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Aim: Evaluation of cardiac structures requires a large study group for accurate data on normal values. The aim of the study was to obtain normal M mode echocardiographic values in a substantial sample of healthy term neonates and children to develop centile charts., Materials and Methods: Data were obtained over 2 years from a single center in Turkey, from 1200 healthy infants and children aged 1 day to 17 years. Using echocardiographic investigation, measurements were obtained of the following: left ventricular dimension at end diastole and end systole; thickness of interventricular septum and posterior wall of the left ventricle; aortic and pulmonary root diameter; and left atrial dimension. The influence of systematic errors as statistical noise in this large sample was decreased using third- degree polynomial curves., Results: Measurements are presented graphically as curved lines of centiles with respect to body weight for healthy term neonates and children. The values showed a good correlation with body weight and allowed the construction of percentile curves (5%, 25%, 50%, 75%, and 95%). Higher values were observed in boys during adolescence., Conclusion: The presented charts and tables make it possible to judge the echocardiographic measurements of a particular patient as normal or abnormal.

Anomalous origin of the left coronary artery arising from pulmonary artery ALCAPA syndrome is a rare congenital heart disease seen in children. If untreated, it may lead to congestive heart failure, dilated cardiomyopathy (DCM), ischemic and arrhythmic complications may lead to patient's death. ALCAPA is diagnosed with echocardiography; in the patients of suspected diagnosis, computerized tomography, magnetic resonance imaging and cardiac catheterization are used for further testing. Surgically correctable ALCAPA syndrome must be considered as etiology of DCM in children. In this report, we presented the case of an infant that was referred to our center with the diagnosis of DCM, who was echocardiographically diagnosed with ALCAPA syndrome and successfully treated with surgery, as well as a review of recent literature. [ABSTRACT FROM AUTHOR]

Mucopolysaccharidosis type 6, also known as Maroteaux-Lamy syndrome, is a rare, multisystemic, progressive disease characterized by somatic involvement of varying severity and normal intelligence. N-acetylgalactosamine-4-sulfatase enzyme is lacking and disfunction at cell, tissue, and organ levels occurs due to dermatan sulfate accumulation. Besides the findings such as coarse facial appearance, hydrocephalus, dysostosis multiplex, corneal opacity, hepatosplenomegaly, and joint stiffness, cardiac involvement is also frequently observed. Valvular heart disease, cardiomyopathy, and arrhythmia may occur in patients. In this article, we presented and discussed a mucopolysaccharidosis type 6 patient having moderate mitral valve regurgitation together with dextrocardia as a case in the light of recent literature. [ABSTRACT FROM AUTHOR]

Univentricular heart is a rare congenital defect that consists, in nearly 80% of cases, of double inlet left ventricle, to which both atrioventricular valves connect. A 31-year-old male patient was diagnosed with single ventricle at the age of 9. He had not received any surgical intervention and was not on any medication. Functional capacity was assessed as New York Heart Association class III. Both atrioventricular valves were found to open into the morphological left ventricle, which was located on the right; the hypoplasic right ventricle was on the left side of the left ventricle, the posteriorly located aorta emerged from the right ventricle, and the anteriorly located pulmonary artery emerged from the left ventricle. There was significant subvalvular and valvular stenosis in the pulmonary valve site. The disease is complex and it is very rare that untreated patients reach adulthood. [ABSTRACT FROM AUTHOR]