Congenital heart disease
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Congenital heart disease · May 2012
Clinical TrialThe safety and effects of bosentan in patients with a Fontan circulation.
Adult patients with a Fontan circulation tend to have diminished exercise capacity. The principal objective of this study was to investigate the safety of the endothelin receptor antagonist bosentan in Fontan patients, and, secondarily, to assess effects on cardiovascular performance, New York Heart Association functional classification (NYHA FC), and ventricular function. ⋯ The small number of patients with a Fontan circulation in our study was able to tolerate bosentan for 6 months. The safety and tolerability of bosentan in a larger patient population remains unknown. The results presented here justify further investigation in larger studies.
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Congenital heart disease · May 2012
The utility of outpatient echocardiography for evaluation of asymptomatic murmurs in children.
The purpose of this study is to review sedated outpatient echocardiograms performed to evaluate asymptomatic murmurs in children between the ages of 1 month and 4 years and describe outcomes of tests done to determine if utility varies among age of study and referral type (primary care physician vs. pediatric cardiologist.) We aim to describe the yield in a contemporary cohort which has increased availability and quality of diagnostic aids such as fetal ultrasound, newborn pulse oximetry, and neonatal echocardiography. ⋯ Among echocardiograms ordered for children over 1 year of age with an asymptomatic murmur, there was no severe and little moderate disease. Cardiac disease is significantly less likely when echocardiograms are ordered without referral to a pediatric cardiologist. The workup for asymptomatic murmurs does not require an echocardiogram, and these results may aid clinicians when deciding whether evaluation of a child should include this study.
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Congenital heart disease · May 2012
Randomized Controlled TrialImpact of vitamin C on endothelial function and exercise capacity in patients with a Fontan circulation.
To evaluate the impact of antioxidant therapy on functional health status in Fontan-palliated patients. Design. Prospective, randomized, double-blind, placebo-controlled trial. ⋯ Short-term therapy with vitamin C does not alter endothelial function or exercise capacity in an asymptomatic Fontan population overall. Vitamin C may provide benefit to a subset of Fontan patients with abnormal vascular function.
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Congenital heart disease · May 2012
Comparative StudyB-type natriuretic peptide and amino-terminal pro-B-type natriuretic peptide in pediatric patients with pulmonary arterial hypertension.
B-type natriuretic peptide (BNP) and the amino-terminal fragment (NTproBNP) correlate with clinical variables, but have not been simultaneously studied in a large number of pediatric patients with pulmonary arterial hypertension (PAH). The purpose of our investigation was to compare BNP and NTproBNP with clinical indicators of disease in a pediatric PAH population for which biomarkers are much needed. ⋯ In pediatric PAH, BNP and NTProBNP are strongly correlated and predict changes in clinical variables and hemodynamics. In a cross-sectional analysis, NTproBNP correlated with echocardiographic and exercise data better than BNP; NTproBNP showed less within patient variability over time; therefore, NTproBNP can add additional information toward predicting these clinical measurements.
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Congenital heart disease · May 2012
Case ReportsCongenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.
Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. ⋯ Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.