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Background. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital heart disease. Adaptive development of sufficient heterocoronary collaterality in the newborn may allow survival to a later age. In older children or adults, malignant ventricular arrhythmias can reveal the disease. Case Report. A 15-year-old girl was referred to the local hospital after a resuscitated out-of-hospital cardiac arrest. CT scan and coronary angiography revealed an ALCAPA. Direct aortic reimplantation of the left coronary artery was performed. Postoperative ECG monitoring showed short episodes of nonsustained ventricular tachycardia. Transthoracic echocardiography and cardiac MRI revealed subendocardial fibrosis of the anterolateral papillary muscle. Beta-blockade therapy was initiated at first intention. After hospital discharge, the patient reported several fainting without loss of consciousness. Considering sudden death nonrelated to effort, episodes of nonsustained ventricular tachycardia, and areas of myocardial fibrosis, the patient underwent subcutaneous cardioverter-defibrillator implantation. 6-month follow-up is satisfactory without clinical or rhythmic abnormalities. Discussion. Indication for surgical correction of ALCAPA is well defined, but rhythmic secondary prevention after resuscitated cardiac arrest is less consensual. Cardiac MRI is an essential tool in the identification of a potential rhythmic substrate and should be taken into account in the discussion of a preventive cardioverter-defibrillator implantation.

OBJECTIVES Pulmonary valve replacement (PVR) performed for pulmonary valve regurgitation is the most common indication for reoperation during mid-to-long-term follow-up after tetralogy of Fallot repair. An aneurysmal dilation of the infundibulum is often associated secondary to the infundibulotomy performed in the first operation. The right ventricular outflow tract reconstruction with endo-exclusion aims to exclude the non-contractile segments of the dilated right ventricular. This study intends to assess the safety and efficiency of the endo-exclusion technique. METHODS Between January 2010 and December 2018, 86 patients underwent a PVR with (n = 46) or without (n = 40) endo-exclusion. The current study compares the outcomes in terms of survival, reintervention, structural valve deterioration, right ventricular function (volume and right ventricular ejection fraction) and pulmonary valve gradient. The median follow-up time was 4.45 years (1.9 months to 9.87 years). RESULTS There was no 30-day mortality. There was no difference in the freedom from reintervention at 7 years (without endo-exclusion, 97%, versus with endo-exclusion, 94%, log-rank = 0.68) or in the freedom from structural pulmonary valve deterioration at 7 years (without endo-exclusion, 94%, versus with endo-exclusion, 89%, log-rank = 0.94). No significant difference was observed in the indexed right ventricular end-diastolic volume (102.2 ± 34 ml/m2 in the PVR without endo-exclusion group and 93.3 ± 22 ml/m2 in the PVR with endo-exclusion group, P = 0.61). No significant difference was observed in the right ventricular function (right ventricular ejection fraction: 46 ± 11% in the PVR without endo-exclusion group and 46 ± 9% in the PVR with endo-exclusion group, P = 0.88). CONCLUSIONS PVR with or without endo-exclusion is a safe and effective procedure. PVR with endo-exclusion allows implantation without structural deformation of the valve and therefore excellent short- and medium-term results.

Background:Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock–Taussig shunt. We compared the clinical outcomes of the two procedures.Methods:From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock–Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth.Results:After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock–Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock–Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63).Conclusions:The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock–Taussig shunt for rate of biventricular repair and time to biventricular repair.

Summary Background Closure of patent foramen ovale is well-managed in adults, but is performed less frequently in children. Aim To analyse all patent foramen ovale closures performed in the past 20 years in French paediatric centres. Methods Retrospective study of patent foramen ovale closures in children without cardiopathy in nine centres between 2000 and 2019. Results Forty-one procedures were carried out in children (median age: 14.9 years). Thirty-one patent foramen ovales were closed after a transient ischaemic attack or stroke, six for a left-to-right shunt and four for other reasons. Transthoracic echocardiography was used for 72.2% of the diagnoses and transoesophageal echocardiography for 27.8%. A substantial degree of shunting was found in 42.9% of patients and an atrial septal aneurysm in 56.2%. General anaesthesia with transoesophageal echocardiography guidance was performed in 68.3% of the procedures; local anaesthesia and transthoracic echocardiography or intracardiac echocardiography was performed in 31.7%. The success rate was 100%. The median fluoroscopy time was 4.14 minutes: 3.55 minutes with transoesophageal echocardiography; and 4.38 minutes with transthoracic echocardiography (P = 0.67). There was only one periprocedural complication (2.4%). Postoperatively, 80,5% of patients were treated with aspirin and 12,2% with an anticoagulant. The rate of complete occlusion was 56.8% immediately after the procedure, 68.6% at 1 year and 92.3% at the last follow-up. There were no delayed complications or cases of recurrent stroke during follow-up (median follow-up: 568 days). Conclusion Closure of patent foramen ovale in children appears to be safe and effective, as we noted a low rate of immediate complications, no delayed complications and no stroke recurrence in this indication.

