Atrial septal defects (ASDs) are associated with an increased risk of atrial arrhythmias due to right atrial dilation, electrical remodeling, and conduction abnormalities. In addition to arrhythmias, autonomic dysfunction may also occur. Although several studies have investigated the impact of transcatheter ASD closure on arrhythmia risk in pediatric patients, direct comparative analyses between surgical and transcatheter closure techniques remain limited in the current literature. This study included patients who underwent ASD closure via surgical or transcatheter methods before age 18 and had at least 12 months of follow-up. A control group of healthy, age- and sex-matched children without cardiac disease was also included. All participants underwent 12-lead electrocardiography (ECG) and 24 h Holter monitoring. Patient data included arrhythmia symptoms, closure method, age at closure, defect size, and catheterization findings. Individuals with other cardiac anomalies, genetic syndromes, or medications affecting conduction were excluded. The study included 131 participants: 91 ASD patients (56 surgical, 35 transcatheter) and 40 controls. Supraventricular premature beats (SVPB) was significantly more frequent in both intervention groups compared to controls, with the highest frequency in the surgical group (p < 0.001). P-wave dispersion was also highest in the surgical group. In the surgical group, Lowns grade correlated positively with Qp/Qs, mean pulmonary artery pressure, and follow-up duration. Heart rate variability (HRV) parameters were significantly lower in the surgical group, indicating sympathetic dominance. Atrial septal defect repair increases atrial arrhythmia risk, particularly following surgical intervention. While autonomic function remained comparable to controls after transcatheter closure, surgical closure was associated with reduced HRV and increased sympathetic activity.

Maternal anti-Ro/SSA (Sjögren's syndrome-related antigen A) [± anti-La/SSB (Sjögren's syndrome type B antigen)] antibodies can lead to neonatal lupus, which may present most severely as autoimmune congenital atrioventricular block (CAVB). Although CAVB is uncommon (~ 2% of anti-Ro/SSA-positive pregnancies), once a complete block develops, spontaneous reversal is rare, and many affected infants require permanent pacemaker implantation. Consequently, prevention and early detection are critical components of management, particularly in pregnancies following previous antibody-mediated losses. A 34-year-old woman with primary Sjögren's syndrome, high-titer anti-Ro/SSA and anti-La/SSB, and a history of five prior pregnancy losses-two early (biochemical, 7 weeks) and three midtrimester (16-21 weeks) complicated with severe fetal complete heart block (one demise, two terminations for hydrops)-presented for her sixth pregnancy. A multidisciplinary protocol was implemented, including hydroxychloroquine 400 mg/day, methylprednisolone (initially 6 mg/day, briefly increased to 32 mg/day at 14-16 weeks, then tapered to 8 mg/day), low-dose aspirin 50 mg/day, and fondaparinux 2.5 mg/day from ovulation throughout pregnancy, along with scheduled intravenous immunoglobulin (IVIG; 20 g at 4, 6 + 6, and 8 + 2 weeks; followed by 20 g/day × 3 at 14, 18, and 22weeks). From 14weeks, weekly fetal echocardiography with Doppler atrioventricular (AV)-interval monitoring (16-26weeks) remained normal. At 38 + 2weeks, a cesarean section delivered a female infant weighing 2,710 g and measuring 49 cm, with Apgar scores of 9/10. Neonatal telemetry/ECG showed sinus rhythm at 144 bpm without AV block. Echocardiography revealed a patent ductus arteriosus and a small atrial septal defect, with moderate pulmonary hypertension (SPAP 51 mmHg). Brain MRI and EEG were normal, and there were no cutaneous, hepatic, hematologic, or other features of neonatal lupus. Postpartum, the mother continued methylprednisolone 6 mg/day, hydroxychloroquine 400 mg daily, and enoxaparin 4,000 IU once daily for 4 weeks maintain disease suppression and thromboprophylaxis. In an anti-Ro/SSA-positive pregnancy at extreme risk, a prevention-first, protocol-driven approach-centered on hydroxychloroquine, judicious immunomodulation, and structured AV-interval surveillance-successfully averted CAVB and resulted in a pacemaker-free live birth. Minor cardiac lesions warrant ongoing follow-up; however, the absence of conduction disease underscores the clinical utility of this strategy in carefully selected, extreme-risk pregnancies.

