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Multi-district coronary tree involvement in a 17-year-old girl with Williams-Beuren syndrome.

Serena T, Valerio E, Castaldi B, Reffo E, Milanesi O - Springerplus (2015)

Bottom Line: Clinical examination, electrocardiogram, and echocardiography were compatible with Williams-Beuren syndrome; such condition was later confirmed by genetic analysis.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.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.

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.

No MeSH data available.


Related in: MedlinePlus

MRI 3D aortic arch reconstruction showing severe hypoplasia and multiple aneurysms of ascending aorta, extending till first brachiocephalic artery; sinotubular junction is also severely stenotic
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Fig3: MRI 3D aortic arch reconstruction showing severe hypoplasia and multiple aneurysms of ascending aorta, extending till first brachiocephalic artery; sinotubular junction is also severely stenotic

Mentions: Cardiac magnetic resonance imaging (MRI) confirmed transmural fibrosis of the apex (Fig. 1); left ventricular ejection fraction (LVEF) was 29 %. Aortic sinotubular junction was severely stenotic, also with involvement of left and right coronary ostia; beyond ostial region, the whole coronary artery tree was affected by multiple stenosis and aneurysmatic tracts (Fig. 2). Ascending aorta proved hypoplastic; aortic arch was affected by post-stenotic dilation and multiple aneurysms (Fig. 3). On the contrary, pulmonary artery tree showed no significant abnormalities.Fig. 1


Multi-district coronary tree involvement in a 17-year-old girl with Williams-Beuren syndrome.

Serena T, Valerio E, Castaldi B, Reffo E, Milanesi O - Springerplus (2015)

MRI 3D aortic arch reconstruction showing severe hypoplasia and multiple aneurysms of ascending aorta, extending till first brachiocephalic artery; sinotubular junction is also severely stenotic
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4542862&req=5

Fig3: MRI 3D aortic arch reconstruction showing severe hypoplasia and multiple aneurysms of ascending aorta, extending till first brachiocephalic artery; sinotubular junction is also severely stenotic
Mentions: Cardiac magnetic resonance imaging (MRI) confirmed transmural fibrosis of the apex (Fig. 1); left ventricular ejection fraction (LVEF) was 29 %. Aortic sinotubular junction was severely stenotic, also with involvement of left and right coronary ostia; beyond ostial region, the whole coronary artery tree was affected by multiple stenosis and aneurysmatic tracts (Fig. 2). Ascending aorta proved hypoplastic; aortic arch was affected by post-stenotic dilation and multiple aneurysms (Fig. 3). On the contrary, pulmonary artery tree showed no significant abnormalities.Fig. 1

Bottom Line: Clinical examination, electrocardiogram, and echocardiography were compatible with Williams-Beuren syndrome; such condition was later confirmed by genetic analysis.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.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Cardiology Unit, Department of Woman and Child Health, Medical School, University of Padua, Via Giustiniani, 3, 35128 Padua, Italy.

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.

No MeSH data available.


Related in: MedlinePlus