Cardiology in the Young最新文献

A 69-year-old man had an abnormal intracardiac course of a pacemaker lead. CT angiography demonstrated a window between the right upper pulmonary vein and the superior caval vein. The window was treated with covered stents in the superior caval vein, which was complicated by a chronic pericardial effusion that was treated with a pericardial window 6 months later.

Objectives: We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

Methods: A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan-Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Results: Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA- patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan-Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA- patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA- patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA- p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy-Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Conclusions: Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

Dilated cardiomyopathy is an expected manifestation and common cause of death in patients with Duchenne muscular dystrophy. We present an unusually rapid progression of cardiomyopathy in a boy with Duchenne muscular dystrophy. Expanded genetic testing revealed a contiguous Xp21 deletion involving dystrophin and XK genes, responsible for Duchenne muscular dystrophy and McLeod neuroacanthocytosis syndrome, respectively, resulting in a more severe cardiac phenotype.

Objective: Syncope is common among pediatric patients and is rarely pathologic. The mechanisms for symptoms during exercise are less well understood than the resting mechanisms. Additionally, inert gas rebreathing analysis, a non-invasive examination of haemodynamics including cardiac output, has not previously been studied in youth with neurocardiogenic syncope.

Methods: This was a retrospective (2017-2023), single-center cohort study in pediatric patients ≤ 21 years with prior peri-exertional syncope evaluated with echocardiography and cardiopulmonary exercise testing with inert gas rebreathing analysis performed on the same day. Patients with and without symptoms during or immediately following exercise were noted.

Results: Of the 101 patients (15.2 ± 2.3 years; 31% male), there were 22 patients with symptoms during exercise testing or recovery. Resting echocardiography stroke volume correlated with resting (r = 0.53, p < 0.0001) and peak stroke volume (r = 0.32, p = 0.009) by inert gas rebreathing and with peak oxygen pulse (r = 0.61, p < 0.0001). Patients with syncopal symptoms peri-exercise had lower left ventricular end-diastolic volume (Z-score -1.2 ± 1.3 vs. -0.36 ± 1.3, p = 0.01) and end-systolic volume (Z-score -1.0 ± 1.4 vs. -0.1 ± 1.1, p = 0.001) by echocardiography, lower percent predicted peak oxygen pulse during exercise (95.5 ± 14.0 vs. 104.6 ± 18.5%, p = 0.04), and slower post-exercise heart rate recovery (31.0 ± 12.7 vs. 37.8 ± 13.2 bpm, p = 0.03).

Discussion: Among youth with a history of peri-exertional syncope, those who become syncopal with exercise testing have lower left ventricular volumes at rest, decreased peak oxygen pulse, and slower heart rate recovery after exercise than those who remain asymptomatic. Peak oxygen pulse and resting stroke volume on inert gas rebreathing are associated with stroke volume on echocardiogram.

Background: Despite patent ductus arteriosus closure in premature infants is a relatively fast procedure, it involves specific steps for equipment exchange and the use of a 4-Fr catheter, which may be large for tiny infants, potentially causing haemodynamic instability or tricuspid valve regurgitation.

Objectives: This study aims to describe the early experience with a new technique designed to simplify transcatheter patent ductus arteriosus closure in premature infants. It enables the closure using the KA micro plug device via a single microcatheter without equipment exchanges and contrast exposure. This technique involves a simple modification of the microcatheter by creating a curved tip.

Methods: This is a retrospective, single-institution study involving premature infants who underwent attempted transcatheter patent ductus arteriosus closure using a manually modified microcatheter of the KA micro plug device at Stead Family Children's Hospital.

Results: Five patients underwent transcatheter patent ductus arteriosus closure using a KA micro plug with a modified microcatheter. The procedure was successful in all patients, with no haemodynamic instability. The median (range) for procedural weight, time, and radiation dose were 1055 g (730-1330), 12 minutes (11-19), and 0.49 µGy.m² (0.14-1), respectively. At a median follow-up of 1.1 months (0.17-2.6), all patients maintained good device position without residual shunt or other concerning echocardiogram findings.

Conclusions: Transcatheter patent ductus arteriosus closure in infants weighing less than 1500 g can be successfully performed using a KA micro plug device through a microcatheter with zero-contrast use, low radiation exposure, and a short procedure time.

Objective: This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.

Methods: Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms. The prevalence of veno-venous collaterals, the risks for the development of veno-venous collaterals, and the impact of veno-venous collaterals on outcomes were analysed.

Results: In total, 586 patients were included. Veno-venous collaterals were found in 72 (12.3%) patients. Majority of veno-venous collaterals originated from the superior caval vein and drained into the inferior caval vein. Before bidirectional cavopulmonary shunt, mean pulmonary artery pressure (16.3 vs. 14.5 mmHg, p = 0.018), and trans-pulmonary gradient (9.5 vs. 8.0 mmHg, p = 0.030) were higher in patients with veno-venous collaterals compared to those without. Veno-venous collaterals intervention was performed in 32 (5.5%) patients, in a median of 29 (16-152) days after bidirectional cavopulmonary shunt. Before total cavopulmonary connection, pulmonary artery pressure (10.3 vs. 9.4 mmHg, p = 0.015) and ventricular end-diastolic pressure (8.4 vs. 7.6 mmHg, p = 0.035) were higher, and arterial oxygen saturation (SaO₂, 80.6 vs. 82.6 %, p = 0.018) was lower in patients with veno-venous collaterals compared to those without. More palliations before total cavopulmonary connection (p < 0.001, odds ratio: 1.689) were an independent risk for the development of veno-venous collaterals. Veno-venous collaterals did not affect survival after total cavopulmonary connection (92.8 vs. 92.7% at 10 years, p = 0.600).

