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Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.
Cardiovascular magnetic resonance assessment of adults late after an atrial redirection operation for transposition is demanding and time consuming. We hypothesised that the relatively fast and standardised 3-dimensional time-resolved contrast-enhanced magnetic resonance angiography, or dynamic angiography, would be valuable in the periodic follow-up of these patients.
We investigated prospectively 36 adults with transposition using dynamic angiography, comparing our results against a comprehensive but non-contrast cardiovascular magnetic resonance protocol. We acquired 6 dynamic angiographic datasets after injection of contrast. The primary aim was to detect significant obstruction of the pathways for venous flow.
In 4 patients (11%), we found evidence of moderate-to-severe, and thus clinically important, obstruction of systemic venous channels on standard cardiovascular magnetic resonance. All these patients were correctly identified by dynamic angiography. In 4 additional patients, we found mild and haemodynamically insignificant obstructions in the systemic venous channels. Of the 8 (22%) patients with any obstruction, 6 were detected by angiography. There were no false positives reported, giving sensitivity of 75% and specificity of 100%, a positive predictive value of 100%, and negative predictive value of 93%. In 1 patient, there was a moderate obstruction of the pulmonary venous compartment which was not readily seen by dynamic angiography.
3-dimensional dynamic angiography is a useful method for detecting anatomically moderate-to-severe, but not mild, obstructions in the systemic venous channels following Mustard repair for transposition. This technique can be used as a single imaging method and/or as complimentary to standard two dimensional cardiovascular magnetic resonance techniques for detection of clinically important obstructions in the systemic venous channels.