Cardiology in the Young最新文献

Three-dimensional computed tomography provides an accurate interpretation of anomalous coronary arteries and related anatomy, which helps determine the ideal surgical approach in rare cases.

Purpose: Children with CHDs are exposed to ionising radiation during diagnostic and interventional cardiac catheterisation procedures. Data regarding radiation doses a child is exposed to during these procedures are scarce. We aimed to investigate the radiation dose and other related parameters for paediatric cardiac catheterisation and propose these as local reference levels.

Methods and materials: We collected radiation exposure data for all diagnostic and interventional cardiac catheterisation cases (excluding electrophysiology procedures) utilising fluoroscopy at "Anonymized for review" between January 1, 2021 and November 30, 2021. Direct measures of radiation usage, i.e. fluoroscopy time, dose-area product value, and air kerma, were reported for each case by the INNOVA 2100 manufactured by Wipro GE Healthcare. The absorbed radiation dose was measured using a Bhabha Atomic Research Centre dosimeter, which was placed directly on the patients' chests. Differences in measures of radiation exposure across procedural and interventional types were tested using the normality of continuous data by Kolmogorov-Smirnov test. Spearman's correlation coefficient was calculated when the data violated normality.

Results: The overall median dose-area product, fluoroscopy time, air kerma, and absorbed dose measured using a Bhabha Atomic Research Centre dosimeter were 300 mg.m², 10.5 mins, 37 mGy, and 2.4 mSv, respectively. We have further categorised the median exposure parameters into 5 age groups and propose them as local dose reference levels. There was a strong correlation between fluoroscopy time with the absorbed radiation dose (Rho = 0.942). Fluoroscopy time showed fair correlation with air kerma (Rho = 0.65) and dose-area product (Rho = 0.59). Absorbed radiation dose showed fair correlation with dose-area product ( Rho = 0.56).

Conclusion: Fluoroscopy time alone is not a sufficient metric to monitor radiation exposure. Even when fluoroscopy times were comparable, air kerma and dose-area product levels were quite different. Therefore, the local dose reference levels proposed can be used as a benchmark for future studies aimed at process optimisation and further reducing the radiation exposure in paediatric patients.

Aim: This study aimed to evaluate the characteristics, difficulties, and outcomes of patients who underwent transcatheter ablation treatment due to arrhythmia with a diagnosis of CHD.

Methods: A total of 166 patients (189 substrates) with CHD who underwent catheter ablation between November 2013 and 2023 were evaluated retrospectively. EnSite™ (St Jude Medical Inc., St Paul, MN, USA) was used in all patients.

Results: The mean age was 14.8 ± 7.9 years (2.9-43 years). The most common CHD's were Ebstein anomaly (n: 40), tetralogy of Fallot (n: 31), atrial septal defect (n: 25), ventricular septal defect (n: 22), great artery transposition (D/L TGA, n: 12), and complex CHD in single ventricle physiology (n: 9). The most common arrhythmia mechanisms were Wolf-Parkinson-White syndrome (WPW, n: 50), intraatrial reentrant tachycardia (IART, n: 39), typical atrioventricular nodal reentrant tachycardia (AVNRT, n: 37), and ventricular tachycardia-ventricular extrasystoles (VT/VES, n: 23). There was more than one arrhythmia in 23 patients and multiple manifest accessory pathways in 10 patients. The average procedure time was 174 ± 69.3 minutes, and the average fluoro time was 8.3 minutes. While successful ablation was performed in 176/189 (acute success 93.1%) substrates, the procedure was unsuccessful in five patients and suboptimal in eight patients. Recurrence was observed in 11 patients (6.4%) during a mean follow-up period of 49.2 ± 30.1 months. A second ablation was performed on 13 patients. Acute success was achieved in all except one patient. A total of 11 patients are being followed up with medical treatment.

Conclusion: Despite the complex anatomy, age, operations, and limited vascular access possibilities in patients diagnosed with CHD, transcatheter ablation treatment with advances in electrophysiology, the introduction of different energy types, special ablation catheters, multipolar mapping catheters, and 3D nonfluoroscopic mapping systems seems to be a safe and effective option.

Pseudoaneurysm after valve-sparing aortic root replacement is uncommon but potentially life-threatening. We report an unusual case of spontaneous resolution of an aortic root pseudoaneurysm that developed in a 14-year-old boy with Marfan syndrome following valve-sparing aortic root replacement. This case suggests that, in carefully selected situations, non-surgical management with intensive follow-up may be beneficial.

Circular shunt is a very rare, albeit critical, condition. We present a case of a post-surgical circular shunt that resulted after correction of an anomalous total pulmonary venous connection to the coronary sinus with a persistent left superior vena cava. A left cavo-pulmonary anastomosis was performed; however, she developed heart failure during follow-up. The anastomosis was closed by interventional cardiac catheterisation without complications that led to resolution of heart failure symptoms.

