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Aims Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA are prone to heart failure and arrhythmias. This study evaluated feasibility, patient adherence, and satisfaction of a smart technology-based care pathway for heart failure treatment optimization in these patients. Methods and results Patients with symptomatic sRV failure eligible for initiation of sacubitril/valsartan were provided with four smartphone compatible devices (blood pressure monitor, weight scale, step counter, and rhythm monitor) and were managed according to a smart technology-based care pathway. Biweekly sacubitril/valsartan titration visits were replaced by electronical visits, patients were advised to continue measurements at least weekly after titration. Data of 24 consecutive sRV patients (median age 47 years, 50% female) who participated in the smart technology-based care pathway were analysed. Median home-hospital distance was 65 km (maximum 227 km). Most patients (20, 83.3%) submitted weekly measurements; 100% submitted prior to electronical visits. Titration conventionally occurs during a hospital visit. By implementing eHealth smart technology, 68 such trips to hospital were replaced by virtual visits facilitated by remote monitoring. An eHealth questionnaire was completed by 22 patients (92%), and 96% expressed satisfaction. After titration, 30 instances of remote adjustment of heart failure medication in addition to scheduled outpatient clinic visits occurred, one (4%) heart failure admission followed, despite ambulant adjustments. Five patients (21%) sent in rhythm registrations (n = 17), of these 77% showed sinus rhythm, whereas supraventricular tachycardia was detected in the remaining four registrations. Conclusion These data suggest that implementation of a smart technology-based care pathway for optimization of medical treatment sRV failure is feasible with high measurement adherence and patient satisfaction.

ObjectivePharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.MethodsData on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment.ResultsSix-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950–358 ng/L, pConclusionsWe describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population.

Background Patients with transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to systemic right ventricular (sRV) failure. Atrioventricular (AV)-conduction disturbances and tricuspid regurgitation aggravate the course of sRV dysfunction. Timely tricuspid valve (TV) surgery stabilizes sRV function. However, TV surgery is an independent risk for AV-block and ventricular pacing in non-congenital cardiothoracic surgery patients. Chronic subpulmonary ventricular pacing-induced dyssynchrony further contributes to sRV failure, potentially reducing the beneficial effects of the tricuspid valve surgery. Purpose The aim of this study is to explore the incidence, timing and functional consequences of AV-conduction block requiring ventricular pacing after TV surgery in sRV patients. Methods Consecutive adolescent and adult patients with a sRV who underwent TV surgery in the period 1989–2020 and follow-up at our tertiary care center were included in this observational cohort study. Patients who were Results Data of 28 patients (54% female, 57% ccTGA) was analysed (Figure). The mean age at surgery was 38±13 years, 5 patients (18%) received chronic ventricular pacing preoperatively. Mean follow-up was 9.7±6.8 years, during which 7 patients (25%) died and 3 (11%) underwent ventricular assist device implantation (VAD). Two patients died awaiting VAD/HTx, one patient died awaiting CRT upgrade. Seven (25%) patients underwent a re-operation, of which 3 (11%) TV replacement, 3 (11%) VAD and 1 (4%) pulmonary valve replacement. Of the 23 patients at risk of developing AV-block, 11 (48%) developed an indication for chronic ventricular pacing, of which 6 within 24 months postoperatively (4 before hospital discharge). Of the 21 patients with a device, 7 (25%) had successful resynchronization therapy (2 before TV surgery). Patients with chronic ventricular pacing had a wider QRS-duration (mean 121 ms vs 194 ms in those without pacing, p Conclusions Patients with a failing sRV who undergo TV surgery are prone to AV-conduction abnormalities with 48% of this group developing an indication for chronic ventricular pacing during follow-up. Pacing-induced dyssynchrony can further contribute to sRV dysfunction. Implantation of an epicardial sRV lead at the time of TV surgery for future CRT may be considered to attenuate the detrimental effects of subpulmonary ventricular pacing in this heart failure prone patient group with complex anatomy, limiting transvenous possibilities. Funding Acknowledgement Type of funding sources: None.

Background Patients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing. The morphological right ventricle (RV) often develops systolic dysfunction as it is unable to withstand the chronic pressure overload it is exposed to when supporting the systemic circulation. Case summary A 56-year-old woman with dextrocardia and complex ccTGA with a history of dual-chamber implantable cardioverter-defibrillator (DDD-ICD, high degree atrio-ventricular-block and syncopal ventricular tachycardia), presented with progressive heart failure and symptomatic atrial arrhythmias. She underwent a successful ablation and concomitant invasive haemodynamic evaluation of potential alternative/biventricular pacing modalities. During biventricular pacing, the QRS narrowed and the systemic RV intraventricular pressure (Dp/Dt) increased with 30%. She underwent a successful transvenous upgrade to cardiac resynchronization therapy (CRT). The electrocardiogram post-implantation showed biventricular capture and patient showed subjective and objective clinical improvement. Discussion Systemic RV dysfunction in ccTGA can be aggravated by chronic pacing-induced dyssynchrony, contributing to progression of heart failure in this patient group. Transvenous CRT is feasible in ccTGA anatomy and may be pursued in order to improve or preserve the functional status of pacing-dependent ccTGA patients. Invasive haemodynamic contractility evaluation can help assess the potential benefit of CRT in patients with complex anatomy.

Background In patients with heart failure with low ejection fraction (EF) (≤35%) sacubitril/valsartan is proven to be beneficial. We aimed to assess the effects of sacubitril/valsartan in adult congenital heart disease patients with failing systemic right ventricle (sRV) in the setting of congenitally corrected transposition of the great arteries (TGA) or TGA after atrial switch. Purpose To evaluate the feasibility and effect of treatment with sacubitril/valsartan in patients with heart failure in the setting of a sRV on optimal medical heart failure treatment. Methods Data of patients with sRV failure (estimated RVEF ≤35%) treated with sacubitril/valsartan were analyzed. Patients were clinically evaluated at baseline and at six months after initiation of the highest tolerated dose. Results Fourteen patients completed six-month follow-up thus far, of which 6 female (42.8%) and 2 (14.3%) with congenitally corrected transposition of the great arteries (TGA). After 6 months, 9 patients (64.3%) were using the highest dose (97/103 mg twice daily) of sacubitril/valsartan. NT-proBNP was significantly lower after 3 months and after 6 months compared with baseline (64% of baseline, p=0.006 and 53% of baseline, p=0.001, respectively). The kidney function did not change significantly (creatinine at baseline 83.6±12.0 and at 6 months 88.5±13.87, p=0.141) and serum potassium remained within normal limits (4.28±0.36 and 4.49±0.30, p=0.007). There was no significant decrease in systolic blood pressure (p=0.960). The echocardiographic sRV fractional area change (FAC) improved (p=0.002) and Global Longitudinal Strain (GLS) showed a trend towards improvement (p=0.058). During the 6 months of treatment, the exercise capacity remained stable, there was no increase in diuretic use, no serious adverse events and no heart failure related admissions occurred. Conclusion Treatment of sRV heart failure patients with sacubitril/valsartan leads to a significant reduction in NT-proBNP and significant increase in FAC. All patients remained clinically stable and no heart failure related admissions or serious adverse events occurred. Changes after 6 months Funding Acknowledgement Type of funding source: None

Background: Exercise testing is commonly performed in children, but protocols and guidelines are optimized for the adult population. Our aim was to identify factors that may influence the objective versus subjective effort on exercise tests in children, as indicated by the peak respiratory exchange