Cardiology in the Young最新文献

Background: Disparities in clinical outcomes exist among children with CHD, and social determinants of health are a significant contributor. Follow-up care plays an important role in long-term health and may be impacted by social determinants of health. We aimed to describe the population with lapses in care to outpatient, general paediatric cardiology from a social determinants of health standpoint and compare them to our primary service area.

Methods: A retrospective chart review was completed for children who had lapses in care within outpatient, general cardiology. Data were compared to the Cincinnati Children's Hospital Medical Center's primary service area population.

Results: A higher proportion of patients who had lapses in care were noted to identify as Hispanic/Latino, utilised some form of public insurance, and lived in communities with more social deprivation.

Conclusion: Patients who identify as a minority race/ethnicity, utilise public insurance, or live in an area with a higher social deprivation may be at a higher risk for lapses in outpatient cardiology care, including both follow-up care and initial evaluations.

Objective: Cardiac tamponade is a rare but life-threatening condition in children, typically requiring urgent percutaneous pericardiocentesis. Despite its clinical importance, comprehensive data on paediatric cardiac tamponade are limited.

Methods: We retrospectively reviewed 251 paediatric patients (0-18 years) with cardiac tamponade who underwent emergency percutaneous pericardiocentesis to evaluate the aetiological spectrum, age distribution, and associated clinical factors between November 2003 and May 2025. Diagnosis was based on echocardiographic criteria indicating haemodynamic compromise. Patients were categorised by age group, underlying aetiology, and recent history of cardiac intervention.

Results: Structural heart diseases were the leading cause of cardiac tamponade (51.6%), particularly in neonates and infants. Pericardiocentesis was most frequently performed in children aged 6-12 years (28.2%). Cardiomyopathies were more prevalent in older age groups. Infectious causes were the second most common aetiology (11.5%). Cardiac Tamponade occurred more frequently following cardiac surgery (12.3%) than after catheter-based interventions. Iatrogenic tamponade was identified in six patients, most commonly during high-risk transcatheter procedures. Genetic syndromes were present in 30 patients, with Down syndrome being the most common.

Conclusions: To the best of our knowledge, this study represents the largest single-centre cohort of paediatric cardiac tamponade requiring pericardiocentesis. Cardiac causes, particularly structural heart diseases, represent the most common aetiology across all age groups; therefore, these patients should be promptly evaluated for cardiac tamponade when presenting with relevant symptoms.

Persistent ductus venosus is an extremely rare disease that causes liver failure, hypoxaemia, and encephalopathy. We report the successful treatment of our patient with elevated transaminase and ammonia levels due to patent ductus venosus, diagnosed in the neonatal period and treated with a vascular plug device.

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.

Background: Recent studies show an association between lower socioeconomic status and worse outcomes in single ventricle patients after stage 1 palliation. We sought to investigate the association between socioeconomic status, using the social deprivation index, after the second and third stage palliations. We hypothesised that higher social deprivation index scores (higher deprivation) are associated with worse short and medium-term outcomes following Glenn and Fontan palliations.

Methods: We performed a retrospective single-centre review on patients who underwent Glenn or Fontan palliation from 2007 to 2024. Social deprivation index was calculated using the last known address. Outcomes were collected at 1 year after Glenn and 1 and 5 years after Fontan.

Results: There were 292 patients included. Higher social deprivation index scores were associated with higher weight (ρ = 0.19, p-value = 0.01) and fewer number of attended cardiology appointments at 1 year after Fontan (ρ = -0.20, p-value = 0.01) and higher weight (ρ = 0.36, p-value < 0.01) and weight percentile (ρ = 0.22, p-value = 0.02) at 5 years after Fontan. After adjusting for race and preferred language, weight at 1 year after Fontan (p-value = 0.007) and weight and weight percentile at five years after Fontan (p-value < 0.0001 and p-value = 0.04, respectively), remained significant. There was no association between social deprivation index score and number of hospitalisations, transplant, or mortality.

