The combination of a right aortic arch with a vascular ring and coarctation of the aorta is a rare association, presenting a unique management challenge for the primary team. A thorough literature review revealed only seven published case reports, with three cases reported in the neonatal period with similar anatomy. This distinctive anatomy inspires inquiry into the development of coarctation in the context of a right aortic arch and vascular ring, as well as the best approach to surgical management. We encountered a similar case in a neonate with a combination of these malformations and a unique aortic arch branching pattern. Cardiac CT was instrumental in the diagnosis and surgical planning. This article reviews the variations in anatomy, clinical presentation, imaging findings, and management challenges encountered in the reported cases. This comprehensive review aims to assist the primary team in making informed decisions when treating these complex patients.

Gender-affirming care is a multi-faceted healthcare provided to transgender and gender diverse individuals to support their physical and mental health. As more adolescents and young adults may be interested in various gender care options, cardiologists should have familiarity with the medical and surgical therapies offered and the risk and considerations these may have, particularly for youth with the history of congenital or acquired cardiovascular disease. The existing literature on exogenous hormone therapy, for the general population and transgender individuals specifically, is by large limited to the adult population, with little relevant information for younger individuals. Current evidence indicates several potential cardiovascular implications to consider with gender-affirming hormone therapy without documented morbidity or clear contraindication for its use. These include the risk of thromboembolism, atherosclerotic and coronary artery disease, hypertension, and dyslipidemia among others. This review summarises existing evidence to assist in navigating the risks and therapeutic options so that offering gender-affirming care can be considered and delivered safely for adolescents and young adults with cardiovascular conditions and makes recommendations for future research.

This manuscript documents the Presidential Address of Jan Janoušek as the incoming President of the Association for European Paediatric and Congenital Cardiology (AEPC) 2025–2028.

Psychogenic pseudosyncope is one of the primary causes of transient loss of consciousness in children and adolescents, essentially classified as a conversion disorder that significantly impacts patients’ quality of life. Clinically, psychogenic pseudosyncope shares certain similarities with vasovagal syncope in terms of pre-syncope symptoms and triggers, making it sometimes difficult to differentiate and easily misdiagnosed. Therefore, placing emphasis upon the characteristics of psychogenic pseudosyncope is crucial for early identification and treatment, which holds significant importance for the mental and psychological health of children and adolescents. In the present review, we aimed to address psychogenic pseudosyncope with clinical features, diagnosis, and treatment.

This study aimed to assess Well-Being Index scores in paediatric cardiac ICU (PCICU) registered nurses and advanced practice providers. Secondary objectives included identifying factors correlating with at-risk Well-Being Index scores and exploring predictors of these scores, with attention to the impact of the coronavirus disease 2019 pandemic. A multicentre electronic survey was conducted between October 2021 and January 2022. Registered nurses and advanced practice providers working in PCICUs at US centres participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society were included.

The survey included the nine-item Well-Being Index and questions about demographics and factors influencing well-being, such as coronavirus disease 2019. The Well-Being Index is a validated tool to predict workforce distress and well-being. Out of 218 participants (180 registered nurses, 38 advanced practice providers), 137 registered nurses (76%) and 15 advanced practice providers (39%) had at-risk Well-Being Index scores. A total of 61% of nurses and 34% of advanced practice providers reported an intent to leave. Intent to leave was significantly linked to lower well-being for registered nurses (p = 0.002). Leadership support reduced registered nurses’ distress risk by 68% compared to no support (p = 0.04). Increased stress since coronavirus disease 2019 raised registered nurses’ poor well-being risk by four times (p = 0.001). PCICU registered nurses and advanced practice providers are at risk for poor well-being, exacerbated by the pandemic. Those with poor well-being may be more likely to leave. Leadership support is vital for nurses’ well-being. Further research is needed to establish baseline well-being.

Hypertensive heart disease and hypertrophic cardiomyopathy both lead to left ventricular hypertrophy despite differing in aetiology. Elucidating the correct aetiology of the presenting hypertrophy can be a challenge for clinicians, especially in patients with overlapping risk factors. Furthermore, drugs typically used to combat hypertensive heart disease may be contraindicated for the treatment of hypertrophic cardiomyopathy, making the correct diagnosis imperative. In this review, we discuss characteristics of both hypertensive heart disease and hypertrophic cardiomyopathy that may enable clinicians to discriminate the two as causes of left ventricular hypertrophy. We summarise the current literature, which is primarily focused on adult populations, containing discriminative techniques available via diagnostic modalities such as electrocardiography, echocardiography, and cardiac MRI, noting strategies yet to be applied in paediatric populations. Finally, we review pharmacotherapy strategies for each disease with regard to pathophysiology.