Catheter ablation is now the preferred early treatment option in children due to its high success rate and low complication rate. The aim of this study was to evaluate the outcomes of patients who underwent electrophysiology studies and a catheter ablation procedure in our centre. Paediatric patients who underwent catheter ablation in our hospital between 2017 and 2024 and were followed up or referred for ablation from another centre were retrospectively screened. Patients who had an intracardiac defibrillator, pacemaker, or ventricular arrhythmia were excluded from the study. The data were screened from a total of 586 patients (M/F = 300/286). The procedure was applied to 288 (49 %) patients with atrioventricular re-entry tachycardia (Wolf Parkinson White 86.4%-n:249; occult accessory pathway 13.9%- n:39), and to 270 (45%) with atrioventricular nodal re-entry tachycardia. Eleven (1.8%) patients with focal atrial tachycardia, 11 (1.8%) with frequent extrasystole or ventricular tachycardia, 6 (1%) with supraventricular premature beat (SVE), and 2 patients with a Mahaim diagnosis underwent the procedure. Fluoroscopy was used during the procedure in 75 (12.8%) patients, and the mean duration of fluoroscopy was 5.2 ± 3.2 mins (range 0.2-19.6 mins). Of these 75 patients, trans-septal punction was performed on the left side for the procedure to be applied in 71 patients. Of the patients diagnosed with atrioventricular re-entry tachycardia, fluoroscopy was used in 4. The results of this study of catheter ablation performed at our institution have shown acute success and recurrence rates similar to those of previously published studies.

Surgical repair of ventricular septal defects (VSDs) with straddling atrioventricular (AV) valve chordae is challenging due to the risk of disrupting valve integrity. We report the successful use of a dual-patch technique in a 5-month-old girl (6.1 kg) with Down syndrome, presenting with a large inlet VSD, secundum atrial septal defect (ASD), and straddling chordae involving both AV valves. Ventricular septal defects closure was performed via right atriotomy using 2 glutaraldehyde-treated autologous pericardial patches placed on the superior and inferior septal margins, encasing the chordae without division. Mitral and tricuspid valve clefts were repaired, and the ASD was closed primarily. Postoperative echocardiography showed no residual VSD and only mild AV valve regurgitation. This approach preserved valvular geometry and avoided conduction disturbance. The dual-patch technique offers a physiologic and conservative solution when conventional VSD repair is precluded by straddling chordae. It avoids chordal translocation or reimplantation, maintaining the native architecture and function of the AV valves. Holt-Oram syndrome (HOS) is characterized by the association of upper-limb defects, congenital heart malformations, and cardiac conduction disease. Upper-limb malformations are usually bilateral/asymmetric, rarely unilateral or bilateral/symmetric, and affect the radial ray. They can range from thenar hypoplasia, triphalangeal thumb(s), or absent thumb(s) to radial agenesis/hypoplasia to phocomelia. Deformities of the carpal and thenar bones, abnormalities of the shoulders and/or elbows, and vertebral defects can occur. A congenital heart malformation is present in 90% of individuals with HOS and most commonly involves the septum. Atrial septal defect and ventricular septal defect can vary in number, size, and location. Complex congenital heart malformations can also occur in individuals with HOS. Individuals with HOS with or without a congenital heart malformation are at risk for cardiac conduction disease (30%). While individuals may present at birth with sinus bradycardia and first-degree atrioventricular (AV) block, AV block can progress unpredictably to a higher grade including complete heart block with and without atrial fibrillation. The clinical diagnosis of HOS is established by the presence in a proband of a preaxial radial ray anomaly and a personal or family history of cardiac septation and/or conduction defects. More than 70% of individuals who meet strict clinical diagnostic criteria have an identifiable heterozygous pathogenic variant in TBX5. Treatment of manifestations: Management involves a multidisciplinary team of specialists in medical