Left atrioventricular valve aneurysm is rare. We present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. The preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the “cleft” and leaflet perforation. But in per-operative, we discovered a left sided atrioventricular valve aneurysm instead of valve perforation. The “cleft” edge and a aneurysm were closed.

Catheter ablation is a safe and effective treatment modality for ventricular arrhythmia. The procedure is routine at many electrophysiology centers and is being undertaken ever earlier in patient care.1 However, the great majority of these procedures involve adults suffering from ischemic heart disease. Only 3 cases of transapical access catheter ablation of ventricular tachycardia (VT) have been reported in children of under 15 months of age.2, 3, 4 Such procedures are technically difficult because the catheters available are ill-suited to the small-sized heart of a child. We report a case of a 10-month-old toddler in whom life-saving ablation of VT was performed via a percutaneous femoral approach with transseptal access to the left ventricle (LV).

Background Percutaneous treatment of native coarctation (coA), gold standard in adult patients, is still debated in children. We aim to report the French multicentre experience. Methods All patients aged 1 to 18 yrs with native coA, treated percutaneously between 01/2000 and 12/2018, were included in this multcentric (9 centres) retrospective survey. Results One hundred and thirty three children (34 female) were included. Indication for treatment was hypertension in 109, exercise hypertension in 8 and other reasons in 16 patients. Mean age at intervention was 11.5 ± 3.9 years, mean weight 41 ± 18 kg. Procedure consisted of isolated balloon dilatation in 5 (mean age 2,63 yrs, mean weight 12 kg), balloon dilatation followed by stent implantation in 8 (mean age 11 yrs, mean weight 37 kg), and immediate stent implantation in 120 (mean age 12 yrs, mean weight 43 kg). Stents used (n = 124/128) were covered in 43, non-covered in 77, including 38 open cell stents. Invasive gradient across coA dropped from 28 mmHg (range 5 to 64) at baseline (n = 124) to 3 mmHg (range -5 to 20) after intervention (n = 105). Three associated lesions were treated: 1 PDA closure, 1 aortic valvuloplasty and 1 left SVC occlusion. Serious vascular complications occurred in 8 patients (6%) (3 pseudo-aneurysms, 2 dissections, 2 stenosis and 1 occlusion) requiring surgery in 2. Reintervention was performed in 22%: 2 patients had surgery, 28 were treated percutaneously (14 balloon dilatations, 14 stent implantations). Mean follow-up reached 4.9 ± 4.7 years. At latest follow-up, 25% remained hypertensive with 15 patients (11%) on anti-hypertensive medication. MRI, CTscan or fluoroscopy imaging, are available in 52 patients during follow-up (39%). Rate of aneurysm formation, stent fracture and risks factors for reintervention or residual hypertension will be presented. Conclusions Percutaneous treatment of native coarctation was safe and efficient in our french population. Close follow-up is required for longer-term results.

