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To compare the measurement of aortic diameters using a novel flow-independent MR-Angiography (3D modified Relaxation-Enhanced Angiography without Contrast and Triggering (modified REACT)) and transthoracic echocardiography (TTE) in Marfan syndrome (MFS) patients. This retrospective, single-center analysis included 46 examinations of 32 MFS patients (mean age 37.5 ± 11.3 years, 17 women, no prior aortic surgery) who received TTE and 3D modified REACT (ECG- and respiratory-triggering, Compressed SENSE factor 9 for acceleration of image acquisition) of the thoracic aorta. Aortic diameters (sinus of Valsalva (SV), sinotubular junction (STJ), and ascending aorta (AoA)) were independently measured by two cardiologists in TTE (leading-edge) and two radiologists in modified REACT (inner-edge, using multiplanar reconstruction). Intraclass correlation coefficient, Bland-Altman analyses, and Pearson's correlation (r) were used to assess agreement between observers and methods. Interobserver correlation at the SV, STJ, and AoA were excellent for both, TTE (ICC = 0.95–0.98) and modified REACT (ICC = 0.99–1.00). There was no significant difference between TTE and modified REACT for diameters measured at the SV (39.24 ± 3.24 mm vs. 39.63 ± 3.76 mm; p = 0.26; r = 0.78) and the STJ (35.16 ± 4.47 mm vs. 35.37 ± 4.74 mm; p = 0.552; r = 0.87). AoA diameters determined by TTE were larger than in modified REACT (34.29 ± 5.31 mm vs. 30.65 ± 5.64 mm; p < 0.01; r = 0.74). The mean scan time of modified REACT was 05:06 min ± 02:47 min, depending on the patient's breathing frequency and heart rate. Both TTE and modified REACT showed a strong correlation for all aortic levels; however, at the AoA, diameters were larger using TTE, mostly due to the limited field of view of the latter with measurements being closer to the aortic valve. Given the excellent interobserver correlation and the strong agreement with TTE, modified REACT represents an attractive method to depict the thoracic aorta in MFS patients. • TTE and modified REACT showed excellent interobserver reliability for measurements of the proximal thoracic aorta. • There was a strong correlation for all levels of measurement between TTE and modified REACT. At the SV and STJ there was no significant difference of measured diameters between both techniques whereas at the AoA, diameters were significantly larger using TTE. • Given the high agreement between both techniques, TTE and modified REACT can be used for surveillance of aortic diameters in MFS. [ABSTRACT FROM AUTHOR]

William's syndrome is a genetic disorder that is associated with a spectrum of cardiovascular disease: congenital heart disease, vascular disease, coronary artery disease, hypertension and arrhythmias. Patients may be at risk of significant morbidity and mortality over their lifetime including a risk of sudden cardiac arrest. Williams syndrome patients may develop progressive multi-site arterial stenosis, systemic hypertension, valve dysplasia, and arrhythmias with advancing age. They should always be considered at risk of having underlying coronary disease prior to anesthesia. Standard screening includes a comprehensive physical exam, electrocardiogram, and echocardiogram; however advanced cardiac imaging with computed tomography and/or magnetic resonance angiography may be beneficial to fully assess the vascular tree. We will review a systematic approach for clinicians caring for Williams syndrome patients to use for the detection of overt and subclinical forms of cardiovascular disease, especially prior to procedures requiring general anesthesia. • Williams syndrome comprehensive cardiovascular screening • Advanced cardiac imaging is beneficial • Williams syndrome patients who are undergoing anesthesia should be assumed to have risk of coronary disease. [ABSTRACT FROM AUTHOR]