Cardiology in the Young最新文献

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.

As staged palliative surgery for patients with hypoplastic left heart syndrome ages to greater than 50 years in practice, providers are increasingly faced with the challenges of managing patients with "failed Fontan" physiology. In this brief case report, we discuss different ways paediatric palliative care can support this population.

Aim: This study aimed to evaluate the characteristics, difficulties, and outcomes of patients who underwent transcatheter ablation treatment due to arrhythmia with a diagnosis of CHD.

Methods: A total of 166 patients (189 substrates) with CHD who underwent catheter ablation between November 2013 and 2023 were evaluated retrospectively. EnSite™ (St Jude Medical Inc., St Paul, MN, USA) was used in all patients.

Results: The mean age was 14.8 ± 7.9 years (2.9-43 years). The most common CHD's were Ebstein anomaly (n: 40), tetralogy of Fallot (n: 31), atrial septal defect (n: 25), ventricular septal defect (n: 22), great artery transposition (D/L TGA, n: 12), and complex CHD in single ventricle physiology (n: 9). The most common arrhythmia mechanisms were Wolf-Parkinson-White syndrome (WPW, n: 50), intraatrial reentrant tachycardia (IART, n: 39), typical atrioventricular nodal reentrant tachycardia (AVNRT, n: 37), and ventricular tachycardia-ventricular extrasystoles (VT/VES, n: 23). There was more than one arrhythmia in 23 patients and multiple manifest accessory pathways in 10 patients. The average procedure time was 174 ± 69.3 minutes, and the average fluoro time was 8.3 minutes. While successful ablation was performed in 176/189 (acute success 93.1%) substrates, the procedure was unsuccessful in five patients and suboptimal in eight patients. Recurrence was observed in 11 patients (6.4%) during a mean follow-up period of 49.2 ± 30.1 months. A second ablation was performed on 13 patients. Acute success was achieved in all except one patient. A total of 11 patients are being followed up with medical treatment.

Conclusion: Despite the complex anatomy, age, operations, and limited vascular access possibilities in patients diagnosed with CHD, transcatheter ablation treatment with advances in electrophysiology, the introduction of different energy types, special ablation catheters, multipolar mapping catheters, and 3D nonfluoroscopic mapping systems seems to be a safe and effective option.

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.

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.

Background: Impaired muscle function, aerobic capacity, and fatigue are common in individuals with Fontan circulation. Knowledge regarding the effects of strength training in this population is limited. Therefore, the study aimed to investigate the effects of strength training on dynamic muscle function, aerobic capacity, and fatigue in adults with Fontan circulation compared to matched controls.

Methods: In this pilot non-randomised controlled trial, nine patients with Fontan circulation (median age 28.9 years [IQR: 23.4-35.0], 44.4% women) and nine age- and sex-matched controls completed a 10-week strength training intervention. Dynamic muscle function was assessed through shoulder flexion, heel rise, elbow flexion, and knee extension tests. Aerobic capacity was evaluated using cardiopulmonary exercise testing, and fatigue using the questionnaire Multidimensional Fatigue Inventory. All assessments were conducted pre- and post-intervention. Within-group changes were analysed using the Wilcoxon signed rank test and between-group differences using the Mann-Whitney U test.

Results: Patients showed improvements in all muscle function tests post-intervention (shoulder flexions 39.3% [IQR: 18.9-69.7], p = 0.008; heel rise 26.7% [IQR:17.5-58.1], p = 0.008; elbow flexions 57.1% [IQR: 50.0-173.8], p = 0.007; knee extensions 66.7% [24.3-92.9], p = 0.008). The improvements were at comparable levels to controls. Only controls reported reduced fatigue (-19.4% [IQR: -28.7, -10.5], p = 0.01), while patients showed no change (-5.9% [IQR: -25.5, 3.2], p = 0.1). Aerobic capacity remained unchanged. No severe adverse events occurred.

Conclusion: Strength training is safe and improves dynamic muscle function in patients with Fontan circulation, with changes comparable to those of healthy controls. However, the effect of strength training on fatigue and aerobic capacity requires further investigation.ClinicalTrials.gov, ID: NCT05454254, https://clinicaltrials.gov.

Background: Dynamic, data-driven predictors of perioperative mortality risks in preterm/early-term neonates with CHD undergoing cardiac surgery in the first 24 months of life are limited.

Aims: To identify risk factors of mortality in the first 24 months of life for pre/early-term neonates with CHD.

Methods: Retrospective cohort study of patients <39 weeks of gestation undergoing cardiac surgery within 24 months of life from 2013-2020 at a tertiary care centre. Independent risk factors of mortality within 24 months of life were determined by multivariable Cox regression analysis.

Results: Among the 205 neonates, 33 (16.1%) died within 24 months. Multivariable analysis revealed that high-frequency ventilation (hazard ratio = 5.15; 95% confidence interval): 2.51, 10.6; p < 0.001), extracorporeal membrane oxygenation support (hazard ratio = 5.77; 95% confidence interval: 2.67, 12.5; p < 0.001), and CHD with a palliated circulation (hazard ratio = 6.07; 95% confidence interval: 2.84, 13; p < 0.001) were significant independent risk factors of mortality at any time during the index hospitalisation or the first 24 months of life.

Conclusions: Identifying and re-evaluating risk factors of mortality for preterm/early-term neonates with CHD at any time during the index hospitalisation or the first 24 months of life may guide resource allocation and therapeutic interventions.Trial registration number and date of registration: IRB P00028833 5/2/2018. Retrospectively registered.

This case report details the diagnosis and surgical management of a 7 cm aortic root aneurysm occurring in a 12-year-old with cutis laxa type B, an extremely rare connective tissue disorder. Our patient underwent a Bentall procedure as primary treatment for the aneurysm and had a successful interventional treatment of a postoperative pseudoaneurysm.

Pseudoaneurysm after valve-sparing aortic root replacement is uncommon but potentially life-threatening. We report an unusual case of spontaneous resolution of an aortic root pseudoaneurysm that developed in a 14-year-old boy with Marfan syndrome following valve-sparing aortic root replacement. This case suggests that, in carefully selected situations, non-surgical management with intensive follow-up may be beneficial.

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.