Summary Background The main difference between extreme tetralogy of Fallot (TOF) and pulmonary atresia with ventricle septal defect (PA/VSD) is anterograde pulmonary blood flow (APBF). It is speculated that the association of modified Blalock–Taussig shunt (mBTS) with APBF favours shunt thrombosis, but promotes better pulmonary artery growth. Aim To compare pulmonary artery growth after mBTS between TOF and PA/VSD. Methods From 1995 to 2018, 77 mBTS procedures were performed in infants (aged Results After matching, the preoperative Nakata index was similar in the two groups (TOF 101 ± 34 vs. PA/VSD 106 ± 35 mm2/m2; P = 0.75). Age and weight were similar (TOF 24 ± 20 days, 3.3 ± 0.6 kg vs. PA/VSD 24 ± 33 days, 3.3 ± 0.9 kg; P = 0.84 and P = 0.77, respectively). There was no difference in rates of in-hospital mortality (TOF 0% vs. PA/VSD 10%; P = 0.13) or mBTS thrombosis (TOF 15% vs. PA/VSD 10%; P = 0.63). The left and right pulmonary artery diameters at time of biventricular repair were similar (TOF 7.5 ± 2.2 and 6.7 ± 2.1 vs. PA/VSD 8 ± 2.7 and 7.1 ± 2.5 mm; P = 0.43 and P = 0.78, respectively), as were delta Nakata (TOF 112 ± 102 vs. PA/VSD 107 ± 66 mm2/m2; P = 0.89), median age for biventricular repair (P = 0.83) and reintervention rates (TOF 10% vs. PA/VSD 15%; P = 0.67). Conclusions We found no difference in pulmonary artery growth between APBF with mBTS versus mBTS alone. Thus, we could not show an increase in mBTS thrombosis with APBF.

Summary Background Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Le VSD coil was recently introduced in France as an alternative to occluder devices. Aims To study the safety and feasibility of pmVSD closure with the Nit-Occlud Le VSD coil. Methods All consecutives cases of pmVSD closure with the Nit-Occlud Le VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. Results Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7–31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7–10) mm on the left ventricle side and 5 (4–6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7–95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9–26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4–20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P = 0.001), residual shunt (P = 0.007) and multi-exit VSD (P = 0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. Conclusion pmVSD closure with the Nit-Occlud Le VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Le VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Background Management of patients with complex congenital heart defects (CHD) requires 3D imagination skills for the spatial evaluation of cardiac structures. Current 3D imaging [echocardiography, MRI, computed tomography (CT)], projected on a 2D screen, limits the appreciation of the relief and the interaction of anatomical structures. Purpose The contribution of 3D stereoscopic relief imagery (3D S/R), built by triangle-based mesh generation, is more qualitative than quantitative, which is why its use, rarely reported in the literature, must be evaluated. Methods 3D S/R was used from 2009 to 2019, as much as the CT made it possible to define a Region of Interest. Data and segmentation processing have been used to obtain four different types of reconstructions: MultiPlanar (MPR), Volume Rendering Technique (VRT), Surface Rendering Technique (SRT), and 3D S/R. The anaglyph 3D S/R effect requires the wearing of glasses (red - cyan blue filters) distributed during the session ( Fig. 1 ). Five complex CHD will be presented in MPR, VRT, then in SRT and 3D S/R: – Ventriculo-Arterial Malposition, – multiple Ventricular Septal Defects, – Anomalous Aortic Origin of a Coronary Artery, – Coarctation and hypoplastic Aortic Arch, – abnormal Pulmonary Arterial Arborization with Major Aorto-Pulmonary Collateral Arteries. Results The qualitative evaluation [MPR + VRT versus SRT and 3D S/R] will be carried out by questions/answers [Wooclap™ interactive system - voting on smartphone, in streaming, using a code associated with the presentation], allowing a global rating of the five CHD, concerning: – the perception of the relief, – the understanding of the cardiopathy, – the probability of appropriation of this 3D S/R imagery. Conclusions This presentation will allow: – to assess the potential influence of 3D S/R on the management strategy of CHD, – to compare these imaging studies with mesh generation techniques leading to the more commonly reported 3D printing.

Ventricular pseudoaneurysm is a rare but well-known complication after valvular endocarditis. The lesion was localised exactly where pre-operative CT scan showed lack of enhancement in the postero-lateral wall of the left ventricle. This case demonstrates how much attention must be paid to myocardial CT images and emphazises the need of close follow-up in patients with infective endocartitis.

Echocardiography plays a new critical role during structural heart interventions (SHIs), although fluoroscopy remains an indispensable tool [(1,2)][1]. Exposure to radiation by staff is directly proportional to the dose the patient receives in interventional cardiology and is affected by higher mean

We report the case of a 78-year-old woman with severe aortic valve stenosis that was successfully treated with transcatheter aortic valve implantation, with initial good hemodynamic results and clinical improvement of the patient. After 3 weeks, her clinical condition worsened, with progressive heart failure. Transthoracic echocardiography revealed an iatrogenic large subaortic ventricular septal defect with important left-to right shunt (Qp/Qs 3:1). The patient underwent successful transcatheter closure of the ventricular septal defect with a 14-mm Amplatzer mVSD Occluder (AGA Medical, Plymouth, MN), resulting in dramatic clinical improvement.

Summary Background Percutaneous implantation of pulmonary valves has recently been introduced into clinical practice. Aim To analyse data of patients treated in France between April 2008 and December 2010. Methods Prospective, observational, multi-centric survey by means of a database registry of the Filiale de cardiologie pediatrique et congenitale. Results Sixty-four patients were included, with a median (range) age of 21.4 (10.5–77.3) years. The majority (60.9%) of the patients were New York Heart Association (NYHA) class II. The most common congenital heart disease was tetralogy of Fallot with or without pulmonary atresia (50%). Indication for valve implantation was stenosis in 21.9%, regurgitation in 10.9% and association of stenosis and regurgitation in 67.2%. Implantation was successful in all patients. Pre-stenting was performed in 96.9% of cases. Median (range) procedure time was 92.5 (25–250) minutes. No significant regurgitation was recorded after the procedure, and the trans-pulmonary gradient was significantly reduced. Early minor complications occurred in five cases (7.8%). Three patients died during a median follow-up of 4.6 (0.2–5.2) years, two from infectious endocarditis and one from end-stage cardiac failure. Surgical reintervention was required in three patients. Follow-up with magnetic resonance imaging demonstrated significant improvements in right ventricular volumes and pulmonary regurgitation in mixed and regurgitant lesions. Conclusions Transcatheter pulmonary valve implantation is highly feasible and mid-term follow-up demonstrates sustained improvement of right ventricular function. Late endocarditis is of concern, therefore longer follow-up in more patients is urgently needed to better assess long-term outcome. Clinical trial registration NCT01250327 .

The Ensembl Trace Archive (http://trace.ensembl.org/) and the EMBL Nucleotide Sequence Database (http://www.ebi.ac.uk/embl/), known together as the European Nucleotide Archive, continue to see growth in data volume and diversity. Selected major developments of 2007 are presented briefly, along with data submission and retrieval information. In the face of increasing requirements for nucleotide trace, sequence and annotation data archiving, data capture priority decisions have been taken at the European Nucleotide Archive. Priorities are discussed in terms of how reliably information can be captured, the long-term benefits of its capture and the ease with which it can be captured.

The EMBL Nucleotide Sequence Database (http://www.ebi.ac.uk/embl) at the EMBL European Bioinformatics Institute, UK, offers a large and freely accessible collection of nucleotide sequences and accompanying annotation. The database is maintained in collaboration with DDBJ and GenBank. Data are exchanged between the collaborating databases on a daily basis to achieve optimal synchrony. Webin is the preferred tool for individual submissions of nucleotide sequences, including Third Party Annotation, alignments and bulk data. Automated procedures are provided for submissions from large-scale sequencing projects and data from the European Patent Office. In 2006, the volume of data has continued to grow exponentially. Access to the data is provided via SRS, ftp and variety of other methods. Extensive external and internal cross-references enable users to search for related information across other databases and within the database. All available resources can be accessed via the EBI home page at http://www.ebi.ac.uk/. Changes over the past year include changes to the file format, further development of the EMBLCDS dataset and developments to the XML format.

The EMBL Nucleotide Sequence Database (www.ebi.ac.uk/embl) at the EMBL European Bioinformatics Institute, UK, offers a comprehensive set of publicly available nucleotide sequence and annotation, freely accessible to all. Maintained in collaboration with partners DDBJ and GenBank, coverage includes whole genome sequencing project data, directly submitted sequence, sequence recorded in support of patent applications and much more. The database continues to offer submission tools, data retrieval facilities and user support. In 2005, the volume of data offered has continued to grow exponentially. In addition to the newly presented data, the database encompasses a range of new data types generated by novel technologies, offers enhanced presentation and searchability of the data and has greater integration with other data resources offered at the EBI and elsewhere. In stride with these developing data types, the database has continued to develop submission and retrieval tools to maximise the information content of submitted data and to offer the simplest possible submission routes for data producers. New developments, the submission process, data retrieval and access to support are presented in this paper, along with links to sources of further information.