In the United States, about 1 in 100 children are born with a CHD, with complex cases requiring intensive, lifelong care. Despite medical severity, little data exist on economic burden, driving low impact scores in federal research funding applications, a lack of specific funding appropriations, and minimal research investment. Here, the financial and economic impact was quantified by identifying direct, indirect, and mortality costs of six complex CHDs and compared to two common cardiovascular diseases: coronary heart disease and congestive heart failure. Despite lower prevalence, complex CHDs exert disproportionate, often invisible financial burdens on families and the healthcare system. Annually, per patient, direct and indirect costs of complex CHDs are four times higher than coronary heart disease and eight times higher than congestive heart failure. The average lifetime direct and indirect cost for complex CHDs is $2.1 million per patient—nearly 44 times that of congestive heart failure. 31% of lifetime costs for complex CHDs are incurred in the first five years of life with families paying an average of $190,000 out-of-pocket. Many caregivers are forced to reduce employment hours, change jobs, or completely withdraw from the labour force. The annual national cost of complex CHDs is $74 billion, and the total lifetime economic impact exceeds $3.35 trillion. Systemic under-recognition has led to chronic underinvestment in research and stagnant patient outcomes. The biomedical community can catalyse an interdisciplinary response from research institutions, policymakers, and clinical systems to alleviate the economic and human toll of complex CHDs.

Kawasaki disease is a childhood vasculitic disorder that has a special predilection for coronary arteries. Kawasaki disease has been reported from all regions of the world, with an increasing incidence in several countries. Kawasaki disease is now the most common cause of acquired heart disease in children all over the world. However, it is concerning that the estimated vast majority of Kawasaki disease cases in low- and middle-income countries are not getting diagnosed and treated. The World Health Organization acknowledges cardiovascular disease in their priority of actions. The World Health Organization is invited to acknowledge the reality of Kawasaki disease in its list of cardiovascular diseases and take steps to facilitate the diagnosis and treatment of Kawasaki disease, especially in low- and middle-income countries. It is a disease of public health importance and needs urgent prioritisation by the World Health Organization.

Vascular rings represent a heterogeneous set of aberrant great vessel anatomic configurations which can cause respiratory symptoms or dysphagia due to tracheal or oesophageal compression. These symptoms can be subtle and may present at varied ages. More recently, many have been identified in patients without symptoms, including fetal echocardiogram, resulting in a conundrum for practitioners when attempting to determine who will benefit from surgical correction. Here, we provide a review of vascular rings and a guide to the practitioner on when to consider additional imaging or referral. Additionally, we discuss the changing landscape regarding asymptomatic patients and fetal echocardiogram.

The Occlutech Atrial Flow Regulator is a self-expandable double-disc nitinol device with a central fenestration designed to create a calibrated communication across the interatrial septum. It has been used in adult patients with heart failure and pulmonary hypertension. Its use in the paediatric population or adults with CHD has been published in several case reports and case series. This study aims to review the potential, efficacy, and safety of this device in the paediatric and adult populations living with CHD, as well as in paediatric patients with pulmonary hypertension or advanced heart failure.

With the steady improvement in the efficacy of Ozaki technique in children over the past decade, it is more active and widely used in children with aortic valve disease. Ozaki technique has obvious advantages over traditional prosthetic valve replacement. It preserves the natural motion of the aortic annulus, preserves the coordination of the left ventricle, the sinus of Valsalva and the aorta, naturally dilates the aortic root during systole, restores the physiological laminar flow pattern, and retains the continuous growth potential of the aortic annulus. It has good early and mid-term valve durability, no need for anticoagulation, short learning curve time, easy to promote, suitable for a wide range of people, and significant economic benefits, especially in developing countries with serious shortage of medical resources but a large number of CHD patients. Theoretically, Ozaki techniques can be considered in patients of all ages (adults and children) and in those with acquired and congenital aortic valve disease, including those with previous mechanical, bioprosthetic, or Ross procedures. We currently support the concept of using Ozaki technique as a surgical repair method for children with severe aortic valve disease, but the specific strategy should be made individually according to the patient’s condition. This article reviews the past and present, surgical indications, surgical procedures, advantages, prognosis, and prospects of Ozaki technique in treating aortic valve disease in children.

While the pathophysiology affecting patients after Fontan palliation versus those with orthostatic intolerance is quite different, a common therapeutic approach exists. Exercise training, specifically augmenting the lower extremity skeletal muscle pump, improves the suboptimal haemodynamics of “preload failure” and thus clinical outcomes for each patient group. In this review, we will describe the problematic physiology affecting these patients, examine the anatomy and haemodynamics of the skeletal muscle pump, and finally review how exercise benefits both groups of patients through augmentation of musculovenous force.

Trisomy 21 is the most common chromosomal anomaly worldwide, and nearly half of the affected individuals are born with CHD, making cardiac complications a leading cause of morbidity and mortality in this population. Over the past century, the management of CHD in patients with Trisomy 21 has evolved dramatically, shaped by shifting societal attitudes, advances in diagnostic and surgical techniques, and landmark legal and ethical milestones. Historically, children with Trisomy 21 faced significant barriers to cardiac care, including delayed referrals and denial of surgical intervention, often rooted in discrimination rather than medical evidence. However, improvements in perioperative management and early surgical repair have led to survival outcomes for many forms of CHD that now approach those of the general population. Despite these advances, challenges persist, particularly in access to heart transplantation, where disparities in referral and eligibility remain. This review provides a historical overview of the evolution of CHD management in individuals with Trisomy 21, highlighting key medical, ethical, and societal developments that have shaped current standards of care.