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Background: Lead management in children and young adults is still a matter of debate. Methods: To assess the course of transvenous lead extraction (TLE) in adults with pacemakers implanted in childhood (CIP) we compared 98 CIP patients with a control group consisting of adults with pacemakers implanted in adulthood (AIP). Results: CIP patients differed from AIP patients with respect to indications for TLE and pacing history. CIP patients were four–eight times more likely to require second-line or advanced tools. Furthermore, CIP patients more often than AIP were prone to developing complications: major complications (MC) (any) 2.6 times; hemopericardium 3.2 times; severe tricuspid valve damage 4.4 times; need for rescue cardiac surgery 3.7 times. The rate of procedural success was 11% lower because of 4.8 times more common lead remnants and 3.1 times more frequent permanently disabling complications. Conclusions: Due to system-related risk factors TLE in CIP patients is more difficult and complex. TLE in CIP is associated with an increased risk of MC and incomplete lead removal. A conservative strategy of lead management, acceptable in very old patients seems to be less suitable in CIP because it creates a subpopulation of patients at high risk of major complications during TLE in the future.

Left ventricular noncompaction (LVNC) is a genetically and phenotypically heterogeneous cardiomyopathy in which myocardium consists of two, distinct compacted and noncompacted layers, and prominent ventricular trabeculations and deep intertrabecular recesses are present. LVNC is associated with an increased risk of heart failure, atrial and ventricular arrhythmias and thromboembolic events. Familial forms of primary sinus bradycardia have been attributed to alterations inFrom March 2008 to July 2021, we enrolled six patients from four families with diagnosed isolated LVNC based on the clinical presentation, family history and echocardiographic and cardiovascular magnetic resonance (CMR) evidence of LVNC. Next generation sequencing (NGS) analysis was undertaken for the evaluation of the molecular basis of the disease in each family.A total of six children (median age 11 years) were recruited and followed prospectively for the median of 12 years. All six patients were diagnosed with LVNC by echocardiography, and five participants additionally by CMR. The presence of late gadolinium enhancement (LGE) was found in three children. Sinus bradycardia and dilation of the ascending aorta occurred in five studied patients. In four patients from three families, the molecular studies demonstrated the presence of rare heterozygous(1) The

Objective Early postoperative arrhythmias are a recognized complication of pediatric cardiac surgery. Methods Diagnosis and treatment of early postoperative arrhythmias were prospectively analyzed in 402 consecutive patients aged 1 day to 18 years (mean 29.5 months) who underwent operation between January and December 2005 at our institute. All children were admitted to the intensive care unit, and continuous electrocardiogram monitoring was performed. Risk factors, such as age, weight, Aristotle Basic Score, cardiopulmonary bypass time, aortic crossclamp time, and use of deep hypothermia and circulatory arrest, were compared. Statistical analysis using the Student t test, Mann–Whitney U test, or Fisher exact test was performed. Multivariate stepwise logistic regression was used to assess the risk factors of postoperative arrhythmias. Results Arrhythmias occurred in 57 of 402 patients (14.2%). The most common types of arrhythmia were junctional ectopic tachycardia (21), supraventricular tachycardia (15), and arteriovenous block (6). Risk factors for arrhythmias, such as lower age ( P = .0041 ⁎ ), lower body weight ( P = .000001 ⁎ ), higher Aristotle Basic Score ( P = .000001 ⁎ ), longer cardiopulmonary bypass time ( P = .000001 ⁎ ), aortic crossclamp time ( P = .000001 ⁎ ), and use of deep hypothermia and circulatory arrest ( P = .0188 ⁎ ), were identified in a univariate analysis. In the multivariate stepwise logistic regression, only higher Aristotle Basic Score was statistically significant ( P = .000003 ⁎ ) compared with weight ( P = .62) and age ( P = .40); in the cardiopulmonary bypass group, only longer aortic crossclamp time was statistically significant ( P = .007 ⁎ ). Conclusion Lower age, lower body weight, higher Aristotle Basic Score, longer cardiopulmonary bypass time, aortic crossclamp time, and use of deep hypothermia and circulatory arrest are the risk factors for postoperative arrhythmias. Junctional ectopic tachycardia and supraventricular tachycardia were the most common postoperative arrhythmias.

Loss-of-function de novo mutations in the SETD5 gene, encoding a putative methyltransferase, are an important cause of moderate/severe intellectual disability as evidenced by the results of sequencing large patient cohorts. We present the first familial case of a SETD5 mutation contributing to a phenotype of congenital heart defects and dysmorphic features, with variable expression, in two siblings and their father. Interestingly, the father demonstrated only mild intellectual impairment. Family based exome sequencing combined to careful parental phenotyping may reveal a more complex clinical picture in newly recognized syndromes. © 2016 Wiley Periodicals, Inc.