Cardiology in the Young最新文献

Levoatriocardinal vein is a rare venous anomaly occasionally presenting as a bidirectional shunt in structurally normal hearts. We describe two unique systemic connections of levoatriocardinal vein without intracardiac defects, one causing paradoxical embolism via the hemiazygos vein, and the other draining from the left jugular vein to the pulmonary vein, requiring surgical correction to preserve cerebral venous return.

Acute rheumatic fever is a systemic autoimmune disease resulting from an abnormal immune response to group A streptococcal pharyngitis. Cardiac findings are an important part of acute rheumatic fever and usually manifest as electrocardiographic abnormalities. The most common arrhythmia is first-degree atrioventricular block; however, in rare cases, complete atrioventricular block may occur. In this article, we present the case of an 11-year-old child who presented with complaints of knee pain and limited movement, in whom the diagnosis of acute rheumatic fever was suspected based on clinical, echocardiographic, and laboratory findings, and in whom complete atrioventricular block was detected on electrocardiographic. The patient's electrocardiographic initially showed complete atrioventricular block, but with the treatment process, different levels of complete atrioventricular block were observed, and the electrocardiographic findings returned to normal. Particularly in patients who develop complete atrioventricular block, the response to treatment should be evaluated before making rapid decisions for pacemaker implantation, taking into account that the arrhythmia may be transient and can be corrected with medical treatment. This article emphasises that electrocardiographic abnormalities should not be ignored when diagnosing acute rheumatic fever and that patients should be evaluated in a holistic manner.

Fulminant myocarditis is a life-threatening event that can present as cardiogenic shock. Human metapneumovirus (hMPV)-associated myocarditis is exceptionally uncommon, particularly in the pediatric population. Treatment may require mechanical ventilation, inotropic agents, vasopressors, and advanced life support systems. In this article, we report an 18-month-old previously healthy infant who presented with severe metabolic acidosis, elevated lactate, and profound biventricular systolic dysfunction secondary to hMPV infection. Despite mechanical ventilation, inotropic support, and initial immunomodulatory therapy with intravenous immunoglobulin, high-dose methylprednisolone, and anakinra, the patient's clinical condition deteriorated rapidly, requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO) within the first 12 hours of admission. Given the absence of a patent foramen ovale and significant ventricular distention risk, surgical left ventricular decompression via a cannula inserted through the right upper pulmonary vein was performed. Hemoadsorption was additionally incorporated to mitigate hyperinflammation. Laboratory findings fulfilled macrophage activation syndrome criteria, and interferon-gamma blockade with emapalumab was initiated due to refractory cytokine storm physiology. Antioxidant therapy (nicotinamide adenine dinucleotide, coenzyme Q10, quercetin) was used as supportive treatment. Progressive improvement in ventricular function was observed under this comprehensive life support-based regimen. By day 12 of ECMO support, biventricular systolic function had normalized, and the patient was successfully weaned and discharged with full recovery. This case underscores the importance of early recognition, advanced immunomodulation, and effective ventricular unloading in managing fulminant hMPV myocarditis in children.

Wolff-Parkinson-White syndrome, characterised by accessory pathways, is rarely seen with dextrocardia. We present a case of situs inversus-dextrocardia with Wolff-Parkinson-White syndrome successfully treated via catheter ablation using 3D mapping (EnSite Precision®). Adjustments included reversed electrocardiogram (ECG) electrode placement, EnSite patch positioning, and fluoroscopic views. Coronary sinus access required counterclockwise manoeuvers. Mapping identified a left-sided accessory pathway, necessitating transseptal puncture with mirror-image adjustments. Ablation at the optimal site resulted in success. This case highlights the feasibility of catheter ablation in dextrocardia with tailored procedural modifications.

Background: Pulmonary artery sling with complete tracheal rings represents a rare and challenging congenital anomaly, particularly in premature infants. We present a case of successful repair in an extremely low-weight premature infant.

Case presentation: A male premature infant (34 weeks of gestation, birth weight 1820 g) was diagnosed prenatally with pulmonary artery sling, perimembranous ventricular septal defect, and patent ductus arteriosus. At one month of age, bronchoscopy revealed severe tracheal stenosis with complete tracheal rings (3.1 mm external diameter). Despite the high surgical risk due to low body weight, complete surgical repair was performed at 2.7 kg through median sternotomy under cardiopulmonary bypass. The procedure included pulmonary artery sling repair with autologous pericardial augmentation, slide tracheoplasty using interrupted everted 6-0 PDS sutures, ventricular septal defect closure, and patent ductus arteriosus ligation. Intraoperative bronchoscopy confirmed adequate airway patency.

Conclusion: This case demonstrates that successful complete repair of complex cardiac and airway anomalies can be achieved in premature, low-weight infants when conventional weight gain thresholds cannot be met. Key factors for success include meticulous surgical technique, precise cardiopulmonary bypass management, careful perioperative care optimisation, and a multidisciplinary approach. While body weight alone should not be an absolute contraindication for surgical intervention, careful patient selection and appropriate institutional expertise are essential.

