Cardiology in the Young最新文献

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.

Bodily isomerism, often referred to as "Heterotaxy," is the clinical entity in which the thoracic organs are themselves mirror-imaged in the same individual. The thoracic isomerism can be right or left. The left thoracic isomerism always has both atria with their atrial appendages of left morphology and frequently a mixed atrioventricular connection. Usually, there are two well-developed ventricles; however, in some cases, there may be univentricular physiology. In the set of left thoracic isomerism, no case of imperforate tricuspid valve with severe hypoplasia associated with a vascular ring has been reported in the literature.

Background & aims: Patients with cyanotic CHD and those with metabolic dysfunction-associated steatotic liver disease are at risk of liver fibrosis. We compared hepatic extracellular volumes and native T1 values to better understand the burden of liver disease in these populations.

Methods: The sample comprised 136 patients in 5 groups: control (n = 23), metabolic dysfunction-associated steatotic liver disease [mild (F0-F1) and significant (F2-F4) fibrosis; n = 45], repaired tetralogy of Fallot (n = 30), and Fontan circulation (n = 38). Differences were assessed using linear regression models, with adjustment for the body mass index and sex.

Results: The hepatic extracellular volume was significantly larger in the Fontan group (43.96% ± 4.22%) than in the other groups, even with adjustment. Patients with Fallot had significantly larger extracellular volumes (36.77% ± 5.63%) than did controls and mild liver disease (p < 0.001 and p = 0.011, respectively), although smaller extracellular volumes than patients with significant liver disease (p = 0.042). These trends were corroborated by native T1 values, which were highest in patients with Fontan (1013.7 ± 86.1 ms), although not significantly different from patients with F2-F4 steatotic liver disease.

Conclusions: The potential burden of CHD-related hepatic injury and steatotic liver disease highlights the importance of early identification. Given the possible additional risk of liver fibrosis in patients with coexisting metabolic dysfunction and CHD, comprehensive clinical management should prioritise regular metabolic risk assessment and the promotion of a healthy lifestyle to reduce the likelihood of liver disease development in this vulnerable population.

Background: Severe obesity in adolescents is a public health crisis of increasing concern. Cardiac maladaptation, such as left ventricular hypertrophy, increases the risk of future cardiovascular disease. This study aims to evaluate the impact of weight loss surgery on cardiac geometry in morbidly obese adolescents.

Methods: A single-centre, randomised controlled trial titled comparing laparoscopic adjustable gastric banding with multidisciplinary lifestyle intervention against a control group with multidisciplinary lifestyle intervention alone. The trial included severely obese adolescents aged 14-16 who had not responded to a minimum of 12 months of multidisciplinary lifestyle intervention. The primary outcome measured was the percentage change in total body weight one year after surgery. Left ventricular geometry was evaluated ultrasonographically before and one year after bariatric surgery.

Results: In the intervention and control group, a total of 10 and 8 patients received both the baseline and follow-up ultrasonographic examination. BMI significantly decreased in the intervention group compared to the control group (-6.75; 95% CI: -10.28 - -3.23). Moreover, left ventricular mass index (-8.21; 95% CI: -15.43 - -0.98) and relative left ventricular wall thickness (-0.04; 95% CI: -0.07 - 0.00) significantly decreased within the intervention group, reflecting amelioration of adverse remodelling, albeit without statistically significant intervention effect when compared to the control group.

Conclusions: Significant weight loss through bariatric surgery may improve left ventricular geometry in severely obese adolescents. These findings support the potential of bariatric surgery not only for reducing weight loss but also for improving cardiac health.

Slim et al.'s paper provided an insight into the differences between repaired tetralogy of Fallot and isolated pulmonary regurgitation in their strain. Repaired tetralogy of Fallot had higher right ventricular circumferential strain, while isolated pulmonary regurgitation relied on longitudinal strain more. This allowed the authors to infer that repaired tetralogy of Fallot can withstand more chronic regurgitation before valve replacement is necessary. We highlighted new findings relevant to this paper. Arrhythmia in repaired tetralogy of Fallot is associated with a reduced global circumferential strain of the right ventricle. Specifically, a value of below -14% was associated with a 6.3 times increase in the risk for an arrhythmic event. We believe this would be beneficial for patients when considered for valve replacements, suggesting modification of current valve replacement guidelines to include strain thresholds alongside current volumetric thresholds. However, the data for isolated pulmonary regurgitation remains scarce. Further investigation is needed to provide clearer timelines for valve replacement. We emphasised the importance of exploring the underlying architecture of repaired tetralogy of Fallot patients' hearts and why they could generate more global circumferential strain. We acknowledged the broader effect of this paper and its specific benefit in our country, Egypt. This paper provided insights useful for broader global health impact, especially in low-income countries.

Background/objectives: Hypoplastic left heart syndrome is considered one of the most severe CHDs and occurs in approximately 2-3% of all CHD cases. Hybrid procedures have been introduced as an alternative to traditional surgical techniques, such as the Norwood procedure, particularly for neonates at high risk. Although hybrid approaches claim to reduce surgical risks and improve recovery, little is known regarding their comparative effectiveness and safety. This review aims to describe the contribution of hybrid procedures in hypoplastic left heart syndrome management regarding survival rates, postoperative complications, and quality of life, through recovery outcomes and long-term results, in light of conventional surgical techniques.

Methods: A systematic review was carried out following the PRISMA guidelines. Data were retrieved from PubMed, Scopus, Web of Science, and Cochrane Library. The search articles were published from January 2014 to November 2024. Inclusion was focused on paediatric patients diagnosed with hypoplastic left heart syndrome and studies comparing hybrid procedures versus traditional surgical methods. All screening, data extraction, and quality assessment were done by two independent reviewers.

Results: This review analysed data from 11 studies comprising 934 neonates with hypoplastic left heart syndrome, of whom 371 underwent hybrid procedures (HP) and 563 received the Norwood procedure. The findings demonstrated that both approaches achieved comparable survival rates at 1, 3, and 5 years. Hybrid procedures were linked to slightly earlier interventions (standardised mean difference = -0.10, 95% confidence interval: -0.61 to 0.41, p = 0.77) and were favored for high-risk neonates due to reduced invasiveness. However, hybrid procedures showed a higher rate of interstage events (risk ratio = 0.81), 95% confidence interval: -0.62 to 2.25, p = 0.09) and higher rates of pulmonary artery stenosis requiring reinterventions (30% vs. 18% for Norwood). Norwood procedures were associated with fewer reinterventions and lower interstage event rates, highlighting their effectiveness for stable patients.

Conclusions: Hybrid procedures offer a practical alternative to the Norwood procedure, especially for high-risk neonates. Although both approaches show similar long-term survival rates, hybrid procedures are associated with a higher risk of complications, including increased interstage mortality. These challenges highlight the need for continued advancements to refine hybrid techniques and to improve long-term outcomes. This review emphasises the critical role of tailored patient selection and calls for further research to enhance hybrid procedure protocols and optimise their effectiveness for specific patient populations.

Burkholderia cepacia is a rare cause of prosthetic valve endocarditis. We report an 18-year-old male with Tetralogy of Fallot and two sequential transcatheter pulmonary valves (Melody and Myval), presenting with persistent bacteraemia unresponsive to antibiotics. Surgical explantation of both valves with homograft replacement and tricuspid repair achieved complete recovery, emphasising surgery in refractory infections.