Congenital heart disease
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Congenital heart disease · May 2012
Transcatheter closure of secundum atrial septal defect in infants less than 12 months of age improves symptoms of chronic lung disease.
Device closure of secundum type atrial septal defects in young children has now become common with extension of this practice to children less than 1 year of age. We hypothesized that patients less than 12 months of age with moderate increases in pulmonary blood flow due to atrial septal defects may improve clinically with device closure, particularly premature infants with chronic lung disease. ⋯ Transcatheter closure of atrial septal defects in infants can be safe, effective, and may be indicated for situations in which the left to right shunt may be implicated as a cause of ongoing chronic lung disease. Moderate increases in pulmonary blood flow due to atrial septal defects may have a negative clinical impact regarding continuing respiratory insufficiency in these patients.
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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
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
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.