Abstract:
:Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenoses of medium- and large-sized arteries including the coronary arteries. Cardiac manifestations are often the initial reason for referral and careful cardiovascular assessment is important as coronary artery involvement confers a significant anesthetic risk and may be associated with ischemia and resultant ventricular dysfunction. Here we review the literature and describe a 2-year-old boy with evolving clinical features of WBS. He presented to our pediatric cardiology department for a routine assessment of peripheral pulmonary branch stenosis. A 12-lead electrocardiogram showed changes consistent with left ventricular ischemia and a two-dimensional echocardiogram showed reduced left ventricular function and mild supravalvar aortic stenosis. Subsequent cardiac catheterization diagnosed severe left main coronary artery stenosis. Deteriorating ventricular function secondary to acute ischemia postcatheterization required intensive care treatment from which the patient did not recover. This case report highlights the necessity of careful cardiology assessment without delay in patients with a suspicion of WBS. Isolated coronary stenosis though rare in WBS should be considered in the presence of ischemia or reduced ventricular function. Larger case series are needed to further characterize the correlation between WBS and acute coronary events.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Pieles GE,Ofoe V,Morgan GJdoi
10.1111/chd.12087subject
Has Abstractpub_date
2014-05-01 00:00:00pages
E85-9issue
3eissn
1747-079Xissn
1747-0803journal_volume
9pub_type
杂志文章,评审abstract:OBJECTIVE:We studied, as a physiological benchmark, acute effects of right ventricular (RV) apical, RV outflow, and left ventricular (LV) pacing in children with normal cardiac function on LV and RV function and ventricular-ventricular interactions. DESIGN:The design of the study was a prospective, acute intervention....
journal_title:Congenital heart disease
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journal_title:Congenital heart disease
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pub_type: 杂志文章,meta分析,评审
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abstract:OBJECTIVE:The 2016 American Board of Pediatrics (ABP) content outline is comprehensive, including more than 50 cardiology-specific objectives within eight content areas. This study complements the quantitative analysis of a Kentucky-wide survey of trainees, pediatricians, and pediatric cardiologists asking them to iden...
journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
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journal_title:Congenital heart disease
pub_type: 杂志文章,评审
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pub_type: 杂志文章,多中心研究
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2019-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2006.00008.x
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
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更新日期:2019-03-01 00:00:00
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journal_title:Congenital heart disease
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更新日期:2013-07-01 00:00:00
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更新日期:2015-07-01 00:00:00
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pub_type: 杂志文章
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更新日期:2015-01-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2016-12-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12060
更新日期:2014-01-01 00:00:00
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journal_title:Congenital heart disease
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journal_title:Congenital heart disease
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journal_title:Congenital heart disease
pub_type: 杂志文章,评审
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更新日期:2014-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2018-01-01 00:00:00
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journal_title:Congenital heart disease
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00086.x
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pub_type: 杂志文章
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更新日期:2019-11-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00200.x
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