Conclusion: Higher social deprivation index scores were associated with higher weight and weight percentile and fewer number of attended cardiology appointments after Fontan palliation. Longer-term follow-up and multi-centre collaboration across diverse regions will be critical to understanding clinical impact.

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: Infants with CHD who undergo cardiopulmonary bypass surgery are at risk of impaired growth and neurodevelopment. However, few studies have thoroughly investigated the risk factors for growth and neurodevelopmental impairments, particularly with respect to the timing of the initial surgical intervention.

Methods: We retrospectively analysed term singleton infants with CHD who underwent cardiopulmonary bypass surgery at a Japanese tertiary centre between 2015 and 2021. Neurodevelopment was assessed at 18-22 months of age using the Kyoto Scale of Psychological Development. We compared outcomes by CHD type (univentricular [UV] vs. biventricular [BV]) and analysed risk factors for growth impairment (weight and height < tenth percentile) and neurodevelopmental impairment (developmental quotient [DQ] < 85), including birth weight, sex, the type of CHD (UV or BV), and timing of the initial cardiopulmonary bypass surgery (<28 days or ≥28 days).

Results: Of the 108 eligible children, 29 had UV physiology and 79 had BV physiology. Both groups showed impaired growth, with significantly lower body weights in the UV group. Neurodevelopmental scores (total DQ) were significantly lower in the UV group. Neurodevelopmental impairment (total DQ < 85) was observed in 44/108 (40.7%) children, and after adjustment, UV repair was significantly associated with neurodevelopmental impairment (adjusted odds ratio [OR] 3.11, 95% confidence interval [CI] 1.27-7.65). Timing of the initial cardiopulmonary bypass surgery was not associated with outcomes.

Conclusion: Infants with CHD in Japan exhibit impaired growth and neurodevelopment at 18-22 months following cardiopulmonary bypass surgery, especially those with UV physiology.

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.

Introduction: There is limited knowledge on titration, optimal dosing, and efficacy of angiotensin-converting enzyme inhibitors in paediatric patients following cardiac surgery.

Methods: Patients after cardiac surgery to repair ventricular septal defect or coarctation of the aorta from 01/2017 to 12/2019 were eligible for a retrospective single-centre study. Medical records were reviewed for patient characteristics and outcomes. Mean arterial pressure response and angiotensin-converting enzyme inhibitor dosage were collected. Controls were patients not receiving angiotensin-converting enzyme inhibitor postoperatively. Appropriate statistics were used for analysis.

Results: Among a total of 286 patients [n = 188 (66%) ventricular septal defect; n = 98 (34%) coarctation of the aorta], 170 (59%) received angiotensin-converting enzyme inhibitor on any postoperative day 1 to 5. The median age was 4.9 months (IQR 1.2-14.4) and weight 5.5 kg (IQR 3.7-9.2). The most common angiotensin-converting enzyme inhibitor was captopril on day 1 [n = 117 (69%)] and lisinopril at discharge [n = 86 (51%)]. Patients in treatment group were shown to have higher median mean arterial pressure at baseline and at time 1, compared with controls (mean difference 3.57 (95% CI: 1.85, 5.35) and 3.46 (95% CI: 1.41, 5.50), respectively. Median mean arterial pressure among controls significantly increased over time with a slope of 0.97 (95% CI: 0.2, 1.74), while median mean arterial pressure among treatment group decreased with a slope of -0.31 (-0.93,0.31). Patients who received high and medium doses of angiotensin-converting enzyme inhibitor showed significantly decreasing median mean arterial pressure over time with a slope of -2.85 (-5.14, -0.56) and -1.25 (-2.4, -0.11), respectively.

Conclusion: High and medium dose angiotensin-converting enzyme inhibitor therapy had a greater effect in decreasing mean arterial pressure when compared to low dose.

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.