Cardiology in the Young最新文献

Neonatal truncal valve insufficiency carries high postoperative mortality. Mechanical replacement is challenging, and leaflet-sparing repair alone may be insufficient due to fragile tissue and annular dilatation. We report a successful case using geometry-based root reconstruction guided by body-weight-adjusted reference values. This native-tissue approach restored physiologic root geometry, preserved the valve, and enabled growth potential, demonstrating a feasible strategy for durable neonatal repair.

Pulmonary valve or main pulmonary artery infective endocarditis is rare in children and is associated with high morbidity and mortality. Fungal infective endocarditis, most commonly caused by Candida species, is particularly aggressive and often requires a combination of antifungal therapy and surgical intervention. We report two infants who developed Candida endocarditis following pulmonary artery banding.In the first case, a female infant developed persistent candidemia after pulmonary artery banding, with echocardiography revealing a mobile mass at the pulmonary bifurcation and computed tomography angiography demonstrating a mycotic pseudoaneurysm. Blood cultures confirmed Candida albicans. She underwent debanding and main pulmonary artery reconstruction, later requiring pacemaker implantation, and recovered without relapse. In the second case, a female infant presented with fever and candidaemia after pulmonary artery banding. Echocardiography identified a distal main pulmonary artery vegetation, and cultures grew Candida parapsilosis. She received amphotericin B-based induction therapy followed by fluconazole step-down after surgical source control, achieving clinical cure at follow-up.These cases highlight the diagnostic and therapeutic challenges of Candida endocarditis after right-sided palliation. Early multimodality imaging, species-directed antifungal therapy, and timely surgery are critical to optimise outcomes in this rare but life-threatening complication.

Background: The impact of absent ductus arteriosus in fetuses with Tetralogy of Fallot remains uncertain with concerns for the lack of prostaglandin responsiveness and compromised pulmonary blood flow. This study evaluated early postnatal outcomes in fetuses with Tetralogy of Fallot-absent ductus arteriosus compared to matched controls with a ductus arteriosus and assessed the predictive value of fetal and neonatal echocardiographic parameters for early intervention.

Methods: A retrospective matched cohort study was performed using a single-centre fetal echocardiography database (2000-2024). Fetuses with Tetralogy of Fallot-absent ductus arteriosus confirmed by neonatal echocardiography were matched 1:1 to Tetralogy of Fallot-ductus arteriosus cases by fetal pulmonary valve Z-score and gestational age. Early intervention was defined as any surgical or transcatheter procedure to augment pulmonary blood flow within six weeks of life. Comparative analyses evaluated predictors of early intervention within each cohort.

Results: Among 253 fetuses with Tetralogy of Fallot and antegrade outflow, 27 had confirmed absent ductus arteriosus and were matched to 27 Tetralogy of Fallot-ductus arteriosus controls. Despite similar fetal pulmonary valve Z-scores, Tetralogy of Fallot-absent ductus arteriosus patients had significantly lower neonatal pulmonary valve and main pulmonary artery Z-scores and main pulmonary artery-to-aorta ratios. Early intervention occurred in 26% of Tetralogy of Fallot-absent ductus arteriosus and 41% of Tetralogy of Fallot-ductus arteriosus (p = 0.25); surgical intervention was more frequent in Tetralogy of Fallot-absent ductus arteriosus (86 vs 36%, p = 0.066). In Tetralogy of Fallot-ductus arteriosus, lower fetal and neonatal pulmonary valve/aortic valve ratios predicted early intervention. No fetal or neonatal markers were predictive in Tetralogy of Fallot-absent ductus arteriosus.

Conclusions: Fetal absent ductus arteriosus was not linked to higher early intervention rates in Tetralogy of Fallot, but when intervention was required, surgical palliation predominated. Conventional fetal echocardiographic predictors were not reliable in Tetralogy of Fallot-absent ductus arteriosus, complicating prenatal counselling.

Hepatopulmonary fusion is a rare defect describing the physical connection of liver and lung. A neonate with hypoplastic left heart syndrome arrived at the hospital with concern for congenital diaphragmatic hernia, later found to have hepatopulmonary fusion. In the coming months, he underwent a series of operations, including ligation of the hepatopulmonary fusion and the first two stages of surgical palliation of hypoplastic left heart syndrome. He ultimately died of septic shock at 15 months of age.

Pericardial cysts are rare, benign congenital cardiovascular malformations that account for approximately 7% of mediastinal masses. Epicardial cysts attached to the cardiac surface with intimate coronary artery involvement are even rarer and pose significant diagnostic and surgical challenges. This case highlights a giant pericardial cyst with intimate right coronary artery involvement in a 10-month-old infant, where subtotal resection was necessary to preserve coronary integrity. A 10-month-old male infant with a pericardial cyst initially detected at 27 weeks of gestation presented with progressive compression of right heart chambers. Imaging revealed a large multiloculated cystic mass (5.3 × 3.5 × 3.9 cm) compressing the right atrium and right ventricle, with associated pulmonary valve stenosis. Intraoperatively, the cyst was found on the epicardial surface with intimate involvement of the right coronary artery. Complete excision was not feasible due to the risk of coronary injury. The main cystic mass was excised with cavity obliteration, while the portion adjacent to the right coronary artery was intentionally preserved. Concurrent pulmonary valve commissurotomy and pulmonary artery augmentation were performed. Histopathology confirmed a mesothelial-lined pericardial cyst. The patient recovered uneventfully and was discharged. This case underscores the importance of comprehensive preoperative coronary artery assessment in pericardial cysts with atypical locations. When complete excision risks vital structure injury, subtotal resection with cavity obliteration represents a safe alternative strategy.

We report two adult patients who had rare systemic artery lesions with a history of Kawasaki disease, with a review of the literature. A 39-year-old male with Kawasaki disease at the age of 2 months underwent coronary artery bypass grafting to the right coronary artery and left anterior descending artery because of coronary artery stenoses. In the screening by thoracic-abdominal CT before the operation, calcification of the distal abdominal aorta was detected, and an abdominal aortic aneurysm with stenosis was diagnosed. He had been asymptomatic, and there were no differences in his extremities' blood pressure. Although a slight dilatation of the distal abdominal aorta had existed in his aortogram at 2 years 9 months old, the abnormal finding had been unnoticed. A 26-year-old female with coronary artery bypass grafting at the age of 23 months had hypertension. A stenosis of the orifice at the right renal artery was detected by two-dimensional echocardiography and a magnetic resonance angiogram. Her hypertension in adulthood was suspected to result from her renal artery stenosis, which acute Kawasaki vasculitis underlies. Although coronary artery lesions in both patients were diagnosed in their childhood, systemic artery lesions were not realised until adulthood. Because abdominal aortic aneurysms and renal artery stenosis are extremely rare, they were not recognised as systemic artery lesions caused by Kawasaki disease. Their lesions can occur in patients with severe acute vasculitis at a significantly younger age, less than 6 months. Systemic artery lesions in such adult patients must be carefully evaluated.

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.