370-629X