Congenital heart disease
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Congenital heart disease · Jan 2010
Multicenter StudyThree-stage palliation of hypoplastic left heart syndrome in the University HealthSystem Consortium.
The objective of this study was to describe current management and short-term outcomes for patients with hypoplastic left heart syndrome representative of nationwide experience. Additionally, this study identifies risk factors associated with mortality in patients undergoing staged surgical palliation. ⋯ First-stage palliative mortality for hypoplastic left heart syndrome fell dramatically over the past decade, while that for second- and third-stage procedures remained stable. The cumulative operative mortality for three-staged repair of hypoplastic left heart syndrome was 39% over the decade, but fell to 24% for procedures in 2007.
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Congenital heart disease · Jan 2010
Case ReportsPerventricular closure of a large ventricular septal defect in congenitally corrected transposition of the great arteries.
We report the case of a 30 year-old male with congenitally corrected transposition of the great arteries, atrial, and ventricular septal defects (VSD), and pulmonary stenosis. He previously underwent three palliative surgical procedures before undergoing intracardiac repair at age 20 with a left ventricular to pulmonary artery (LV-PA) conduit, VSD closure, and replacement of the systemic atrioventricular valve. A residual VSD was noted postoperatively. ⋯ Following deployment, the residual shunt was small and the inferior vena cava-to-pulmonary artery saturation step-up was only 4%. The left ventricular systolic pressure decreased to one half systemic. This case highlights the utility and efficacy of a hybrid approach in the treatment of complex congenital heart disease.
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Congenital heart disease · Jan 2010
Adults with Down syndrome: safety and long-term outcome of cardiac operation.
As limited data exist, we sought to review the safety and outcome of cardiac surgery in the adult Down syndrome population. ⋯ At an experienced center, adult patients with Down syndrome can undergo cardiac surgery with a low risk of mortality and acceptable morbidity. Atrial arrhythmias and pulmonary infections are common postoperatively.
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Congenital heart disease · Jan 2010
Rehabilitation program in adult congenital heart disease patients with pulmonary hypertension.
It is commonly believed that physical activity may have a negative impact on pulmonary hypertension patients. The object of this study is to determine the tolerability of a directed exercise program in congenital heart disease patients with pulmonary hypertension. ⋯ Cardiopulmonary rehabilitation appears to be a safe intervention in patients with congenital heart disease and pulmonary hypertension.
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Congenital heart disease · Jan 2010
Case ReportsHypoplastic right lung associated with right pulmonary vein stenosis and systemic collateral.
We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidetector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities.