Patent ductus arteriosus stenting in duct-dependent pulmonary circulation is a challenging procedure. Percutaneous carotid artery access for ductal stenting has proven to be feasible; however, comparison with femoral artery access in terms of procedure details and complications either immediate or late is scarce. Therefore, we evaluated carotid artery access in comparison with femoral artery for stenting of patent ductus arteriosus.

Forty neonates were reviewed, 20 were stented via carotid artery access, and 20 via the traditional femoral artery access. Comparison variables were neonatal demographics at the procedure, angiographic ductal anatomy, procedure details, and immediate complications. Follow-up Doppler ultrasound on access site was performed to document late complications.

Median age of included cases was 10.5 (3–28) days with complex ductal anatomy more frequently accessed via carotid artery than femoral. Immediate access-related complications were significantly higher with femoral than carotid artery access; 9 (45%) versus 3 (15%) respectively, p = 0.038. With carotid access, we had only one case with small pseudoaneurysm and acute hemiparesis 3 days after the procedure. Delayed local complications were more common with femoral access (15%) than carotid access (5%), mild stenosis in one case, and severe in another with femoral access; while with transcarotid arterial access, only one case had mild narrowing.

Percutaneous carotid artery access in neonates is a more convenient approach for patent ductus arteriosus stenting especially with complex ductal anatomy. Moreover, local complications are limited and vascular patency is better preserved, in comparison with trans-femoral arterial access. However, the potential for neurological adverse events should not be overlooked.

Systemic inflammation has been suggested to underlie in part the elevated risk of arrhythmias and myocardial dysfunction during the first weeks after cardiac surgery. Recent transthoracic Doppler studies from our centre indicated increased basal coronary arterial flow in children 5 days after cardiopulmonary bypass surgery. In these children, we investigated whether the inflammatory mediator, C-reactive protein, could influence this association.

The peak flow velocity, velocity time-integral in diastole and systole, and basal blood flow in the proximal part of the left anterior descending artery, were assessed by transthoracic Doppler echocardiography 1 day before, and 5 days after, cardiac surgery in 17 children with ventricular and atrioventricular septal defects whose mean age at surgery was 6 months. Levels of C-reactive protein in the plasma were measured at both time-points.

Prior to surgery, all children had levels of C-reactive protein under the limit for detection, that is less than 0.8 milligrams per litre. The levels of the protein had increased significantly by the second day, when the median value was 25, and the range from 20 to 142 milligrams per litre. They remained elevated on the fifth day after surgery, when the median was 11, and the range from 3 to 20 milligrams per litre. On the fifth day, the percentage increase in velocity time integral corrected for left ventricular mass was significantly lower in those patients with C-reactive protein greater than or equal to 10 milligrams per litre than in the remaining patients. Also, both the velocity time integral and the velocity of diastolic peak flow correlated inversely with log C-reactive protein, r being equal to −0.54 and p less than 0.02 and r equal to −0.74 and p less than 0.01, respectively, particularly among those patients in whom clamping of the aorta lasted for more than 1 hour, r for this statistic being equal to −0.8.

The postsurgical increase in the velocity of coronary arterial flow in children is inversely associated with rising levels of C-reactive protein. The duration of the aortic cross-clamping during surgery strengthens the association between levels of C-reactive protein and the microcirculatory changes.

IL-27, a member of the IL-12 family, has been involved in maternal tolerance to the foetus and successful pregnancy. Growing evidences indicate that IL-27 plays a crucial role in pregnancy.

We carried out the present study in order to investigate whether polymorphisms in the IL27 are associated with the risk for CHDs, including atrial septal defect and ventricular septal defect.

We conducted this case–control study among 247 atrial septal defect patients, 150 ventricular septal defect patients, and 368 healthy controls in a Chinese population using polymerase chain reaction-restriction fragment length polymorphism assay.

Significantly increased risk for atrial septal defect (p=0.001, OR=1.490, 95% CI=1.178–1.887) and ventricular septal defect (p=0.004, OR=1.502, 95% CI=1.139–1.976) was observed to be associated with the allele G of rs153109. In a dominant model, we have also observed that increased susceptibilities for atrial septal defect (p<0.01, OR=1.89, 95% CI=1.35–2.63) and ventricular septal defect (p<0.01, OR=2.50, 95% CI=1.67–3.85) were statistically associated with rs153109; however, no association was found between CHD risk and rs17855750 in the IL27 gene.

The 153109 of the IL27 gene may be associated with the susceptibility to CHD, including atrial septal defect and ventricular septal defect.

To describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels.

Endovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent.

We describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result.

Endovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.

This study aimed to assess the results of endomyocardial biopsy from the right ventricle to establish the possible cause for drug-refractory arrhythmias in children.

We enrolled 19 consecutive young patients with drug-refractory arrhythmia, from 2010 to 2013, who underwent endomyocardial biopsy. Inclusion criteria were as follows: age <18 years with a structurally normal heart or mild changes in a structure of the heart initially diagnosed as arrhythmia-induced cardiomyopathy. Overall, 86 biopsies were performed in 19 patients. Histopathological analysis, immunohistochemistry, and polymerase chain reaction were used for the interpretation of the endomyocardial biopsy.

