Congenital heart disease
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Congenital heart disease · Nov 2009
Case ReportsLeft atrial appendage aneurysm-a rare anomaly with an atypical presentation.
Left atrial appendage aneurysm (LAAA) is a rare condition caused by congenital dysplasia of the atrial muscles. Patients usually present with atrial tachyarrhythmias as a result of ectopic foci of atrial rhythm generation or systemic thromboembolism. ⋯ This structure was later confirmed as a LAAA on cardiac magnetic resonance imaging. Patient underwent aneurysmectomy without any complications.
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Congenital heart disease · Sep 2009
ReviewThe spectrum of long-term electrophysiologic abnormalities in patients with univentricular hearts.
Patients with univentricular hearts experience a wide range of electrophysiolgic abnormalities which tend to develop years after cardiovascular surgical interventions. Intra-atrial reentrant tachycardia (atrial flutter) in the Fontan population is the most common arrhythmia and, as such, has the largest body of literature addressing its cause and treatment. However, sinus node dysfunction, other atrial arrhythmias, ventricular arrhythmias, and cardiac dysynchrony also occur in this patient population. The purpose of this article is to review the prevalence, mechanisms, and treatment of these electrophysiologic abnormalities within the single ventricle and Fontan patient.
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This report describes the history of a 41-year-old female patient born with atresia of the right atrioventricular valve, hypoplasia of the right ventricle, an atrial septal defect, and malposed great arteries. The patient underwent surgical pulmonary artery banding in infancy but did not undergo corrective surgery due to the development of pulmonary arterial hypertension. This report touches on several important clinical issues that may arise when caring for the adult with complex palliated cyanotic congenital heart disease, including the development and treatment of pulmonary arterial hypertension.
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Congenital heart disease · Sep 2009
Development of a pediatric cardiology quality improvement collaborative: from inception to implementation. From the Joint Council on Congenital Heart Disease Quality Improvement Task Force.
While clinical outcomes in pediatric cardiac disease have improved in recent years, marked institutional and individual cardiology practice variability exists. Quality improvement science has demonstrated that reducing process variation leads to more favorable outcomes, safer practices, cost savings, and improved operating efficiency. This report describes the process undertaken to develop the first collaborative quality improvement project of the Joint Council on Congenital Heart Disease. ⋯ It is anticipated that this project will add to our understanding of care for this challenging group of children with hypoplastic left heart syndrome, identifying clinical care changes with the potential to lead to improvements in outcome. It will also introduce the field of pediatric cardiology to the science of collaborative quality improvement and assist in reducing clinical process variation and improving patient outcomes across centers. Finally, it will establish an ongoing network of pediatric cardiologists and their teams linked through a longitudinal data set and collaboration for improvement and research.
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Congenital heart disease · May 2009
Case ReportsAsymptomatic congenital atresia of the left inferior pulmonary vein: a case report.
We present an unusual fortuitously discovered case of atresia of the left inferior pulmonary vein with drainage via anastomosis to the left upper pulmonary vein and the left atrium. Atresia of the pulmonary veins is a very rare anomaly that results from defective incorporation of the pulmonary venous system into the left atrium. The initial diagnosis, drawn from plain radiographs, was confirmed by cardiac catheterization and pulmonary angiography. The appearance of clinical signs, prognosis, and the need for treatment depend on how well substitute drainage is performed.