Cardiology in the Young最新文献

We described a 14-year-old boy who underwent catheter ablation for atrial tachycardia that had difficulty in creating the whole circuit of 3D map due to widely spread scar after repeated surgery for multivalvular heart disease. The classical atrial entrainment method was very effective in planning the catheter ablation for the invisible circuit of the atrial tachycardia.

Background: Right atrial appendage aneurysm, or giant right atrial appendage, is extremely rare, with very few cases reported in scientific literature. We sought to systematically review the published cases of right atrial appendage aneurysm in terms of age, sex, clinical presentation, electrocardiography, imaging (chest X-ray, echocardiography, CT/cardiac magnetic resonance), and outcome.

Methodology: An electronic search for case reports, case series, and related articles published until March 2025 was carried out, and clinical data were extracted and analysed.

Results: Forty-four cases of right atrial appendage aneurysm were identified with a clear male prevalence (68.2%) and commonly presenting in the third decade of life. Palpitation (27.3%) and dyspnoea (18.2%) were the most common clinical presentations, whereas 40.9% of right atrial appendage aneurysm patients were asymptomatic. Electrocardiography was done in 77.3% of the sample. It displayed an atrial arrhythmia (atrial fibrillation or flutter, atrial tachycardia, supraventricular tachycardia) in 31.8%. A chest X-ray was done in 65.9%. Echocardiography was the most common diagnostic modality (93.2%). Right atrial appendage aneurysm diagnosis was confirmed on CT and/or MRI in 79.5%. The mean size of the right atrial appendage aneurysm was 93 × 70 mm. In 12 patients (27.3%), an associated congenital cardiac abnormality was found, mostly in the form of an atrial septal defect/patent foramen ovale (22.7%). Half of the patients (50.0%) were treated surgically, whilst 47.8% were treated medically with close follow-up. One patient experienced right atrial appendage aneurysm reduction in size after atrial septal defect device closure. One death (2.3%) was reported also.

Conclusion: Although very uncommon, right atrial appendage aneurysm can be linked to considerable morbidity. Surgical removal is recommended for patients who are symptomatic.

This case describes a 32-year-old adult male who was incidentally diagnosed with isolated dextrocardia, Double inlet left ventricle (l-looped), l-transposed great arteries with subpulmonary stenosis during evaluation for an orbital abscess. This case highlights protective factors that enabled this patient's survival into adulthood without cardiac surgeries or medications despite single ventricle physiology, namely his "self-banded" pulmonary flow.

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.

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.

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.

Dextro-transposition of the great arteries is a critical CHD traditionally considered sporadic, with low familial recurrence. Emerging evidence suggests a genetic component in select cases, particularly with rare familial clustering. We report concordant d-TGA in monochorionic diamniotic twins, a highly unusual occurrence, strengthening the argument for a heritable predisposition.

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.

Aberrant subclavian artery arising from the pulmonary artery is an extremely rare anomaly of the aortic arch and is often associated with CHD. It can remain asymptomatic or result in subclavian or pulmonary steal syndrome. We present three cases with aberrant subclavian arteries (two left-sided, one right-sided) and reviewed 44 case reports through an extensive PubMed research to contextualise our findings to the existing literature. Fifty-five per cent of patients had a left, 45% had a right aberrant subclavian artery. The majority of patients (86%) had associated CHD. Blood pressure discrepancies and imaging-particularly transthoracic echocardiography, CT, and cardiac catheterisation-were pivotal in diagnosis. Aberrant subclavian arteries are a rare vascular anomaly, understanding of the embryology and anatomy is essential for the understanding of complex congenital heart variations. Early detection and surgical intervention are crucial to prevent complications.