The mean age of the patient population was 14.1±2.9 year (range from 7 to 17 years). All these patients had a history of drug-refractory arrhythmia for >5 months (mean 30 months). Patients underwent a complete history investigation, physical examination, laboratory studies, echocardiography, electrocardiography, treadmill test, and Holter monitoring before endomyocardial biopsy; two patients with arrhythmogenic right ventricular dysplasia had implantable cardioverter defibrillator implantation and further appropriate successful device shocks. Myocarditis was diagnosed based on histopathological and immunohistological analyses in nine (47.4%) patients. Polymerase chain reaction was positive for viral genome in four of them; five patients had active myocarditis. Radiofrequency ablation was performed in 17 patients; five out of six (83%) endomyocardial biopsy-proved myocarditis patients had successful radiofrequency ablation. No significant complication was reported during ablation and endomyocardial biopsy.

Approximately half of the children with drug-refractory arrhythmia had unsuspected myocarditis according to the results of the endomyocardial biopsy.

Mitral valvar prolapse is the most common anomaly of the mitral valve apparatus throughout childhood. Fibrillin is one of the structural components of the elastin-associated microfibrils found in the mitral valve. A case-controlled study has performed to investigate the relationship between fibrillin 1 gene intron 56 polymorphism and risk of mitral valvar prolapse in Turkish children.

A total of 77 patients with mitral valvar prolapse diagnosed by clinical evaluation and echocardiography and 89 normal children of same age and sex were studied. The fibrillin-1 gene intron 56 polymorphism was identified by the polymerase chain reaction-based restriction analysis.

There was a significant difference in the distribution of fibrillin-1 gene intron 56 genotypes (p = 0.0001) and allelic frequency (p = 0.0001) between the cases and the controls.

Patients with mitral valvar prolapse have higher frequencies of fibrillin-1 gene intron 56 GC genotypes. Healthy children have higher frequencies of fibrillin-1 gene intron 56 CC genotypes. We speculate that the higher frequency of fibrillin-1 gene intron 56 G-allele increases the risk of mitral valvar prolapse.

Canadian Cardiovascular Society consensus guidelines recommend that tetralogy of Fallot patients be seen by a congenital cardiologist every 2 years. In Atlantic Canada, tetralogy of Fallot patients are followed up at either tertiary or satellite clinics, which are held in the community and attended by paediatric cardiologists. The effectiveness of satellite clinics in congenital cardiac disease follow-up is unproven. Our objective was to compare patient-reported quality of life measures to determine whether these were impacted by the site of follow-up.

We included patients with tetralogy of Fallot undergoing surgical repair at the Izaak Walton Killam Health Centre from 1 November, 1972 to 31 May, 2002. Quality of life surveys, SF-10 or SF-36v2, were administered to consenting patients. We analysed the subjective health status by patient age and site of follow-up.

Of the 184 eligible patients, 72 were lost to follow-up. Of the locatable patients, 61% completed the questionnaires. In all, 90% (101 out of 112) were followed up at recommended intervals. Of the 112 (68%) patients, 76 were followed up at a tertiary clinic. These patients were older, with a mean age of 18.4 years versus 14.7 years, and scored higher on the SF-36 physical component summary (52.6 versus 45.7, p = 0.02) compared with satellite clinic patients. The SF-36 mental component summary scores were similar for patients regardless of the site of follow-up. SF-10 physical and psychosocial scores were similar regardless of the site of follow-up.

Tetralogy of Fallot patients followed at either satellite or tertiary clinics have similar subjective health status.

To assess the effectiveness of radiofrequency catheter ablation for lone atrial fibrillation in young adults.

This single-centre, retrospective, observational study enrolled 75 consecutive patients (86.7% men) under 35 (median, 30) years old with lone atrial fibrillation (68% paroxysmal, 26.7% persistent, and 5.3% long-standing persistent) without other cardiopulmonary diseases who underwent catheter ablation between April 2009 and May 2017. Procedural endpoints were circumferential pulmonary vein ablation for atrial fibrillation with pulmonary vein trigger, and target ablation or bidirectional block of lines and disappearance of complex fractionated atrial electrograms for atrial fibrillation with clear and unclear non-pulmonary vein triggers, respectively.

Main study outcome was rate of survival free from atrial tachyarrhythmia recurrence, which at median 61 (range, 5–102) months follow-up was 62.7% (64.7 and 58.3% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after single ablation, and 69.3% (68.6 and 70.8% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after mean 1.2 ablations (two and three ablations in 11 and 2 patients, respectively). In multivariate analysis, non-pulmonary vein trigger was a significant independent predictor of recurrent atrial tachyarrhythmia (OR, 10.60 [95%CI, 2.25–49.96]; p = 0.003). There were no major periprocedural adverse events.

In patients under 35 years old with lone atrial fibrillation, radiofrequency catheter ablation appeared effective particularly for atrial fibrillation with pulmonary vein trigger and regardless of left atrial size or atrial fibrillation duration or type. Atrial tachyarrhythmia recurrence after multiple ablations warrants further study.

This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention.

All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data. Diagnosis was confirmed by reviewing echo studies. Critical aortic stenosis was defined as valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function. Primary outcome was all-cause mortality and secondary outcomes were reintervention and aortic valve replacement.

Sixty-one patients were identified (50 boys, 11 girls). Primary treatment was surgical valvotomy in 52 neonates and balloon valvotomy in 6. Median age at initial treatment was 5 days (0–26), and median follow-up time was 10.8 years (0.14–22.6). There was no 30-day mortality but four late deaths. Freedom from reintervention was 66%, 61%, 54%, 49%, and 46% at 1, 5, 10, 15, and 20 years, respectively. Median time to reintervention was 3.4 months (4 days to 17.3 years). Valve replacement was performed in 23 patients (38%).

Surgical valvotomy is a safe and reliable treatment in these critically ill neonates, with no 30-day mortality and long-term survival of 93% in this national study. At 10 years of age, reintervention was performed in 54% and at end of follow-up 38% had had an aortic valve replacement.