The Fontan circulation is defined by unique physiological constraints that limit preload, elevate systemic venous pressure, and reduce the capacity to compensate for stress or injury. Traditional paradigms for managing biventricular heart failure are often misaligned with the pathophysiology of this preload-limited, non-pulsatile system. In this editorial, we propose a conceptual reframing of medical therapy in the Fontan circulation, centred on preserving physiological reserve and long-term durability rather than reversing established failure. We introduce a serial constraints model that emphasises the interconnected roles of the pulmonary vascular bed, ventricular filling, systemic output, venous and lymphatic function, and end-organ tolerance. As limitations shift over time, therapeutic focus must also evolve from pulmonary vasodilation in early stages to addressing ventricular dysfunction, venous congestion, and fibrosis later in the trajectory. We review emerging applications of phosphodiesterase type 5 inhibitors, sodium-glucose cotransporter-2 inhibitors, nonsteroidal mineralocorticoid receptor antagonists and exercise within this framework, while cautioning against routine use of conventional renin-angiotensin-aldosterone system inhibition or beta-blockers in the absence of clear comorbid indications. Antithrombotic strategies remain important due to a persistent thrombo-inflammatory milieu. We advocate for a shift from reactive to trajectory-preserving therapy, targeting flow under stress, organ protection, and reserve conservation. This physiology-aligned, evidence-informed approach provides a rationale for earlier intervention and testable hypotheses for future Fontan-specific trials.

The 2024 American Heart Association scientific statement broadened risk assessment in repaired tetralogy of Fallot beyond right ventricular volume, bringing together ejection fraction, exercise capacity, pressure load, and a validated risk score. One group of patients remains difficult: those whose right ventricular volumes are borderline and who meet no threshold for intervention. We describe this borderline range as a grey zone, with an indexed end-diastolic volume of approximately 140 to 160 mL/m2 and an indexed end-systolic volume of approximately 70 to 80 mL/m2. Within this range, no single value decides the timing of pulmonary valve replacement, and the direction of change across serial studies carries more information than any single borderline result. We outline a structured approach that places volume alongside markers of ventricular function, exercise capacity, the rhythm substrate, tissue characterisation, and loading conditions, in order to estimate the risk of continued surveillance and to set the interval to the next review. We also note that the imaging methods underlying this framework are unevenly available and unevenly taught across centres, drawing on a recent survey from the Association for European Paediatric and Congenital Cardiology, and that a framework intended for diverse centres is most useful when it separates assessments available to every unit from those available only to some. We present this framework as a way to structure reasoning at the bedside and to seed prospective multicentre study of borderline patients, rather than as a new set of cut-offs.

Survivorship among individuals with CHD has expanded, bringing more pregnancies into routine care. In this issue, a single-centre retrospective case–control study (162 CHD deliveries vs 321 controls) reports higher composite maternal cardiac events (8.6 vs 3.4%; risk ratio 2.5, 95% CI 1.2–5.4), greater use of operative or assisted delivery, and an approximately doubled length of stay. Modified WHO class correlates with length of stay but not with discrete cardiac events; maternal age independently predicts cardiac events; rates of pre-eclampsia are lower in CHD. These findings support first-trimester cardio-obstetric triage, standardised delivery bundles, and capacity planning that uses risk class to anticipate monitoring needs, with routine 6–12-week postpartum review. Limitations include selection, era differences, and modest sample size. Overall, anticipatory planning and disciplined intrapartum decision-making remain key to safe, resource-aware care for pregnant women with CHD.

Neonates with ductal-dependent congenital heart disease (CHD) will receive a continuous infusion of a prostaglandin E1 analogue such as alprostadil prior to surgical correction. Alprostadil can cause apnoea, which may be mitigated in practice with the use of caffeine citrate. Caffeine citrate can lead to tachyarrhythmias, which could be detrimental in this patient population. The purpose of this study was to determine if the practice of utilising caffeine citrate in neonates with ductal-dependent CHD receiving continuous alprostadil infusions is safe. In this single-centre, retrospective, cohort study of neonates admitted to the congenital heart service from November 2017 to September 2024, patients were included if they had ductal-dependent CHD and a gestational age greater than or equal to 35 weeks. Neonates that received continuous infusion of alprostadil alone were compared to those that received alprostadil in addition to caffeine citrate. The primary outcome was the frequency of sustained tachycardia during treatment. Seventy-five patients were included for review in each cohort. The median number of incidences of sustained tachycardia per patient was 7 (interquartile range 1–20) in the alprostadil plus caffeine citrate group and 4 (interquartile range 0–18) in the alprostadil alone group (p = 0.293). Other arrhythmias during the treatment course were common and similar between each cohort. Utilising caffeine citrate was not associated with a statistically significant increased incidence of tachycardia in neonates with ductal-dependent CHD on alprostadil infusions at the University of Florida.

