We report a 20-year-old female patient (76 Kg/164 cm) with an extra-cardiac Fontan circulation who was referred to our institution for exertional dyspnoea and desaturation. The patient was diagnosed with a large calcified thrombus at the level of the Fontan fenestration, protruding inside the lumen of the conduit and reducing the diameter by half with a 3 mmHg pressure gradient. Transcatheter stent expansion of the obstructed extra-cardiac conduit was done with a 48 mm long XXL PTFE-covered Optimus-CVS® under temporary cerebral embolic protection with a TriGUARD-3™ deflection filter device (Keystone Heart). There was no procedural complication and the 3 months clinical outcomes are good.

In neonatal Ebstein’s anomaly of the tricuspid valve, prolonged ductal patency in patients without anatomic pulmonary valve atresia can be deleterious. Circular shunts may develop in patients with different degrees of pulmonary and tricuspid insufficiency. Closure of the arterial duct may result in haemodynamic improvement in particular scenarios. The transcatheter approach is a valuable closure alternative despite some technical difficulties in large-sized arterial ducts and low birth weight neonates. Herein, we report on two consecutive term newborns with Ebstein’s anomaly and large arterial ducts in whom mechanical stimulus of the arterial duct during failed attempts of transcatheter closure led after two days to definitive closure followed by good clinical outcomes.

KONAR-MultifunctionalTM VSD Occluder (Lifetech, Shenzhen, China) is one of the most recent additions to the armamentarium of device closure interventions offering special features to tackle complex cardiac anatomies. Herein, we report the first use of the KONAR-MFO in an 8.5-year-old female patient (27 kg/129 cm) with stage III palliated univentricular heart to close an acquired post-operative tunnel-like communication (5 mm long × 2.6 mm large) between the right anterior non-coronary aortic sinus and the rudimentary right ventricular cavity. The shunt was diagnosed two and a half years after bulboventricular foramen surgical enlargement. The 5× 3 mm KONAR-MFO was retrogradely implanted under ultrasound and biplane fluoroscopic guidance. Immediate and 12-month follow-up confirmed successful outcomes with complete shunt closure and preserved aortic valve competence.

A newborn girl was referred for a suspected abnormal pulmonary venous return in coronary sinus. Echocardiogram revealed a large outpouching of the left ventricle creating an accessory cardiac chamber that was connected to the left ventricle behind the mural leaflet of the mitral valve, and with the right atrium via the coronary sinus. Ten years later, the orifice between the submitral aneurysm and the left ventricle was closed percutaneously.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

The International Pediatric and Congenital Cardiac Code (IPCCC) states that visceral heterotaxy is defined as “a congenital malformation in which the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, in congenital cardiology, heterotaxy syndrome does not include patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as “total mirror imagery” or “situs inversus totalis”.” [www.ipccc.net]

In patients with heterotaxy, it is important to describe both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. Particular attention is required for the venoatrial connections, since these are so often abnormal. The relationship and arrangement of the remaining thoraco-abdominal organs, including the lungs, the spleen, the liver, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. Therefore, in patients with heterotaxy, it is important to describe each thoracic and abdominal organ independently.

Extensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.

Coronary artery compression by epicardial leads is a rare complication in children and can be difficult to identify with potentially lethal outcomes. Herein, we report the case of a previously asymptomatic paced-dependant 5-year-old girl who presented to our institution with resuscitated cardiac arrest. We describe the atypical sequence of clinical findings misleading initial diagnosis. Hardware failure and the commonly occurring lead fracture were incriminated in the mechanism of cardiac arrest, precipitating implantation of a new pacing system while concealing dynamic compression of the left anterior descending coronary artery.

The hybrid perventricular approach for the closure of trabecular ventricular septal defects is an attractive treatment modality for small children. Worldwide experience has shown that procedure success is influenced by the defect anatomical accessibility, operators’ expertise, and device technical features. In May 2018, a new promising device, the KONAR-Multi-functional™ ventricular septal defect occluder (Lifetech, Shenzhen, China), obtained CE-marking for septal defect transcatheter closure after the first-in-man implantation in 2013. Herein, this is the first report of successful perventricular closure of ventricular septal defect using this new device in a child with significant co-morbidities.

Percutaneous closure is the gold standard treatment for atrial septal defects, but the procedure can be complex in case of femoral thrombosis. Although unusual for congenital interventionists, transhepatic atrial septal defect closure is an attractive alternative to the internal jugular vein, especially when approaching the interatrial septum. Herein, we report the case of an adult patient with significant co-morbidities who had successful transhepatic atrial septal defect closure after a failed transjugular attempt. We describe the use of an absorbable haemostatic gelatin sponge to efficiently and safely achieve haemostasis after the use of a large vascular sheath with combined anticoagulation and antiplatelet therapy.

We report the case of a fetus with anamnios sequence and VACTERL syndrome, having a circumflex right aortic arch. Two arterial ducts join anteriorly to form a common vessel that connects to the pulmonary trunk with confluent pulmonary branches. Embryologically, the dorsal right 6th aortic arch did not disappear and the aortic arch development stopped in a symmetrical state with an exceptional “Y-shaped” merged bilateral arterial duct.

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient’s age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

Intracardiac teratomas are rare primary tumours. We report the case of an infant prenatally diagnosed with an isolated multi-cystic mass developed in the right ventricle causing neonatal refractory ventricular arrhythmia. Despite rescue extracorporeal support and partial surgical resection, he died as almost all the previous reported perinatal intracardiac teratomas whatever the prenatal tolerance and the size of the tumour. The common poor outcome of fetal intracardiac teratomas should be known when counselling parents during pregnancy.

Advanced medical and surgical treatment of heart failure and management of patients following heart transplantation is an emerging area. Treatment options at various levels are becoming available in an increasing number of countries. This rapidly evolving field involves a complex multi-disciplinary approach with a number of complementary medical and surgical strategies, including pharmacotherapy, structural cardiac interventions, electrophysiological optimisation, mechanical circulatory support, and heart transplantation. Furthermore, the importance of psycho-social support and care of patients and their families cannot be overstated. The aforementioned challenges and dynamics of new developments require guidance for core and advanced medical training in heart failure and transplantation. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

The need for population-based studies of adults with CHD has motivated the growing use of secondary analyses of administrative health data in a variety of jurisdictions worldwide. We aimed at systematically reviewing all studies using administrative health data sources for adult CHD research from 2006 to 2016. Using PubMed and Embase (1 January, 2006 to 1 January, 2016), we identified 2217 abstracts, from which 59 studies were included in this review. These comprised 12 different data sources from six countries. Of these, 55% originated in the United States of America, 28% in Canada, and 17% in Europe and Asia. No study was published before 2007, after which the number of publications grew exponentially. In all, 41% of the studies were cross-sectional and 25% were retrospective cohort studies with a wide variation in the availability of patient-level compared with hospitalisation-level episodes of care; 58% of studies from eight different data sources linked administrative data at a patient level; and 37% of studies reported validation procedures. Assessing resource utilisation and temporal trends of relevant epidemiological and outcome end points were the most reported objectives. The median impact factor of publication journals was 4.04, with an interquartile range of 3.15, 7.44. Although not designed for research purposes, administrative health databases have become powerful data sources for studying adult CHD populations because of their large sample sizes, comprehensive records, and long observation periods, providing a useful tool to further develop quality of care improvement programmes. Data linkage with electronic records will become important in obtaining more granular life-long adult CHD data. The health services nature of the data optimises the impact on policy and public health.

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry.

Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg).

At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy.

There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.