P4131Abnormal flow pattern in the main pulmonary artery of Marfan patients is related to local dilation
Authors: Guala A , Izagirre N , Ruiz Munoz A , Dux-Santoy L , Madrenas L , Gandara M , Granato C , Valente F , Gutierrez L , Galian L , Servato L , La Mura L , Evangelista A , Rodriguez-Palomares J F , Teixido Tura G 
Published: October 2019

Introduction

Marfan syndrome (MFS) is a hereditary connective tissue disorder caused by mutation in the FBN1 gene. Main pulmonary artery (MPA) dilation is very prevalent in MFS patients. Indeed, the old Ghent nosology considered main pulmonary artery (MPA) dilation as diagnostic criterion of MFS patients. Although clinical complications related to pulmonary dilation in MFS are rare, this may potentially lead to MPA dissection or be a marker of vascular disease in MFS. Studies regarding potential causes of MPA dilation in MFS patients are very scarce.

Purpose

Through 4D flow CMR, we aimed to assess whether flow abnormalities exist in the MPA of MFS patients and their relation to local diameter.

Methods

Fifty-five consecutive Marfan syndrome adults (MFS) and 22 healthy volunteers (HV) were prospectively enrolled. All subjects underwent non-contrast-enhanced 4D flow-MRI, obtaining 4D flow field and a 3D angiography. The MPA was segmented from the 3D angiography, and the segmentation was used to mask 4D velocity data. Four, equidistant analysis planes were placed in the MPA between the pulmonary valve and the pulmonary artery bifurcation. Common descriptors of large arteries hemodynamics were computed at each plane: maximum velocity, systolic flow reversal ratio (a descriptor of the amount of systolic backward flow) and circumferentially-averaged axial and circumferential wall shear stress (WSS). Pulmonary artery diameters were measured on axial images. MPA dilation was defined as a diameter larger than 27 mm in women and 29 mm in men. Systolic (SBP) and diastolic (DBP) systemic blood pressure were measured at the brachial artery with a calibrated cuff immediately after the scan.

Results

Compared with HV, MFS patients presented similar age, BSA, SBP and maximum blood velocity, but had larger MPA diameter (27.8 vs 25.1 mm, p<0.001) and higher DBP (75.5 vs 66.8 mmHg, p=0.003). According to the used threshold, 45% (27) of MFS patients had MPA dilation. Compared with HV, Marfan patients presented an increased systolic flow reversal ratio in the proximal part of the MPA (Figure 1). In MFS patients axial WSS was reduced in central sections of the MPA, while the circumferential component was not difference with respect to HV. All these flow abnormalities were also present in the subset of 28 MFS patients without pulmonary artery dilation. In multivariable analysis, MPA diameter was independently related to age (B=0.056; p=0.032), sex (B=−2.3; p=0.02) and axial (B=6.4; p=0.039) and circumferential (B=33.9; p<0.001) WSS.

Figure 1

Conclusions

Dilation of the main pulmonary artery is prevalent in Marfan syndrome patients. Abnormal increase in systolic vortexes and reduction in axial WSS were present in dilated and non-dilated MPA in MFS patients. Axial and circumferential WSS were independently related to MPA diameter. The eventual predictive role of abnormal pulmonary flow pattern in pulmonary artery dilation in MFS patients remain to be established

Acknowledgement/Funding

Instituto de Salud Carlos III (PI14/0106), La Maratό de TV3 (20151330), CIBERCV and FP7/People (267128)