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Background/Aims Most transcatheter aortic valve implantation procedures are now performed under conscious sedation, rather than general anaesthetic. This study evaluated nurse-led conscious sedation, compared with anaesthetist-led sedation, to determine the feasibility of the former. Methods Consecutive patients undergoing transcatheter aortic valve implantation under either nurse-led or anaesthetist-led sedation between July 2018 and September 2021 were reviewed. Assessed outcomes were analysed and compared, including 30-day mortality rate, major vascular access site bleeding, moderate or severe aortic regurgitation, stroke, new pacemaker implantation, duration of procedure and length of stay. Results A total of 212 patients who underwent nurse-led sedation and 412 who underwent anaesthetist-led sedation were identified. There were no significant differences in risk scores between the two groups. In both groups, incidence of conversion to general anaesthetic was low, with nurse sedatitionists seeking anaesthetist support in 13 (6.2%) cases. Cases with nurse-led sedation had a significantly lower average duration of the procedure (90 minutes vs 111 minutes; P=0.001) and length of stay (2 days vs 3 days, P=0.0002). Conclusions Nurse-led sedation can be safely incorporated into transcatheter aortic valve implantation practice. Nurse-led sedation delivered similar outcomes to anaesthetist-led sedation, but with shorter procedural times and legnth of stay. This could allow more flexible scheduling, increased capacity and improved access for patients.

Background No data currently exist comparing the contemporary iterations of balloon‐expandable (BE) Edwards SAPIEN 3/Ultra and the self‐expanding (SE) Medtronic Evolut PRO/R34 valves. The aim of the study was the comparison of these transcatheter heart valves with emphasis on patients with small aortic annulus. Methods and Results In this retrospective registry, periprocedural outcomes and midterm all‐cause mortality were analyzed. A total of 1673 patients (917 SE versus 756 BE) were followed up for a median of 15 months. A total of 194 patients died (11.6%) during follow‐up. SE and BE groups showed similar survival at 1 (92.6% versus 90.6%) and 3 (80.3% versus 85.2%) years ( P log‐rank =0.136). Compared with the BE group, patients treated with the SE device had lower peak (16.3±8 mm Hg SE versus 21.9±8 mm Hg BE) and mean (8.8±5 mm Hg SE versus 11.5±5 mm Hg BE) gradients at discharge. Conversely, the BE group demonstrated lower rates of at least moderate paravalvular regurgitation postoperatively (5.6% versus 0.7% for SE and BE valves, respectively; P P log‐rank =0.042). In propensity‐matched patients treated with small transcatheter heart valve, there remained a trend for higher survival among the SE group at both 1 (97% SE versus 92.3% BE) and 3 years (91.8% SE versus 78.7% BE), P log‐rank =0.096). Conclusions Real‐world comparison of the latest‐generation SE and BE devices demonstrated similar survival up to 3 years’ follow‐up. In patients with small transcatheter heart valves, there may be a trend for improved survival among those treated with SE valves.

Aims Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. Methods and results This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate Conclusion Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.

AimsLess than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition.Methods and resultsOverall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition.ConclusionA transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.

Objectives We sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). Background To date there is no robust ri...

Background Aortic-to-right ventricle (ARV) fistula is an uncommon complication of transcatheter aortic valve implantation (TAVI). Even though surgical closure is usually the treatment of choice in such communications, percutaneous treatment options are valuable alternatives for these high-risk surgical patients. Case summary In this article, we present the percutaneous closure of an ARV fistula after TAVI, in a highly symptomatic patient with recurrent episodes of heart failure decompensation with worsening right ventricular function, who failed conservative medical treatment and was deemed inoperable. Successful closure of the fistula with the use of the Amplatzer atrial septal occluder was performed 6 months post-TAVI, under general anaesthesia and transoesophageal echocardiography (TOE). A detailed multi-modality imaging pre-procedural planning was performed utilizing 4D cardiac computed tomography and echocardiography. The patient has remained asymptomatic and in good health 5 months after the ARV fistula closure, with marked improvement in his clinical picture and echocardiographic parameters. Discussion Aortic-to-right ventricle fistulas with significant shunt post-TAVI could lead to biventricular failure and are associated with increased mortality if left untreated. This case demonstrates that TOE-guided percutaneous closure of a TAVI-related ARV fistula, although technically challenging, is feasible, and can be a valuable option for the treatment of symptomatic high-risk surgical patients.

