Circulating NT-proBNP levels and several surface ECG parameters constitute safe, cost-effective and widely available surrogate markers of systemic right ventricular function and provide additional information on heart failure status. Both measures hold promise as prognostic markers and their association with long-term outcome should be determined.

This is the accepted version of the paper.This version of the publication may differ from the final published version. Tel No: +44 207 040 5767, Fax No: +44 207 0408750 Email: Stanton.newman.1@city.ac.uk Word Count: 3,215(excluding title page, abstract, key questions, references, figures, tables). Permanent repository linkKey Words: Adult Congenital Heart Disease, Cognition, Neuropsychological assessment, Mood 3 ABSTRACT ObjectiveA cross-sectional study assessed cognitive function in a sample of Adult Congenital Heart Disease patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. MethodsThree hundred and ten patients participated. Patients were classified into four structural complexity groups (Tetralogy of Fallot, Transposition of the Great Arteries, Single Ventricle and Simple conditions). Each participant underwent neuropsychological assessment to evaluate cognitive function (memory, executive function) and completed questionnaires to assess depression and anxiety. ResultsForty one percent of the sample showed impaired performance (>1.5 SD below the normative mean) on at least 3 tests of cognitive function compared to established normative data. This was higher than the 8% that would be expected in a normal population. The sample exhibited significant deficits in divided attention, motor function and executive functioning. There was a significant group difference in divided attention (F=5.01, p=.002) and mean total composite score (F=5.19, p=.002) between different structural complexity groups with the Simple group displaying better cognitive function. ConclusionThe results indicate that many Adult Congenital Heart Disease patients display impaired cognitive function relative to a healthy population, which differs in relation to disease complexity. These findings may have implications for clinical decision making in this group of patients during childhood. Possible mechanisms underlying these deficits and how they may be 4 reduced or prevented are discussed; however further work is needed to draw conclusive judgments.

AimsThe purpose of this study was to create an epicardial electroanatomic map of the right ventricle (RV) and then apply post-operative-targeted single- and dual-site RV temporary pacing with measurement of haemodynamic parameters. Cardiac resynchronization therapy is an established treatment for symptomatic left ventricular (LV) dysfunction. In congenital heart disease, RV dysfunction is a common cause of morbidity—little is known regarding the potential benefits of CRT in this setting.Methods and resultsSixteen adults (age = 32 ± 8 years; 6 M, 10 F) with right bundle branch block (RBBB) and repaired tetralogy of Fallot (n = 8) or corrected congenital pulmonary stenosis (n = 8) undergoing surgical pulmonary valve replacement (PVR) for pulmonary regurgitation underwent epicardial RV mapping and haemodynamic assessment of random pacing configurations including the site of latest RV activation. The pre-operative pulmonary regurgitant fraction was 49 ± 10%; mean LV end-diastolic volume (EDV) 85 ± 19 mL/min/m2 and RVEDV 183 ± 89 mL/min/m2 on cardiac magnetic resonance imaging. The mean pre-operative QRS duration is 136 ± 26 ms. The commonest site of latest activation was the RV free wall and DDD pacing here alone or combined with RV apical pacing resulted in significant increases in cardiac output (CO) vs. AAI pacing (P < 0.01 all measures). DDDRV alternative site pacing significantly improved CO by 16% vs. AAI (P = 0.018), and 8.5% vs. DDDRV apical pacing (P = 0.02).ConclusionSingle-site RV pacing targeted to the region of latest activation in patients with RBBB undergoing PVR induces acute improvements in haemodynamics and supports the concept of ‘RV CRT’. Targeted pacing in such patients has therapeutic potential both post-operatively and in the long term.