Conclusions: The prevalence of veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection was 12%. Veno-venous collaterals may be induced by the elevated pulmonary artery pressure and trans-pulmonary gradient, and also by more previous palliations. However, they had no impact on clinical outcomes following total cavopulmonary connection.

Background: Transcatheter closure of large and complex atrial septal defect can pose challenges and complications during device placement. To improve stability, several assistive techniques have been developed.

Methods: This retrospective study evaluated the efficacy of the device-assisted device closure technique for large secundum atrial septal defects. Patients who underwent device-assisted device closure of atrial septal defect between December 2023 and August 2024 were analysed.

Results: Twenty patients (mean age 38.69 years) underwent device closure of large secundum atrial septal defect with device-assisted device closure technique. The mean atrial septal defect diameter was 31.9 mm. The average thick-to-thick measurement was 38.3 mm, which determined the device size. The majority (18 cases) had thin, floppy margins and two had deficient inferior rim. Successful closure was achieved in 18 patients (90%), while two patients (10%) required other methods of assistance. Based on fluoroscopic guidance, patients were divided into two groups: Group A (8 patients) used anteroposterior projection, and Group B (12 patients) used left anterior oblique-cranial view. After initial two failures with anteroposterior view, all cases were successfully closed using left anterior oblique-cranial projection. Device sizes ranged from 36 to 50 mm (median 40 mm). Cocoon devices were used for sizes up to 42 mm, and Occlunix for larger devices. No significant procedural complications occurred, although two patients had minor post-procedural events.

Conclusions: Device-assisted device closure technique offers a promising and safe dynamic assistance approach for transcatheter closure of large and challenging atrial septal defects. The left anterior oblique-cranial view showed promising results, though without statistical significance. While results are encouraging, larger prospective studies are needed to validate its effectiveness.

Background: Guidelines recommend screening for psychiatric co-morbidities in patients with congenital heart defects alongside cardiac outpatient follow-ups. These recommendations are not implemented in Denmark. This study aimed to investigate the psychiatric co-morbidities in children and adolescents with Fontan circulation in Denmark and to evaluate the feasibility of an online screening measure for psychiatric disorders.

Methods: Children, adolescents, and their families answered the Development and Well-Being Assessment questionnaire and a questionnaire about received help online. Development and Well-Being Assessment ratings present psychiatric diagnoses in accordance with ICD-10 and DSM-5. Parent-reported received psychiatric help is also presented. Feasibility data are reported as participation rate (completed Development and Well-Being Assessments) and parental/adolescent acceptability from the feasibility questionnaire.

Results: The participation rate was 27%. Of the participating children and adolescents, 53% (ICD-10)/59% (DSM-5) met full diagnostic criteria for at least one psychiatric diagnosis. Of these, 50% had not received any psychiatric or psychological help. Only 12% of participants had an a priori psychiatric diagnosis.

Conclusions: We found that a large proportion of children and adolescents with Fontan circulation are underdiagnosed and undertreated for psychiatric disorders. The results from our study emphasise the need for psychiatric screening in this patient group. Development and Well-Being Assessment may be too comprehensive for online electronic screening in children and adolescents with CHD.

Hybrid procedure of hypoplastic left heart syndrome, comprising ductus arteriosus stenting and bilateral pulmonary artery banding, is a good surgical option for initial palliative procedure for high-risk patients for Norwood procedure. However, ductal stenting may cause retrograde aortic blood flow obstruction. Furthermore, complete removal of stent while performing the Norwood procedure make the operation more difficult. We report a case that overcame these problems using a novel surgical technique.

Objective: Children with congenital adrenal hyperplasia are considered to be at an elevated risk for cardiovascular morbidity and mortality. The objective of this study was to evaluate the association between periaortic fat thickness and the cardiometabolic profile in children diagnosed with congenital adrenal hyperplasia.

Method: A total of 20 children and adolescents with congenital adrenal hyperplasia and 20 healthy control subjects were enrolled in the study. We investigated metabolic and anthropometric parameters, comparing these values to those of the control group. Periaortic fat thickness was assessed using an echocardiographic method that has not previously been applied to paediatric patients with congenital adrenal hyperplasia.

Results: The subjects in the congenital adrenal hyperplasia group were significantly shorter than the control subjects (p = 0.021) and exhibited a higher body mass index (p = 0.044) and diastolic blood pressure (p = 0.046). No significant differences were observed between the congenital adrenal hyperplasia group and control subjects concerning age, weight, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol levels. Additionally, dyslipidemia was identified in 5% (N = 1) of the congenital adrenal hyperplasia group. The mean fasting glucose, fasting insulin, homeostasis model assessment of insulin resistance, and fasting glucose-to-fasting insulin ratio were similar between the congenital adrenal hyperplasia group and the control subjects. However, 15% (n = 3) of the congenital adrenal hyperplasia group had insulin resistance. Two children with congenital adrenal hyperplasia (10%) were diagnosed with hypertension.Periaortic fat thickness was significantly greater in the congenital adrenal hyperplasia group compared to the control group (p = 0.000), with measurements of 0.2039 ± 0.045 mm in the congenital adrenal hyperplasia group and 0.1304 ± 0.022 mm in the control group. In children with congenital adrenal hyperplasia, periaortic fat thickness exhibited a negative correlation with high-density lipoprotein cholesterol (r = -0.549, p = 0.034) and a positive correlation with the dose of hydrocortisone (r = 0.688, p = 0.001).

Conclusion: Our results provide further evidence of subclinical cardiovascular disease in children with congenital adrenal hyperplasia. It is crucial to regularly assess cardiometabolic risk in children with congenital adrenal hyperplasia. The measurement of periaortic fat thickness in this population may serve as a valuable tool for identifying individuals at high risk for developing early atherosclerosis.