Abnormal Cardiac Magnetic Resonance-Derived Ascending Aortic Area Strain Demonstrates Altered Ventriculo-Vascular Function in Marfan Syndrome

被引：0

作者：

Jacquemyn, Xander ^{[1
,2
]}

Cordrey, Kyla ^{[1
]}

van den Eynde, Jef ^{[1
,2
]}

Guerrerio, Anthony L. ^{[3
]}

MacCarrick, Gretchen ^{[4
,5
]}

Dietz, Hal C. ^{[6
]}

Kutty, Shelby ^{[1
]}

机构：

[1] Johns Hopkins Univ, Blalock Taussig Thomas Heart Ctr, Sch Med, Dept Pediat, M2315,1800 Orleans St, Baltimore, MD 21287 USA

[2] Johns Hopkins Univ, Sch Med, Dept Pediat, Baltimore, MD USA

[3] Johns Hopkins Univ, Sch Med, McKusick Nathans Inst Genet Med, Baltimore, MD USA

[4] Johns Hopkins Univ, Howard Hughes Med Inst, Baltimore, MD USA

[5] Johns Hopkins Univ, Sch Med, Dept Genet Med, Baltimore, MD USA

[6] Katholieke Univ Leuven, Dept Cardiovasc Sci, Leuven, Belgium

来源：

JOURNAL OF THORACIC IMAGING | 2024年 / 39卷 / 05期

关键词：

aortic stiffness; cardiovascular magnetic resonance imaging; Marfan syndrome; left ventricular dysfunction; aortic root replacement; CONNECTIVE-TISSUE DISORDERS; BIOMECHANICAL PROPERTIES; YOUNG-ADULTS; STIFFNESS; ROOT; ANEURYSMS; CHILDREN; SIZE; AGE;

D O I：

10.1097/RTI.0000000000000784

中图分类号：

R8 [特种医学]; R445 [影像诊断学];

学科分类号：

1002 ; 100207 ; 1009 ;

摘要：

Purpose:There remains a need for improved imaging markers for risk stratification and treatment guidance in Marfan syndrome (MFS). After aortic root replacement (ARR), vascular remodeling and progressive aneurysm formation can occur due to alterations in up- and downstream wall biomechanics and hemodynamics. We aim to compare the ventriculo-vascular properties of patients with MFS with controls, and investigate the correlation between ascending aortic area strain and descending aortic area strain (DAAS) with other clinical variables. Patients and Methods:Nineteen patients with MFS (47% males), including 6 with ARR were studied. In 26 studies, aortic area strain was measured using cross-sectional cardiac magnetic resonance images at the ascending and proximal descending aortic levels. Left atrial, left ventricular longitudinal, and left ventricle circumferential strain (left atrial longitudinal strain, left ventricular longitudinal strain, and left ventricular circumferential strain, respectively) were measured using cardiac magnetic resonance-feature tracking. Results:Compared with healthy controls, patients with MFS had significantly impaired left ventricular longitudinal strain and left ventricular circumferential strain (-15.8 +/- 4.7 vs -19.7 +/- 4.8, P = 0.005, and -17.7 +/- 4.0 vs -27.0 +/- 4.1, P < 0.001). Left atrial longitudinal strain was comparable between patients with MFS and controls. AAAS was significantly reduced (19.0 [11.9, 23.7] vs 46.1 +/- 11.3, P < 0.001), whereas DAAS was not significantly decreased. AAAS and DAAS were negatively correlated with age, whereas no significant associations were identified with left ventricle function indices. No significant differences were observed between the ventriculo-vascular properties of patients with MFS who underwent ARR and those who did not. Conclusion:Patients with MFS demonstrated impaired ventricular and vascular function compared with healthy controls. Further investigations are warranted to determine clinical utility of aortic stiffness indices for predicting primary and repeat aortic events.

引用

页码：319 / 327

页数：9