Background/Objectives: Angiotensin receptor neprilysin inhibitor (ARNI) and sodium-glucose co-transporter 2 inhibitors (SGLT2i) are essential medications in heart failure (HF) therapy, and their potential antiarrhythmic effects have been reported. Recently, ARNI and SGLT2i use for HF in adult congenital heart disease (ACHD) has been studied. However, whether any beneficial effects may be achieved on the arrhythmic burden in the complex population of ACHD with a systemic right ventricle (sRV) is still to be determined. Methods: We retrospectively collected all significant arrhythmic events from a cohort of patients with a failing sRV attending our tertiary care center on optimal guideline-directed medical therapy (GDMT) with ARNI and/or SGLT2i. Results: A total of 46 patients (mean age 38.2 ± 10.7 years, 58% male) on sacubitril/valsartan were included. Twenty-three (50%) patients were also started on dapagliflozin. After a median follow-up of 36 [Q1–Q3: 34–38] months, arrhythmic events occurred globally in 13 (28%) patients. Survival analysis showed significant reduction of clinically relevant atrial and ventricular arrhythmia at follow-up (p = 0.027). Conclusions: Our findings suggest that GDMT including sacubitril/valsartan and dapagliflozin may also offer an antiarrhythmic effect in ACHD patients with a failing sRV, by reducing the incidence of arrhythmic events at follow-up.

Pulmonary disfunction is frequent in repaired congenital heart diseases. Both pulmonary regurgitation and pulmonary stenosis are possible complications over time. In the past, the surgical approach was the only feasible management but exposed the patient to a redo-surgery with its consequent risks. Nowadays, the development of novel devices and techniques has made possible a transcatheter pulmonary valve implantation. The Melody Transcatheter Pulmonary Valve (TPV) (Medtronic Inc., Minneapolis, MN, USA) and the Edwards Sapien XT and S3 Transcatheter Heart Valve (Edwards Lifesciences LLC, Irvine, CA, USA) are balloon-expandable valvular bioprostheses approved for pulmonary position. Venus P-Valve (Venus Medtech, Shanghai, China) and Harmony TPV (Medtronic Inc., Minneapolis, MN, USA) are self-expandable pulmonary valves. Alterra Adaptive Prestent (Edwards Lifesciences LLC, Irvine, CA, USA) is an hourglass self-expandable stent that reduces the size of large right ventricular outflow tracts, creating a suitable landing zone to implant an Edwards Sapien S3 THV 29 mm. Novel stents and percutaneous valves are being planned and experimented with to widen the field of transcatheter approach. The aim of this review is to describe both the current approaches, strategies, and techniques as well as the future perspective to deal with the patients with significant pulmonary stenosis and/or regurgitation.

Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases. We reviewed and explored the data behind indications for WCD use and discuss its usefulness in congenital heart disease (CHD) patients. We considered 8 consecutive patients (mean age 35.25 years, range 18–51 years, average duration of WCD use 4 months, range 3–6 months) with complex CHD, in which a WCD was used between June 2018 and January 2022. No sustained ventricular arrhythmias requiring shocks were recorded in the observation period. No inappropriate shocks were recorded. All the patients showed a good compliance and a very high mean wear time per day (21.2 ± 1 h a day). Four patients implanted a permanent device (3 CRT-D, 1 ICD), three underwent cardiac surgery at the end of the WCD period and one is still on the waiting list for the operation. Larger trial could confirm the possible conceivable benefit from an extended use of the WCD in certain populations with complex CHD as in our case series, especially in patients with life-treating ventricular arrhythmias waiting for surgery for residual cardiac defects or in the early phases following the surgical/hemodynamic interventions, patients with tachycardiomyopathy expected to improve after the arrhythmias are removed and patients awaiting implantation of an ICD at high risk due to active infection.

Limited data are available on the use of irrigated contact force (CF) catheters for radiofrequency (RF) ablation of permanent junctional reciprocating tachycardia (PJRT) in children. We considered five consecutive pediatric patients with diagnosis of PJRT who underwent RF ablation with irrigated CF catheter guided by electroanatomic mapping, obtaining a low number of RF pulses and minimal fluoroscopy exposure. High CF values are not necessary to obtain effective RF lesions and successful ablation.