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Background Sacubitril/valsartan has been shown to reduce mortality and morbidity inpatients with heart failure and reduced systolic function. However, the effects of this novel association in patients with congenital heart disease and a systemic right ventricle (sRV) have not been investigated yet. Purpose We aimed to assess tolerability and efficacy of sacubitril/valsartan in patients with a sRV Methods From September 2020 to March 2021, 38 patients with congenitally corrected transposition of the great arteries or transposition of the great arteries after Senning or Mustard repair were prospectively enrolled. Inclusion criteria were: age ≥18 years, optimal medical therapy including ACEi/ARB for at least 6 months and EF of the sRV ≤40%. Patients with univentricular physiology, systolic blood pressure (SBP) 5.5mEq/L were excluded. RV systolic function was assessed on echocardiography using a multiparametric evaluation. The study protocol contemplates serial assessments at 1, 3, 6 and 12 months after treatment initiation. Results Up to March 31th, 23 patients completed 1-month and 15 completed 3-month assessment after treatment initiation. Baseline patients' characteristics are summarized in table 1. The medication dose was up-titrated to the highest tolerated dose during follow-up. During early follow-up, no major adverse events were reported. Treatment did not impact significantly on the values of serum potassium (basal K+ 4.4 [4.2–4.6] mEq/L, K+ at 3 months 4.4 [4.3–4.6] mEq/L, p=0.7) and GFR (basal GFR 113.9±35ml/min, GFR at 3 months 107.8±21 ml/min, p=0.7). Although SBP did not change significantly (114±12 vs 113.9±19 mmHg at 1-month and 117.3±12 mmHg at 3 months; p=0.9 for both), 2 (5%) patients ceased the treatment due to symptomatic hypotension during the first month of treatment. There was no significant change in the NYHA class. However, the 6-minute walking distance increased significantly after 3 months (365±120 vs 498.3±71 min; p=0.01). Furthermore, while traditional echocardiographic parameters of RV systolic function (TAPSE, s wave and FAC) did not change significantly, RV global longitudinal strain (GLS) and RV free wall GLS demonstrated subclinical improvement in right ventricular systolic function (table 2). Conclusions Our short-term results from an ongoing prospective study showed that sacubitril/valsartan is well tolerated in patients with a sRV with early evidence of improvement in exercise tolerance and sRV systolic function. Longer follow-up is warranted to confirm these data. Funding Acknowledgement Type of funding sources: None. Table 1Table 2

Introduction Brain natriuretic peptide (BNP) is a well-established marker for heart failure in the general population however limited data are available on the value of BNP as a diagnostic and prognostic marker in adults with congenital heart disease (ACHD). The purpose of our study is to evaluate the relation between BNP levels and biventricular systolic and diastolic function in patients with ACHD. Methods We evaluated clinically stable ACHD patients who underwent echocardiography from May 2015 until January 2018. We studied the correlation of BNP with the degree of systolic and diastolic dysfunction of the left ventricle. Moreover we also investigate the relation of BNP with the right tricuspid annular plane systolic excursion (TAPSE), the fractional area change (FAC) and the pulmonary arterial systolic pressure (PASP). Results In total, 385 patients were included in our study (median age, 43 ±12 years; 61% male). Of the 385 patients, 193 (50%) had tetralogy of Fallot, 94 (24%) had systemic RV, including patients with transposition of the great arteries (TGA) after atrial switch operation (Mustard or Senning) and congenitally corrected TGA -ccTGA , 43 (11%) had univentricular hearts and Fontan physiology, 55 (14%) had other lesions. BNP levels were 66 ± 26 pg/mL and 93, ±31pg/mL, for patients with EF > 50% and 50%> EF >35% respectively (p = 0.003). BNP levels correlate with parameters reflecting LV filling pressure, including transmitral early diastolic velocity (E) 81 ± 29pg/mL and its ratio to early diastolic annular myocardial tissue velocity (E/Ea) 77 ± 17pg/mL. (r=-0.607, p = 0.003 r=-0.598, p = 0.005, respectively) BNP levels were also significantly higher in patients with impaired tricuspid annular plane systolic excursion 87 ± 21 pg/mL (TAPSE

