Salusins have emerged as a new biomarker that reflects an increased inflammatory state, which is associated with cardiovascular risk. We investigated the predictive value and usefulness of salusins as an inflammatory biomarker in obese children. This prospective cohort study included 75 obese children and 101 healthy children (as a control group). Salusin-α, Salusin-β, and various cardiovascular parameters were assessed in both groups. Correlation analyses of Salusin-α and Salusin-β with body mass index standard deviation scores and inflammatory and cardiovascular markers were performed. The mean patient age was 11.9±2.4 years for the obese group and 12.5±2.1 years for the control group. The obese children had a significantly higher heart rate, systolic blood pressure, diastolic blood pressure, epicardial adipose tissue thickness, and left ventricular mass than did the children in the control group. There was no significant correlation between Salusin-α and Salusin-β and body mass index; however, there was a negative correlation between Salusin- α and diastolic blood pressure (r = 0.277, p = 0.004). Overall, there was no significant difference in the Salusin-α and Salusin-β levels between obese and healthy children. However, a negative correlation was found between Salusin-α and diastolic blood pressure. Although this result suggests that Salusin-α might be an early marker of cardiovascular involvement in obese children, further studies are needed to demonstrate the predictive value of salusins.

Stenosis, or narrowing, of the branches of the pulmonary artery is a type of CHD that, if left untreated, may lead to significant complications. Ideally, interventions to treat stenosis occur before significant complications or long-term sequelae take place, often within the first 2 years of life. Treatment depends on specifics of the condition, the presence of other malformations, and age of the child. Research and recent innovation to address these shortcomings have provided physicians with safer and more effective methods of treatment. This has further continued to push the ceiling of pulmonary arterial stenosis treatment available for patients. Despite continuous advancement in angioplasty – such as conventional and cutting balloon – and stenting, each treatment method is not without its unique limitations. New technological developments such as bioresorbable stents can accommodate patient growth and pulmonary artery stenosis treatment. As more than a decade has passed since the review by Bergersen and Lock, this article aims to provide a contemporary summary and investigation into the effectiveness of various therapeutic tools currently available, such as bare metal stents and potential innovations including bioresorbable stents.

Advances in biomedical engineering have led to three-dimensional (3D)-printed models being used for a broad range of different applications. Teaching medical personnel, communicating with patients and relatives, planning complex heart surgery, or designing new techniques for repair of CHD via cardiac catheterisation are now options available using patient-specific 3D-printed models. The management of CHD can be challenging owing to the wide spectrum of morphological conditions and the differences between patients. Direct visualisation and manipulation of the patients’ individual anatomy has opened new horizons in personalised treatment, providing the possibility of performing the whole procedure in vitro beforehand, thus anticipating complications and possible outcomes. In this review, we discuss the workflow to implement 3D printing in clinical practice, the imaging modalities used for anatomical segmentation, the applications of this emerging technique in patients with structural heart disease, and its limitations and future directions.

The range and number of educational and networking events that are available for fellows, trainees, and junior faculty to attend grows every year. Each meeting useful in its own way; each adding value to the development and the growth of an interventionist. Within paediatric, congenital, and structural heart disease, three of the standout meetings are: Pediatric and Interventional Cardiac Symposium (PICS-AICS), Congenital and Structural Interventions (CSI), and International Workshop on Interventional Pediatric and Adult Congenital Cardiology (IPC). All of these were started by leaders in our field; people known to be passionate educators and innovators. International congresses focusing more broadly on congenital cardiac disease in children and adults are rare. These forums allow more interdisciplinary discussions between the interventionist, surgeon, and non-invasive specialists. Purely interventional meetings are essential to allow colleagues to debate and explore the nuances and intricacies of technique and approach, developing concepts to be challenged in wider forums. During the recent 21st PICS-AICS meeting Prof. Ziyad M. Hijazi, Shakeel A. Qureshi, Mario Carminati, and Dr Damien Kenny shared their time to engage in frank, recorded conversations which provide a unique insight in to the process and concepts behind three of our most important educational congresses.

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.

Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas. This review describes lessons learned through the development of iCARD, initial efforts and scientific output, challenges, and future directions. This information can aid in the use and optimisation of data integration methodologies across other research networks and organisations.

The history of congenital interventional cardiology has seen numerous groundbreaking innovations typically related to the introduction of a new device or a novel treatment technique. Similarly, imaging of cardiac defects has changed dramatically over the past decades, although some of the advancements have seemed to omit the catheterisation laboratories. Rotational angiography, one of the imaging techniques for guidance of cardiac catheterisation currently referred to as “advanced”, in fact was described already in 1960s.1 More recently its improved version, including three-dimensional reconstruction (3DRA), became a valuable intra-procedural imaging tool in interventional cardiology and neuroradiology.2 Dr Evan Zahn was one of the pioneers of 3DRA in the field of congenital cardiology, setting an example for many to follow. With his innovative publication and subsequent lecture at 2011 Pediatric and Adult Interventional Cardiac Symposium (PICS-AICS) on “The Emerging Use of 3-Dimensional Rotational Angiography in Congenital Heart Disease” he motivated many to explore benefits of this modality to strive for improved procedural outcomes and reduced patients’ burden of cardiac catheterisation3. I was one of those to take Dr Zahn’s thoughts and implement them into routine workflow.4–6 However, almost a decade after Dr Zahn shared his important work, despite tremendous efforts by teams from Utrecht, (Netherlands) and Columbus (Ohio, United States of America) to popularise 3D imaging in catheterisation laboratory during dedicated meetings, two-dimensional (2D) angiography does not seem to be threatened in many, otherwise-progressive, laboratories. During the recent 30th Japanese Pediatric Interventional Cardiology (JPIC) meeting I had the opportunity to ask Dr Zahn why giving up knowledge is almost never a good idea, what is technology’s natural order of things, and why the technology has to be more than just exciting, pretty, and new.

A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.

Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Splanchnic circulation constitutes a major portion of the vasculature capacitance and plays an important role in maintaining blood perfusion. Because patients with asplenia syndrome lack this vascular bed as a blood reservoir, they may have a unique blood volume and distribution, which may be related to their vulnerability to the haemodynamic changes often observed in clinical practice. During cardiac catheterisation, the mean circulatory filling pressure was calculated with the Valsalva manoeuvre in 19 patients with Fontan circulation, including 5 patients with asplenia syndrome. We also measured the cardiac output index and circulatory blood volume by using a dye dilution technique. The blood volume and the mean circulatory filling pressure and the venous capacitance in patients with asplenia syndrome were similar to those in the remaining patients with Fontan circulation (85 ± 14 versus 77 ± 18 ml/kg, p = 0.43, 31 ± 8 versus 27 ± 5 mmHg, p = 0.19, 2.8 ± 0.6 versus 2.9 ± 0.9 ml/kg/mmHg, p = 0.86). Unexpectedly, our data indicated that patients with asplenia syndrome, who lack splanchnic capacitance circulation, have blood volume and venous capacitance comparable to those in patients with splanchnic circulation. These data suggest that (1) there is a blood reservoir other than the spleen even in patients with asplenia; (2) considering the large blood pool of the spleen, the presence of a symmetrical liver may represent the possible organ functioning as a blood reservoir in asplenia syndrome; and (3) if this is indeed the case, there may be a higher risk of hepatic congestion in patients with Fontan circulation with asplenia syndrome than in those without.