title: Multi-district coronary tree involvement in a 17-year-old girl with Williams-Beuren syndrome.
abstract: We describe a case of 17-year-old Chinese girl referred to our Pediatric Cardiology Unit for asthenia, reduced exercise tolerance, and dyspnea. Past medical history was relevant for multiple chest pain episodes in childhood and several syncopal episodes, for which the patient had been never evaluated. Clinical examination, electrocardiogram, and echocardiography were compatible with Williams-Beuren syndrome; such condition was later confirmed by genetic analysis. Cardiac magnetic resonance imaging showed transmural fibrosis of the apex with impaired left ventricular ejection fraction (29 %), severe stenosis of aortic sinotubular junction with left and right coronary ostia involvement; more importantly, the whole coronary artery tree beyond ostia was affected by multiple stenosis and aneurysmatic tracts. Ascending aorta proved hypoplastic, with post-stenotic dilation and multiple aneurysms. At the end of the diagnostic process, surgical risk was considered too high to proceed with the correction. The presented case is of educational value since it provides good iconographical illustration of diffuse, multiple-site coronary artery tree involvement, a rather rare co-morbidity in Williams-Beuren syndrome.
authors: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.
authors: Tiziana
authors: T
authors: Serena
authors: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.
authors: Enrico
authors: E
authors: Valerio
authors: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.
authors: Biagio
authors: B
authors: Castaldi
authors: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.
authors: Elena
authors: E
authors: Reffo
authors: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.
authors: Ornella
authors: O
authors: Milanesi
keywords: Multi-district coronary artery disease; Myocardial infarction; Pediatric cardiology; Supravalvular aortic stenosis; Williams–Beuren syndrome