Congenital heart disease
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Congenital heart disease · May 2014
Review Case ReportsSevere left main coronary artery stenosis with abnormal branching pattern in a patient with mild supravalvar aortic stenosis and Williams-Beuren syndrome.
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. ⋯ 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.
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Congenital heart disease · May 2014
Validation of the Cardiac Children's Hospital Early Warning Score: an early warning scoring tool to prevent cardiopulmonary arrests in children with heart disease.
Most inpatient pediatric arrests are preventable by early recognition/treatment of deterioration. Children with cardiac disease have the highest arrest rates; however, early warning scoring systems have not been validated in this population. The objective of this study was to validate the Cardiac Children's Hospital Early Warning Score (C-CHEWS) tool in inpatient pediatric cardiac patients. The associated escalation of care algorithm directs: routine care (score 0-2), increased assessment/intervention (3-4), or cardiac intensive care unit (CICU) consult/transfer (≥5). ⋯ C-CHEWS has excellent discrimination to identify deterioration in children with cardiac disease and performed significantly better than PEWS both as an ordinal variable and when choosing cut points to maximize AUROC. C-CHEWS has a higher sensitivity than PEWS at all cut points.
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Congenital heart disease · May 2014
Declining cardiopulmonary exercise capacity is not associated with worsening systolic systemic ventricular dysfunction in adults with transposition of great arteries after atrial switch operation.
Cardiopulmonary exercise capacity is often reduced in patients with transposition of the great arteries after atrial switch operation. Reduced exercise capacity may be caused by deterioration of systemic right ventricular function over time. This study analyzed serial changes in systemic right ventricular function and cardiopulmonary exercise capacity in young adults with transposition of the great arteries after atrial redirection surgery. ⋯ After atrial switch operation for transposition of the great arteries we observed a declining functional exercise capacity. This was not associated with worsening systemic right ventricular function, suggesting that other factors are contributing to the decline in physical exercise capacity.
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Congenital heart disease · May 2014
Cardiac resource utilization in adults at a freestanding children's hospital.
This article aims to give a comprehensive description of cardiac resource utilization in adults at a freestanding children's hospital. ⋯ A significant and growing number of adult patients are currently being cared for at pediatric institutions. Excellent outcomes have been achieved at these centers with dedicated adult congenital heart disease services, consisting of both cardiologists and other faculty trained in adult medicine. Others with training in adult medicine, whether they be staff or contracted consulting services, are also required to help manage adult comorbidities.