Congenital heart disease
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Congenital heart disease · Jul 2014
Comparative StudyRight ventricular volumes assessed by echocardiographic three-dimensional knowledge-based reconstruction compared with magnetic resonance imaging in a clinical setting.
A technique that uses two-dimensional images to create a knowledge-based, three-dimensional model was tested and compared to magnetic resonance imaging. ⋯ The knowledge-based three-dimensional right ventricular volume method was highly feasible. Intra and interobserver variabilities were satisfactory. Agreement with magnetic resonance imaging measurements for volumes was reasonable but unsatisfactory for ejection fraction. Knowledge-based reconstruction may replace magnetic resonance imaging measurements for serial follow-up, whereas magnetic resonance imaging should be used for surgical decision making.
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Congenital heart disease · Jul 2014
Case ReportsCoronary sinus obstruction after atrioventricular canal defect repair.
The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.
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Congenital heart disease · Jul 2014
The significance of transesophageal echocardiography in assessing congenital heart disease: our experience.
The rapid expansion of pediatric cardiovascular surgery, along with the increase in the number of centers and operations, has also increased the use of intraoperative transesophageal echocardiography (TEE). Preoperative TEE allows for the confirmation of diagnoses established by transthoracic echocardiography and angiography, and also identifies possible additional pathologic conditions. TEE is also used to follow up on surgical repairs, determine the need for reintervention, and evaluate myocardial performance and the need for inotropic treatment. Our intention is to share the TEE findings before and after intervention for congenital heart disease in patients who received operations at our center, and the conclusions reached. ⋯ The use of intraoperative TEE in surgical centers for congenital heart disease allows for a significant reduction in mortality and morbidity. Intraoperative TEE performed by experienced pediatric cardiologists is therefore an absolute necessity.
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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.