Hypertension is an increasingly prevalent condition in both adults and children and is associated with significant long-term cardiovascular and renal morbidity. Current paediatric guidelines recommend a stepwise pharmacological approach, initiating treatment with a single antihypertensive agent at a low dose, followed by gradual titration up to the maximum tolerated dose and the addition of a second agent only if blood pressure control is not achieved. In contrast, adult hypertension guidelines increasingly support early combination therapy, particularly in patients with stage 2 hypertension, to achieve faster and more consistent blood pressure control and improve medication adherence. Given that blood pressure regulation involves multiple physiological systems, combining agents with complementary mechanisms of action may enhance antihypertensive efficacy while reducing dose-dependent adverse effects. Although the use of fixed-dose combination therapies is well established in adults, their application in paediatric populations remains limited due to regulatory-, clinical-, and formulation-related challenges. We propose that, in selected paediatric patients with an anticipated inadequate response to monotherapy, early combination therapy may be considered as an alternative to strict stepwise escalation. Expanding paediatric clinical research and developing age-appropriate formulations are essential to optimise hypertension management and improve long-term outcomes in this population.

Textbooks continue to serve as essential central repositories of knowledge for postgraduate education in paediatric cardiology, despite the widespread availability of digital learning tools. Recent studies confirm that trainees still value textbooks for their structure, depth, and accessibility, while also requesting improved pedagogy and organisation. In preparing the 5th edition of Anderson’s Pediatric Cardiology, our editorial team implemented several deliberate educational innovations. These included (1) re-focusing towards our target audience, the congenital cardiac trainee and practicing congenital cardiologist; (2) adoption of a consistent lesion-based framework, termed the “red line,” linking anatomy, physiology, and clinical features; (3) expansion of concise visual elements such as tables, figures, algorithms, and flowcharts; and (4) incorporation of clear learning objectives throughout. These modifications were informed by feedback from trainees, educators, and practicing providers, balancing the desires and needs of the modern learner, and firmly grounded in principles of cognitive and educational science. This paper outlines the rationale, design, and pedagogical implications of these innovations within the broader context of modern medical education.

The increasing reliance on bibliometric indicators to define academic success has reshaped surgical training and evaluation worldwide. While research productivity plays a critical role in advancing medical knowledge, its growing dominance as a surrogate for professional excellence poses unique challenges in low-and middle-income countries. In these settings, surgeons shoulder a disproportionate burden of surgical disease, often managing high case volumes, advanced pathology, and limited resources. Despite this, academic recognition remains closely tied to publication metrics that inadequately capture operative skill, clinical judgement, and patient-centred outcomes. This editorial examines the misalignment between academic metrics and surgical competence in low-and middle-income countries, explores the influence of emerging technologies such as artificial intelligence on scholarly output, and argues for a more context-sensitive definition of surgical excellence. Reframing evaluation frameworks to balance scholarly contribution with clinical mastery is essential to preserving patient safety, training integrity, and the ethical foundations of surgical practice.