We report the case of a 15-year-old male patient with severe pulmonary regurgitation who successfully underwent Alterra Adaptive Prestent implantation through the right jugular vein because of bilateral femoral vein occlusion.

Electrocardiogram interpretation is critical in neonatal care, as artefacts may mimic life-threatening arrhythmias. We report the case of a preterm newborn girl, delivered at 31 weeks of gestation due to acute fetal distress, who developed tachycardia shortly after birth. The initial ECG showed chaotic electrical activity, initially raising suspicion of artifact; however, a subsequent tracing confirmed a life-threatening arrhythmia with wide QRS complexes, prolonged QTc, and torsades de pointes. As the patient remained haemodynamically stable, intravenous lidocaine and magnesium sulphate were administered, achieving pharmacological cardioversion. Propranolol was subsequently initiated, and the lidocaine infusion was tapered and replaced with oral mexiletine. Serial electrocardiograms showed QTc interval improvement, left ventricular function normalised, and the patient was discharged on the 39th postnatal day following stabilisation and supportive care for prematurity. Genetic testing identified a de novo SCN5A c.796C > G (p.L266V) mutation associated with Long QT syndrome type 3. At 1 year of age, the patient remains stable and asymptomatic while receiving combined therapy with propranolol and mexiletine.

Congenital junctional ectopic tachycardia is a rare arrhythmia that poses significant management challenges. This report presents a case of neonatal-onset congenital junctional ectopic tachycardia treated with cedilanid, amiodarone, and propafenone but persisted in episodes. Sinus rhythm was restored following the initiation of ivabradine therapy. The review of the literature indicates that ivabradine demonstrates efficacy in the treatment of paediatric junctional ectopic tachycardia, particularly in refractory cases, without significant side effects. These findings suggest that ivabradine has broad applications in the treatment of refractory arrhythmias.

Background: This study aims to evaluate patient outcomes related to mitral valve disease (stenosis, regurgitation, or mixed) who benefited from mitral repair or replacement under one year of age.

Methods: Monocentric retrospective study including all children with mitral valve repair or replacement under 1 year of age over a period of 22 years (2001-2023).

The outcomes assessed were: early mortality (at 30 days), late mortality, and need for re-intervention.

Results: A total of 56 patients were identified, with a median age of 147 days and median weight of 5.1 kg. Of these, 39 underwent mitral valve repair and 17 underwent replacement. The median follow-up duration was 2.9 years (interquartile range 0.3-8.1). Patients who underwent replacement had longer ICU stays, hospital stays, and assisted ventilation times (p = 0.005, p = 0.01, p = 0.019), with higher early mortality (12% vs. 0%). Survival was significantly higher in the repair group (p = 0.039). Re-intervention was required in 23 patients (41.1%): 16 had replacement, 6 had re-repair, and 2 needed pacemaker implantation. Seven patients (12.5%) needed more than one re-intervention. Re-intervention-free survival rates after repair were 81%, 65%, and 46% at 1, 5, and 10 years, respectively. After replacement, rates were 74% at 1 year and 55% at 5 and 10 years. Conclusion Mitral valve surgery in infants is particularly high risk and is associated with high rate of re-intervention. While mitral repair demonstrates superior outcomes in mortality, it often delays but does not always prevent the need for valve replacement.

Background: In children with tetralogy of Fallot and associated right ventricular outflow tract obstruction, right ventricular outflow tract stenting has emerged as a viable alternative to surgical shunting, particularly in high-risk patients who are unsuitable for early definitive repair.

Methods: This prospective and retrospective observational study involved 55 symptomatic children with Fallot-type physiology who underwent right ventricular outflow tract stenting over a 30-month period at a tertiary-care centre in India. Data from pre-procedural imaging, intraoperative parameters, and post-procedural outcomes were analysed. The primary endpoints were improvement in systemic oxygen saturation and pulmonary artery growth. Secondary endpoints included complication rates, ICU stay duration, and factors associated with procedural failure.

Results: The median age at intervention was 11 months. All patients presented with cyanosis, and 80% had documented cyanotic spells. The right internal jugular vein was used for vascular access in 75% of cases. A significant improvement was observed in systemic oxygen saturation (from 67.8 to 87.1%, p < 0.001), along with an increase in pulmonary artery Z-scores. Complications included pulmonary oedema (47%), stent migration (7.2%), and new-onset tricuspid regurgitation (26.9%). The procedure had a success rate of 83.6%. Although no significant predictors of failure or prolonged ICU stay were identified, furosemide use was associated with a longer recovery time.

Conclusion: Right ventricular outflow tract stenting serves as a safe, effective, and reproducible palliative option in selected neonates with Fallot physiology. It facilitates improved systemic oxygenation and promotes pulmonary artery development, thereby serving as a bridge to definitive surgical repair.