With the aging of congenital heart disease (CHD) patients, the burden of arrhythmias is expanding. Atrial arrhythmias, especially intra-atrial reentrant tachycardia and atrial fibrillation, are the most prevalent forms of arrhythmia. Managing comorbidities, such as obesity, using pharmacotherapy, including antiarrhythmics and anticoagulants, and ablation therapy has become the cornerstone of arrhythmia management. Ventricular tachycardias are also not rare; however, except for tetralogy of Fallot patients, recommendations for the use of implantable cardioverter defibrillators for primary prevention in other CHD patients are still not well established. Patients with CHD might also present with atrioventricular blockages because of their anatomy or following a surgical procedure. The scope of this article is to review the current knowledge and discuss the future directions regarding arrhythmia management in CHD patients.

The adult congenital heart disease (ACHD) population is expanding and ageing. As survival rates of these patients continue to improve, acquired cardiac conditions, such as ischaemic cerebrovascular events and coronary artery disease (CAD), are likely to gain importance.1 However, limited information about the spectrum and the mechanisms of these conditions is available. With regard to stroke, patients with ACHD are at a low absolute, yet a relative high risk compared with the general population. A retrospective analysis of 29 638 patients from Quebec showed that 1 in 11 men and 1 in 15 women between 18 and 64 years of age had a history of a stroke.2 In Euro Heart Survey on ACHD, in which the inclusion of patients with more complex lesions was quite high, 4% of 4168 patients experienced a cerebrovascular accident or transient ischaemic attack (TIA).3 In the Swedish Patient Registry of congenital heart disease, 0.5% of patients experienced an ischaemic stroke.4 A very low incidence was also found by Bokma et al , who reported that 56 out of 6904 patients had a history of ischaemic stroke/TIA.5 However, it should be taken into account that although population-based studies are of high importance, there are limitations inherited with this type of studies, as some details in the medical records cannot be adequately accessed. Jensen et al showed that in a cohort of 98 patients with cyanotic ACHD, findings consistent with previous stroke diagnosed with a brain MRI were reported in 47% of patients, while only 21% had a known medical history of a stroke, indicating the significant under-reporting and the high prevalence of ‘silent’ cerebrovascular events …

Nowadays, most patients with congenital heart disease survive to adulthood due to advances in pediatric cardiac surgery but often present with various comorbidities and long-term complications, posing challenges in their management. The development and clinical use of risk scores for the prediction of morbidity and/or mortality in adults with congenital heart disease (ACHD) is fundamental in achieving optimal management for these patients, including appropriate follow-up frequency, treatment escalation, and timely referral for invasive procedures or heart transplantation. In comparison with other fields of cardiovascular medicine, there are relatively few studies that report prediction models developed in the ACHD population, given the small sample size, heterogeneity of the population, and relatively low event rate. Some studies report risk scores originally developed in pediatric congenital or non-congenital population, externally validated in ACHD with variable success. Available risk scores are designed to predict heart failure or arrhythmic events, all-cause mortality, post-intervention outcomes, infective endocarditis, or atherosclerosis-related cardiovascular disease in ACHD. A substantial number of these scores are derived from retrospective studies and are not internally or externally validated. Adequately validated risk scores can be invaluable in clinical practice and an important step towards personalized medicine. Multicenter collaboration, adequate study design, and the potential use of artificial intelligence are important elements in the effort to develop reliable risk scores for the ACHD population.

Hyperinflammation and uncontrolled cytokine release in infections and autoimmune diseases require therapy to reduce the innate immune response. Here, we present first in-human data showing reduction in pro-inflammatory cytokine release with ultrasound stimulation of the spleen in healthy subjects and in rheumatoid arthritis patients. Single cell RNA sequencing reveals a decrease in IL-1β and IL-8 transcript levels in circulating monocytes. There is also a down regulation of pathways involved in TNF and IL-6 production, and IFNγ- and NFκB-regulated genes. Additional pre-clinical studies reveal that ultrasound can boost B cell activation and antibody production. Splenic ultrasound offers a new non-invasive therapy for treating hyperinflammation without compromising the adaptive immune response.

Over the last decades, significant progress has been achieved in the pulmonary hypertension (PH) field. Pathophysiology of PH has been studied, leading to the classification of PH patients into five groups, while the hemodynamic definition has been recently revised. A diagnostic algorithm has been established and awareness has been raised in order to minimize diagnosis delay. The pulmonary arterial hypertension (PAH) treatment strategy includes the established three pathways of endothelin, nitric oxide-phosphodiesterase inhibitor, and prostacyclin pathway, but new therapeutic options are now being tested. The aim of this review is to summarize the existing practice and to highlight the novelties in the field of PH.

The implications of the adult congenital heart disease anatomic and physiological classification (AP-ACHD) for risk assessment have not been adequately studied. A retrospective cohort study was conducted using data from an ongoing national, multicentre registry of patients with ACHD and atrial arrhythmias (AA) receiving apixaban (PROTECT-AR study, NCT03854149). At enrollment, patients were stratified according to Anatomic class (AnatC, range I to III) and physiological stage (PhyS, range B to D). A follow-up was conducted between May 2019 and September 2021. The primary outcome was a composite of death from any cause, any major thromboembolic event, major or clinically relevant non-major bleeding, or hospitalization. Cox proportional-hazards regression modeling was used to evaluate the risks for the outcome among AP-ACHD classes. Over a median 20-month follow-up period, 47 of 157 (29.9%) ACHD patients with AA experienced the composite outcome. Adjusted hazard ratios (aHR) with 95% confidence intervals (CI) for the outcome in PhyS C and PhyS D were 1.79 (95% CI 0.69 to 4.67) and 8.15 (95% CI 1.52 to 43.59), respectively, as compared with PhyS B. The corresponding aHRs in AnatC II and AnatC III were 1.12 (95% CI 0.37 to 3.41) and 1.06 (95% CI 0.24 to 4.63), respectively, as compared with AnatC I. In conclusion, the PhyS component of the AP-ACHD classification was an independent predictor of net adverse clinical events among ACHD patients with AA.