Search

Background: Hypertrophic cardiomyopathy (HCM) is the second most common pediatric cardiomyopathy. Although there is a large body of literature about HCM in adults, there is limited information on HCM in childhood. We evaluated various aspects of pediatric HCM patients treated at our center. Methods: We identified 152 pediatric patients with HCM between October 2011 and October 2019. Clinical history, invasive (ICD, pacemaker, electrophysiologic study, catheter ablation therapy) and non‐invasive (ECG, holter moniterization, echocardiography, cardiac MR, genetic study, medicam treatment) data were collected and evaluated. Results: The mean ± standard deviation age of patients was 8.9 ± 5.7 years (1 month–18 years) and 67.8% were male. The most frequent clinical symptoms were murmur and palpitations. Three cases (2%) had aborted sudden death as the first manifestation of HCM. Of these patients, 120 (78.9%) had non‐syndromic HCM and 32 (27.2%) had syndromic HCM. Asymmetric septal hypertrophy was common (48.3%) in the non‐syndromic group, whereas concentric hypertrophy was common (56.2%) in syndromic group. Left ventricular outflow tract obstruction (LVOTO) occurred in 39 (25.6%) patients. Nine (5.9%) patients underwent electrophysiologic study and/or ablation and 16 patients underwent surgical intervention. Implantable cardioverter defibrillator (ICD) insertion was performed in 38 patients (26 transvenous, 12 epicardial). ICDs were inserted in three (7.9%) patients for secondary prevention; in the remaining patients (92.1%) the devices were placed for primary prevention. Mean SD follow‐up time was 27.1 ± 22 months. Five (3.3%) patients died during the follow‐up. No patient had heart transplantation or a long‐term assistive device. Conclusion: The etiology of HCM is heterogeneous and present at any age. It is important to determine the timing of surgery and potential risks for sudden cardiac arrest. As most cases of HCM are familial, evaluation of family members at risk should be a routine component of clinical management. [ABSTRACT FROM AUTHOR]

Background: Permanent pacemaker (PM) implantation is performed for various indications and by different techniques in children; however, many problems with lead performance are encountered during follow‐up. This study aims to evaluate the possible effects of different lead types and implantation techniques on pacing at early and midterm in children with a permanent PM. Patients and methods: Pediatric patients who underwent permanent PM system implantation at our tertiary cardiac surgery center between January 1, 2010 and January 1, 2020 were evaluated retrospectively. Patients were categorized in the epicardial pacing lead (EP), transvenous pacing lead (TP), and transvenous bipolar lumenless (Select Secure [SS]) lead groups according to the lead implantation technique and lead type with the same manufacturer. Groups were evaluated statistically for demographic features, pacing type and indication for implantation, lead electrical performance, lead failure, complications, and outcome. Results: Over 10 years, 323 lead implantations were performed on 167 patients (96 males, median age 68 months [5 days‐18 years]). Of 323 leads, 213 (66%) were EP, 64 (20%) were TP, and 46 (14%) were SS. Of the total, 136 of the leads were implanted in atria, and 187 were implanted in ventricles. Primary pacing indications were postoperative complete atrioventricular (AV) block (n = 95), congenital AV block (n = 71), sinus node dysfunction (n = 13), and acquired complete AV block (n = 1). Additional cardiac diseases were present in 115 patients (69%). No statistically significant difference was observed in gender, syndrome, or pacing indication (P >.05). Atrial and ventricular capture, threshold, sensing, and lead impedance measurements were not significantly different at the initial and follow‐up periods (P >.05). The median follow‐up duration was 3.3 years (6 months‐10 years). Twenty lead failures were determined in 15 patients (EP: 14 lead failures in 10 patients; TP: two lead failures in two patients; and SS: four lead failures in three patients) during follow‐up, and no statistically significant difference was found between groups (P =.466). The 5‐year lead survival was 98% for TP, 95% for EP, and 90% for SS; the 10‐year lead survival was 90% for TP, 70% for EP, and 70% for SS. There was no mortality related to chronic pacing or due to the procedure of implantation. Conclusions: Despite improvements in technology, lead failure is still one of the most critical problems during these patients' follow‐up. Early to midterm lead survival rates of all three lead types were satisfactory. [ABSTRACT FROM AUTHOR]

Despite the advancements in technology, establishing the optimal implantation technique for pediatric patients with a pacemaker (PM) indication remains challenging. Although the implantation of an epicardial PM is recommended, especially in children weighing less than 10 kg, transventricular placement of endocardial leads can be performed safely, offering a practical substitute for an epicardial pacing system, particularly in situations where a transvenous approach is unfeasible due to patient size, anatomical constraints or epicardial PM leads were not available as in our case. [ABSTRACT FROM AUTHOR]

Aim: This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction., Methods: This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022., Results: In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery-Dreifuss muscular dystrophy., Conclusion: Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.