genetics, cardiology, orthopedics, and hand surgery. Treatment of upper-limb malformations per orthopedist can include surgery, physical therapy, occupational therapy, and/or prostheses in those with severe limb shortening. Social and psychological support for affected individuals and families; standard treatment for congenital heart malformation per cardiologist and cardiac surgeon; anticoagulants and antibiotic prophylaxis for bacterial endocarditis if recommended by cardiologist; treatment for arrhythmias may require medication, surgery, and/or pacemaker implantation; pharmacologic treatment for individuals with pulmonary hypertension per cardiologist and/or intensivist. Surveillance: Assess limb function and activities of daily living per orthopedist, physical therapist, and/or occupational therapist; annual EKG in those at risk of developing a conduction defect; annual EKG combined with Holter monitor for individuals with known conduction disease; echocardiogram according to the absence/presence of congenital heart defect and history of heart surgery, every five years in the absence of congenital heart defects to assess for rare cardiomyopathy; surveillance in those with pulmonary hypertension per cardiologist and/or intensivist. Agents/circumstances to avoid: Certain medications may be contraindicated in individuals with arrhythmias, cardiomyopathy, and/or pulmonary hypertension. Evaluation of relatives at risk: Presymptomatic diagnosis and treatment is warranted in relatives at risk to identify those who would benefit from appropriate cardiac management. Pregnancy management: Affected women who have not undergone cardiac evaluation should do so prior to pregnancy or as soon as the pregnancy is recognized; those with a known history of a structural cardiac defect or cardiac conduction abnormality should be followed by a cardiologist during pregnancy. HOS is inherited in an autosomal dominant manner. Some individuals diagnosed with HOS have an affected parent; up to 60% of affected individuals represent simplex cases. Significant intrafamilial variability in limb and heart defect severity is observed among affected family members. Offspring of an individual with HOS have a 50% risk for HOS. If the TBX5 pathogenic variant has been identified in an affected family member, prenatal and preimplantation genetic testing are possible. If the pathogenic variant in the family is not known, prenatal ultrasound (US) examination evaluating for characteristic limb and cardiac manifestations is recommended (a normal US examination does not eliminate the possibility of HOS in the fetus).

Wolff-Parkinson-White (WPW) syndrome is a disorder characterized by presence of an accessory pathway (AP) which predisposes patients to tachyarrhythmia and sudden death. The aim of this study was to evaluate the prevalence of high-risk electrophysiologic AP features among WPW patients referred for electrophysiological study (EPS) and to explore whether the AP location can predict the high-risk nature of the AP. This descriptive observational study was carried out on 70 patients with WPW subjected to invasive EPS. All patients were subjected to determination of AP anterograde conduction properties [AP effective refractory period (APERP) and shortest pre-excited RR interval (SPERRI)], mapping for localization, and ablation attempts of the AP. Twenty-five patients (35.7%) had an AP antegrade refractory period (APERP) ≤ 240 ms. The shortest RR interval during pre-excited AF (SPERRI) was ≤ 250 ms in 17 patients. Thirty-two APs (45.7%) were classified as high-risk AP (having APERP ≤ 240 ms and/or SPERRI ≤ 250 ms). Radiofrequency ablation was successful in 59/63 patients (93.7%). The mean APERP was significantly lower in postero-septal than anteroseptal and mid-septal APs (p < 0.003), and in left sided than right sided APs (p value = 0.001). Left non-septal APs (specially the left lateral APs) were significantly associated with the presence of high-risk than low-risk parameters. Septal APs (anterior, mid, and posterior) were significantly associated with the presence of low-risk than high-risk parameters. High-risk AP features as determined by the antegrade conduction properties are common among WPW patients. AP location may hold significance in predicting the presence of those high-risk electrophysiologic features.