Background: Infective endocarditis is a leading cause of morbidity and mortality in children and adolescents with underlying CHD. Appropriate diagnostic workup and management in the inpatient setting can be challenging in this patient population due to the spectrum of disease complexity and the dynamic nature of the field. Therefore, the Paediatric Acute Care Cardiology Collaborative has undertaken the creation of this clinical practice guideline.

Methods: A panel of paediatric cardiologists, infectious disease specialists, intensivists, advanced practice practitioners, pharmacists, cardiothoracic surgeons, and a dentist was convened. The literature was systematically reviewed for relevant articles on the management of infective endocarditis in patients with CHD. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus for inclusion.

Results: Based on 127 articles that met the inclusion criteria, 82 recommendations were generated, 50 of which achieved consensus for inclusion and are included in this guideline. They address risk factors specific to CHD lesion type and prior interventions including implanted material, diagnostic considerations, management strategies, and recommendations on counselling other healthcare providers, patients, and families. Of the 50 consensus recommendations, 36 are strong recommendations, though 20 have low or very low quality of evidence.

Conclusions: A central theme in this guideline is that an individual's specific CHD lesion and prior interventions must be carefully considered for risk stratification, diagnostic approach, and management. While most are strong recommendations, many are supported by low quality of evidence, emphasising the need for further research in this subject.

Introduction: 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.

Materials and method: 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.

Conclusion: 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.

Objectives: To evaluate the right ventricle function by conventional echocardiographic methods and strain analysis in the long term after balloon pulmonary valvuloplasty. In addition, we investigated the relationship between pulmonary regurgitation, demographic data at the time of the procedure, and right ventricle dysfunction in late follow-up.

Methods: The records of patients submitted to balloon pulmonary valvuloplasty from 2001 to 2015 in a single centre were reviewed. From that sample, a revised cohort was formed, and the patients were submitted to clinical and echocardiographic evaluations.

Results: The retrospective and the revised cohort analyses included 73 and 18 patients, respectively. In the follow-up, pulmonary regurgitation was observed in all patients, and there was a significant worsening of its magnitude over time (p < 0.001); the severity of pulmonary regurgitation was associated with balloon pulmonary valvuloplasty performed in patients with weight < 3 kg (p < 0.03), body surface area < 0.3 m² (p < 0.04), and < 1 year of age (p < 0.006). Global longitudinal systolic strain of the right ventricle was abnormal in 8 of 18 patients, and conventional methods were abnormal in 2 of 18 patients (p = 0.001). There was a significant relationship between severe pulmonary regurgitation and right ventricle dysfunction detected only by strain evaluation (p = 0.01).

Conclusions: The severity of pulmonary regurgitation was related to the impairment of right ventricle function detected by strain. The predictors of pulmonary regurgitation severity in late follow-up were age < 1 year, weight < 3 kg, and body surface area < 0.3 m².

L-carnitine is essential for myocardial metabolism, and its depletion may compromise heart function. We conducted a prospective observational study to assess serum carnitine profiles in 26 children under 36 months of age undergoing elective open-heart surgery with cardiopulmonary bypass. Measurements included free carnitine, acylcarnitine, and the acylcarnitine-to-free carnitine ratio, recorded preoperatively and at multiple postoperative time points up to 24 hours. We observed a significant postoperative decline in free carnitine and an increase in the acylcarnitine-to-free carnitine ratio, both of which correlated with cardiopulmonary bypass duration. These results highlight a perioperative disturbance in carnitine metabolism, suggesting potential relevance for postoperative cardiac recovery.

Background: Limited data exist on how trainers are trained in paediatric cardiology training centres in Europe.

Methods: A cross-sectional study employing a structured and approved questionnaire was circulated to educationalists/trainers in 95 Association for European Paediatric and Congenital Cardiology training centres.

Results: Trainers provided complete data for 46 centres in 20 countries. The median number of trainers in each centre was 6 (range 1-16). The median number of years trainers were in a training role was 20 years (range 2-32 years). Sixty-six per cent of trainers received some training in being a trainer, most commonly a course by a local governing college (almost 50%). Almost 78% found such courses helpful as trainers. Sixty-eight per cent of trainers felt their education in training was optimal to be an effective trainer. Assessment of trainees varies from daily to monthly between centres. Workplace assessments (used by >90% trainers) with combined formative and summative feedback were the most common type of assessment. Only one-third of trainers understood or used entrustable professional activities. Time constraints in providing training were reported by 54% of trainers as the greatest challenge in providing training. The majority of trainers expressed a need for formal "training the trainers" courses and development of standards in training.

Conclusion: There is a marked variation in the level of training of trainers across Europe. A wide mix of assessment tools is used. Feedback is provided by the majority of trainers. Adopting a basic training programme for trainers may promote the training skills of paediatric cardiology trainers.