Untitled

Abstract

Axial SE MRI is a reliable and easy acquisition to measure pulmonary artery dimensions in patients with Marfan syndrome, and could be used for follow-up, especially in patients with severe involvement of the cardiovascular system. Not only the pulmonary artery trunk but also the asymmetric pulmonary root should be measured, although the clinical relevance of the asymmetric root is not yet known.

The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

“…The old Ghent nosology considered dilatation of the main pulmonary artery (MPA) as a diagnostic criterion of Marfan syndrome (MFS) [ 1 ] but data on the prevalence of MPA dilatation or aneurysm remained both scarce and conflicting in MFS [ 2 - 5 ]. Most likely, little attention has been paid to MPA because cardiovascular complications of MFS usually result only from rupture or dissection of the aortic root [ 6 ] or from ventricular arrhythmia [ 7 , 8 ].…”

“…As a consequence, there is no current data on the echocardiographic prevalence of MPA dilatation in MFS patients, and for this reason the revised Ghent nosology removed MPA dilatation from the list of diagnostic criteria [ 9 ]. Some case reports highlight that MPA dilatation may lead to severe complications in MFS [ 10 ], and current magnetic resonance imaging (MRI) and computed tomography (CT) studies suggest that pathology of the MPA is frequent in MFS [ 3 - 5 , 11 , 12 ]. Hence, echocardiographic examination of the MPA may be important in the evaluation of adults with MFS to further elucidate the diagnostic and prognostic implications of MPA dilatation.…”

“…Normative MPA-d and MRA-r values on CT and MRI do not apply to transthoracic echocardiography for various reasons. First, echocardiographic MPA-d and aortic root diameters are measured at other sites of the vessel than on CT or MRI, which may infer substantial differences of measurements because of the asymmetric anatomy of both MPA and the aortic root [ 2 , 5 , 24 ]. Second, measurements on CT and MRI are usually obtained as outer diameters of the vessel rather than with the leading-edge method [ 24 - 26 ].…”

“…MPA aneurysms may develop in patients with heritable connective tissue diseases such as Loeys-Dietz syndrome [ 46 , 47 ], and arterial tortuosity syndrome [ 48 ], but it is most typically observed in MFS [ 2 - 5 , 49 - 54 ]. MFS is an autosomal dominantly inherited disease with a phenotype that involves multiple organ systems and is caused by mutations in the gene coding for fibrillin-1, FBN1 .…”