Paediatric dysautonomia has become increasingly recognised in children and adolescents, particularly in the post-COVID era. Affected patients commonly present with dizziness, palpitations, exercise intolerance, fatigue, and syncope, although reported prevalence varies widely because of evolving definitions and heterogeneous referral patterns. Contemporary evidence suggests that post-COVID dysautonomia arises from complex interactions among central autonomic network dysfunction, neurovascular dysregulation, impaired venous return, endothelial injury, hypovolemia, and altered cerebral perfusion, with tachycardia often representing a compensatory physiological response rather than a primary cardiac abnormality. Clinical phenotypes include postural orthostatic tachycardia syndrome, neurocardiogenic syncope, orthostatic hypotension, inappropriate sinus tachycardia, and undifferentiated orthostatic intolerance, frequently accompanied by fatigue, cognitive dysfunction, gastrointestinal symptoms, sleep disturbances, and post-exertional symptom exacerbation. Paediatric dysautonomia is best conceptualised as a distributed brain–heart–vascular network disorder that requires mechanistic understanding, standardised orthostatic assessment, and careful exclusion of structural heart disease and arrhythmia. The rapid expansion of specialised dysautonomia programmes and direct-to-consumer diagnostic pathways has also contributed to broader, and occasionally premature, application of autonomic diagnoses. Management should follow a stepwise, mechanism-guided approach emphasising patient education, trigger avoidance, hydration and salt optimisation, lower-body compression, individualised exercise rehabilitation, pacing strategies when post-exertional symptom exacerbation is present, school accommodations, and phenotype-directed pharmacotherapy for persistent functional impairment. Although post-COVID dysautonomia shares features with established paediatric autonomic disorders, important gaps remain in disease definitions, mechanistic understanding, and evidence-based treatment, underscoring the need for multidisciplinary care, standardised diagnostic frameworks, and prospective paediatric research.

Congenital heart disease (CHD) often requires device implantation during childhood, when somatic growth and changing haemodynamics place particular demands on permanent implants. Metallic stents and nitinol occluders are effective, but their lifelong persistence can complicate repeat interventions, limit imaging surveillance, and interfere with growth-related remodelling. This review examines the rationale, current evidence, biomechanical constraints, and translational challenges of biodegradable stents and occluders in CHD. These devices aim to provide temporary mechanical support followed by controlled resorption, thereby reducing the burden of permanent foreign material and preserving future interventional options. Translation to congenital practice is, however, not straightforward, as early structural integrity must be balanced against predictable degradation, adequate endothelialisation, and compatibility with somatic growth. Experience from adult coronary bioresorbable scaffolds cannot be directly extrapolated to paediatric anatomies or timelines. Biodegradable septal occluders have accumulated more clinical experience and appear closer to routine use, whereas biodegradable vascular stents remain largely investigational and lack sufficient long-term outcome data to support broader adoption. Further long-term studies in paediatric and congenital populations are needed before biodegradable implants can be recommended as standard alternatives to permanent devices in CHD.

Familial hypercholesterolaemia remains substantially underdiagnosed worldwide despite effective preventive therapies. Evidence increasingly supports paediatric-centred detection combined with cascade and reverse cascade strategies to improve population-level identification and reduce premature atherosclerotic cardiovascular disease. We summarise contemporary familial hypercholesterolaemia screening models with emphasis on paediatric approaches, implementation barriers, and health system requirements, and we present the EPIRUS Familial Hypercholesterolaemia Reverse Cascade Project as a pragmatic regional model in Greece. A literature search (December 2025 to January 2026) was performed in PubMed, Scopus, and Google Scholar using terms related to “familial hypercholesterolemia,” “familial hypercholesterolaemia,” “screening,” “cascade,” “reverse cascade,” and “population programme.” Eligible publications included human studies and evidence syntheses evaluating universal, opportunistic, cascade, or paediatric screening strategies, and implementation or economic analyses. Evidence consistently indicates that opportunistic detection and conventional cascade screening alone cannot close the diagnostic gap because of low index-case ascertainment and fragmented pathways. Universal or structured paediatric programmes demonstrate feasibility, high diagnostic yield after infancy, and capacity to trigger family-based case-finding. Recent data show low-density lipoprotein cholesterol discrimination is limited in newborns but becomes reliable from age ≥1 year, supporting childhood screening. We describe EPIRUS Familial Hypercholesterolaemia, a targeted paediatric programme (primarily ages 4–16 years) using lipid thresholds and family history triggers, systematic phenotyping, and next-generation sequencing to confirm diagnosis and enable cascade and reverse cascade screening. EPIRUS Familial Hypercholesterolaemia illustrates how paediatric entry points can operationalise family-based detection within routine clinical care in a decentralised setting and can generate real-world implementation evidence to inform scalable national strategies.