Background Surgical repair of transposition of the great arteries (TGA) is most commonly via the arterial switch operation (ASO). This involves translocation of the aorta and pulmonary trunk, typically with anastomosis of the branch pulmonary arteries anteriorly (LeCompte manoeuvre) and re-implantation of the coronary arteries onto the posterior neo-aorta. As such, the position of the coronary ostia may differ from their expected locations. Purpose To use ECG-gated CT angiography to describe the anatomic position of coronary ostia in post-switch TGA patients guiding potential catheter interventions in this population. Methods All post ASO patients who underwent CT imaging between 2008–2018 were identified. Patients with complex anatomy such as double outlet right ventricle were excluded. The positions of the coronary ostia were measured in degrees from vertical on a double-oblique reconstruction in the aortic valve plane. Ostium positions were compared to those of patients with no congenital heart disease via Watson's two-sample test of homogeneity for circular data. Angular dispersion was compared between groups via the Wallraff test. P Results Of 206 adult patients with TGA and ASO followed in our adult congenital heart disease centre, 38 (18.4%) had CT imaging available for analysis during the study period (mean age 24±6.8, 75% male). The control group consisted of 15 patients investigated for chest pain (mean age 54±15.1, 73% male). In the control group, the right and left coronary ostia arose at a mean angle of −19 and +125 degrees from vertical (figure 1a). This was significantly different to the mean ASO coronary ostia clustered at mean angles of −70 and +29 degrees from vertical (Watson p There was no significant difference in spread of left ostia (Rho 0.9 vs 0.99, p=0.12), but right ostia were significantly more variable in ASO patients than controls (0.71 vs 0.96, p=0.003). Figure 1 Conclusions Coronary ostial positions in the neo-aorta of post-ASO patients differ significantly from those of normal controls, with considerable variability, especially in right coronary position. CT can demonstrate coronary ostia in 3D space and derive appropriate tube angles to guide catheterisation in post-operative congenital cardiac patients, optimise catheter selection, reduce catheterisation tome, radiation and contrast dose.

Introduction Cardiac resynchronization therapy (CRT) has become a treatment of choice in patients with chronic heart failure (HF). About 25% of patients with systemic right ventricle (SRV) progress to symptomatic HF, which may be refractory to drug therapy and is commonly associated with significant morbidity and mortality. For these reasons, CRT has been emerging as an effective treatment strategy for patients with SRV failure and electrocardiographic signs of ventricular dyssynchrony. Few studies have reported the acute and long - term effects of CRT in SRV subjects, with different findings. Our study aimed to describe the experience with CRT in SRV patients in a single tertiary centre. Purpose Assess the long term efficacy of CRT in patient with SRV Materials and methods All consecutive SRV patients who underwent CRT implantation and/or upgrading between 1994 and 2018 at our tertiary centre were included. Clinical and echocardiographic parameters before and after CRT implantation were collected and analyzed. Results A total of 21 patients (mean age 47.8±14.8 years, 13 M) were implanted with CRT-P (12, 57%) or CRT-D (9,43%) during the study period. 90% of patients showed an anatomy of congenitally corrected transposition of the great arteries (CCTGA), whereas 9.5% underwent Mustard procedure for transposition of the great arteries (TGA). Among CCTGAs, 11 (52.5%) subjects underwent previous surgical procedures, including implant of a conduit between the left ventricle (LV) and the pulmonary artery (PA) in 8 (38%) patients, tricuspid valve repair in 2 (9.5%) and surgical closure of atrial septal defect in 1 (5%). Before CRT implant/upgrading, 10 (48%) patients had a moderate to severe reduction in the SRV ejection fraction (EF) and 7 (33%) had a moderate to severe tricuspid regurgitation (TR). Overall, 15 (71%) patients referred a NYHA II or III. After a median follow up of 57 months (IQR 35–83), 43% of patients showed an improvement in their functional status, which was associated with an improvement of SRV EF and TR only in 22% and 33% of these patients. On the contrary, no patient reported a worsening in NYHA class, while SRV EF decreased in 28.5% and the grade of TR worsen in 23.8% of patients. Conclusions CRT is emerging as an effective treatment for SRV dysfunction. However, criteria for implantation are not well defined and the deterioration of SRV function related to subpulmonary univentricular pacing should be considered. Moreover, TR did not improve in this study, suggesting that concurrent tricuspid valve interventions may be necessary in patients with severe TR and may facilitate the improvement in RV function achieved with CRT. Proper planning, tertiary expertise and international collaborations are all paramount in this field.