Congenital heart disease
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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.
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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 · Mar 2012
Preoperative management in patients with single-ventricle physiology.
Advancements in the preoperative management of patients with single-ventricle physiology continue to evolve. Previous reports have questioned the benefit of using inhaled nitrogen in single-ventricle patients, suggesting that this therapeutic modality may not provide adequate systemic cardiac output. The objective of this study was to review our institutional experience managing preoperative patients with single-ventricle physiology using a combination of afterload reduction and inhaled hypoxemic therapy. ⋯ Our results suggest that a combination of afterload reduction and hypoxemic therapy was able to maintain an appropriate distribution of the cardiac output in the majority of preoperative patients with single-ventricle physiology. An adequate balance of systemic and pulmonary blood flow was successfully achieved with an increase in arterial PaO2 values.
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Congenital heart disease · Jan 2012
ReviewCan "inoperable" congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality?
The decision whether to repair congenital heart defects in patients with raised pulmonary vascular resistance to alleviate pulmonary hypertension is a complex one. The degree of pulmonary vascular disease is of paramount importance. Operating on patients with pulmonary vascular resistance above a certain threshold runs the risk of postoperative persistent pulmonary hypertension and a worse long-term prognosis. This review focuses on patients deemed "borderline inoperable" or "inoperable" due to pulmonary vascular disease and asks whether they can be "converted to an operable status" with pulmonary arterial hypertension-specific drugs that potentially modify the pulmonary vascular lesions and resistance.