Cardiology in the young
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Cardiology in the young · Aug 2014
Tissue motion annular displacement of the mitral valve using two-dimensional speckle tracking echocardiography predicts the left ventricular ejection fraction in normal children.
The gold standard for determining the left ventricular ejection fraction is cardiac magnetic resonance imaging. Other parameters for determining the ejection fraction such as M-mode echocardiography are operator-dependant and often inaccurate. Assessment of the displacement of the mitral valve annulus using two-dimensional speckle tracking echocardiography may provide an accurate and simple method of determining the left ventricular ejection fraction in children. ⋯ TMAD of the mitral valve is a simple, effective, and highly reproducible method of assessing the ejection fraction in normal children. It shows a strong linear correlation with magnetic resonance imaging-derived ejection fraction and is superior to M-mode-derived ejection fractions.
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Cardiology in the young · Aug 2014
Extracorporeal life support with an integrated left ventricular vent in children with a low cardiac output.
The aim of this study was to evaluate our experience in central extracorporeal life support with an integrated left ventricular vent in children with cardiac failure. ⋯ In case of a low cardiac output and an insufficient inter-atrial shunt, additional left ventricular decompression via a vent could help avoid left heart distension and might promote myocardial recovery. In pulmonary dysfunction, separate blood gas analyses from the venous cannula and the left ventricular vent help detect possible coronary hypoxia when the left ventricle begins to recover. We recommend the use of central extracorporeal life support with an integrated left ventricular vent in children with intractable cardiac failure.
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Cardiology in the young · Aug 2014
Case ReportsPaternal fibrillin-1 mutation transmitted to an affected son with neonatal marfan syndrome: the importance of early recognition.
We describe a case of neonatal Marfan syndrome diagnosed because of a family history, dysmorphic features, and cardiac abnormality. The echocardiogram showed aortic root dilatation. Molecular genetic studies showed a mutation in exon 31 of the FBN1 gene in the infant and father. The infant was treated with losartan, which significantly slowed the rate of enlargement of the aorta.
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Cardiology in the young · Jun 2014
Accelerated junctional rhythm in children with acute rheumatic fever: is it specific to the disease?
During the course of acute rheumatic fever, some electrocardiographic changes are seen. First-degree atrioventricular block is the most common electrocardiographic abnormality. Second- and third-degree atrioventricular block, ventricular tachycardia, and junctional acceleration are also seen. In the present study, the specificity of accelerated junctional rhythm to acute rheumatic fever was INVESTIGATED. ⋯ Accelerated junctional rhythm is specific to acute rheumatic fever. Although its frequency is low, it seems that it can be used in the differential diagnosis of acute rheumatic fever, especially in patients with isolated polyarthritis.
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Cardiology in the young · Jun 2014
Aberrant subclavian artery origin in tetralogy of Fallot with pulmonary stenosis is associated with chromosomal or genetic abnormality.
We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.2 deletion (n = 16); other chromosomal abnormalities (n = 9); CHARGE (n = 2); Pierre Robin (n = 2); and Kabuki, Alagille, Holt-Oram, Kaufman McKusick, Goldenhar, and PHACE (n = 1 each). ⋯ Syndromes in patients with an aberrant subclavian artery included trisomy 21 (n = 4); chromosome 22q11.2 deletion (n = 5); and Holt-Oram, PHACE, CHARGE, and chromosome 18p deletion (n = 1 each). Aberrant right or left subclavian artery in tetralogy of Fallot with pulmonary stenosis is associated with an increased incidence of chromosomal or genetic abnormality, whereas right aortic arch with mirror image branching is not. The assessment of aortic arch anatomy at prenatal diagnosis can assist counselling.