Double aortic arch anomalies in Children: A Systematic 20-Year Single Center Study.

Aortic arch anomalies underlie numerous congenital disorders. Effectively diagnosing and treating them requires close understanding of cardiovascular embryology. As our Center serves the entire pediatric population of our country, we performed a comprehensive retrospective analysis of all aortic arch anomalies diagnosed at our Center over the past 20 years. We analyzed 40 children with aortic arch anomalies, distinguishing two defect types: Group 1 displayed ring-forming anomalies, and Group 2 other types of aortic arch anomalies that did not form a vascular ring. We performed detailed morphological analyses using echocardiography, angiography, computed tomography, or magnetic resonance imaging and generated a catalog of all aortic arch anomalies present. Group 1 was represented by 25 patients; 40% with persistent both aortic arches, and 60% with various forms of right aortic arch and an incomplete left aortic arch. Group 2 was represented by 15 patients with complex heart defects. On the basis of our dataset, the incidence of all aortic arch anomalies was 0.033%, and of ring-forming pathologies 0.021%. Although aortic arch anomalies are rare, it is important to diagnose them correctly. It is critical to distinguish ring-forming types. Although in complex defects the aortic arch anomaly represents only an additive diagnosis, its correct definition could be crucial for further management. Cumulatively, this unique, long-term study provides a systematic patient registry and offers critical epidemiological data to aid the study of rare congenital cardiovascular defects. Clin. Anat. 30:929-939, 2017. © 2017 Wiley Periodicals, Inc.

Arrhythmias in congenital heart defects.

AIM OF THE STUDY: Evaluation of the incidence and severity of late arrhythmias in patients with predisposing congenital heart defects--either due to the anatomy of the defect itself or as a result of a particular type of surgical intervention. PATIENTS AND METHODS: In a retrospective long-term study authors analyzed 158 patients (divided into 5 groups) with congenital heart defects after surgical correction. Evaluated were: the incidence of rhythm disturbances, the type of arrhythmia and the need for medication or intervention. RESULTS: The most rhythm disturbances occurred in patients after physiological correction of D-transposition of the great arteries (68.5%) and these patients also mostly needed medication or pacemaker implantation; followed were by patients with hypoplastic left heart syndrome after Fontan procedure (40%), then were patients after long-term correction of tetralogy of Fallot (31.1%), atrial septal defect sinus venosus type with partial anomalous pulmonary venous return after Warden correction (25.7%) and congenitally corrected L-transposition of the great arteries (25 %). Most of these arrhythmias were asymptomatic and there was no need to treat them. There was an increased incidence of arrhythmias with time (p < 0.05). DISCUSSION: During childhood in patients after surgical correction late arrhythmias mostly do not represent a severe problem, but with time, when reaching adulthood, this may be an issue. It is therefore very important to understand the anatomy, physiology and the arrhythmogenic substrate of every high risk congenital heart defect (Tab. 2, Fig. 6, Ref. 10).

[Risk factors for cardiovascular diseases in childhood]. / Rizikové faktory kardiovaskulárnych ochorení v detskom veku.

Risk factors for atherosclerosis in children were studied in families where the father had suffered from myocardial infarction before the age of 45 years and in the families of the closest relatives. The values of metabolism of lipids and atherogenic indexes were compared with the findings in a series of children with no positive family history. In children of at risk families cholesterol and triglyceride values were increased in 34.3%, HDLc was decreased in 42.1%, and apoprotein B was increased in 53.1%. Of the atherogenic indexes the LDL/HDL ratio proved to be the most significant. More than half of the children from at risk families can be considered to be threatened with atherogenesis. These children have to be followed up regularly and great care is to be given to a proper regimen.

[Myocarditis in childhood]. / Problematika myokarditíd v detskom veku.

In a group of 82 children followed up for prolonged periods the authors draw attention to some problems of virus myocarditis. They focused attention on the diagnosis of Coxsackie myocarditis for which they elaborated a scoring system. Based on this system they separated the prognostically most adverse group where cardiac decompensation was the dominant sign from the very beginning. They confirmed the theoretical assumption that in some children virus myocarditis develops into cardiomyopathy. This was proved in six children by histological examination and in 19 children by means of echocardiography.