Pediatrics international : official journal of the Japan Pediatric Society
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Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. ⋯ In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.
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Case Reports
Magnetic resonance tagging for diagnosis of focal hypertrophic cardiomyopathy in a child.
Magnetic resonance imaging has become an important diagnostic tool in the differential diagnosis of lesions for evaluation of cardiovascular disorders. In magnetic resonance tagging (MRt), tissue elements are magnetically labeled so that their positions can be tracked as a function of time. Thus, MRt evaluates heart wall motion both qualitatively and quantitatively. ⋯ MRt indicated active displacement and deformation of the tags at the level of the hypertrophic myocardium during systole, as with normal myocardium. Thus, the tagged images supported the diagnosis of focal hypertrophic cardiomyopathy (HCM). In view of these results, MRt should be considered as a useful technique for differentiating between a mass-like focal lesion such as neoplasm and HCM.