Patients with adult congenital heart disease (ACHD) are a rapidly growing cardiovascular population with increasing health needs and co-morbidities. Furthermore, their management requires frequent and ongoing hospital visits which can be burdensome. Digital health and remote monitoring have been shown to have a vast potential to enhance delivery of healthcare for patients, reducing their need for travel to clinic appointments therefore reducing costs to the patient and the healthcare service.Patients over the age of 16 with a diagnosis of ACHD were invited to use the tailored digital application too. They were monitored for a period of 6 months. Information on patient demographics, time using the application, flagged events that prompted clinical reviews and their feedback through patient surveys were collected.A total of 103 patients were enrolled and registered to use the digital application tool. There were 57 (56%) males, median age at the time of enrolment was 39 (16-73) years. The majority (96%) had a moderate or complex ACHD according to the ACC/AHA classification. There was a total of 7 modules that were completed on a weekly basis. The median length of a participant session was 2.2 min and the mean time to complete a module was 21 s. In total, 35 (67%) felt that the application helped them better manage their cardiac condition. Almost all (94%) of patients expressed that they would like to continue using the application beyond the pilot. There were 18 flagged events during the 6 month observation period, and 50% of received early clinical intervention.Application based remote monitoring in this select group was well received and potentially holds large benefit to patients both clinically and economically. There were no safety concerns in our pilot feasibility study. Our data may inform much needed and timely investment in digital health.

Background As the ACHD population ages, an increasing number of patients are being admitted for heart failure. Management is complicated by the limited evidence on pharmacological and other therapies and lack of risk stratification tools. Improved surgical and medical treatment options in this patient cohort has improved life expectancy. Purpose Assess the mortality and rate of rehospitalisation in ACHD patients following admission with decompensated HF and identify clinical predictors of outcome. Methods All ACHD patients admitted for HF requiring IV diuresis from February 2016 to December 2020 in a single tertiary centre were included. Cox analysis was used to identify predictors of death after the index admission. Results 91 patients were included: 44.0% female, 45.8±14.2 years. The most frequent underlying diagnoses were: 15.4% with primary valve or left ventricular outflow tract disease, 12.1% with transposition of the great arteries post-atrial switch, 9.9% with tetralogy of Fallot and 30.8% had a systemic right ventricle. Most (56.7%) patients had CHD of “great” complexity according to the Bethesda classification, whereas no patients had “simple” CHD. Cyanosis was present in 26.4%. Heart failure was predominantly related to systemic ventricular dysfunction in 35.2%, subpulmonary ventricular dysfunction in 25.3%, biventricular dysfunction in 24.2% and univentricular dysfunction in 15.4%. Pulmonary hypertension was present in most (58.2%) patients, only half (46.0%) of these had pre-capillary pulmonary hypertension. On admission, approximately half of patients were on standard heart failure therapy: 48.4% ACE-inhibitors or angiotensin receptor blockers, 54.9% beta-blockers, 54.9% mineralocorticoid receptor antagonists and 2% on sacubitril/valsartan. In-hospital mortality was only 3.3% in this high-risk population. During a median follow-up of 22.1 [0.2–58.4] months, 37.5% patients were rehospitalised for HF and 37.5% patients died (22.7% mortality at 1 year). On univariable Cox analysis, the following parameters were predictors of mortality: pulmonary hypertension, cyanosis, unoperated or palliated CHD, chronic kidney disease (CKD), hyponatraemia, mineralocorticoid receptor antagonist use on admission, admission BNP>350ng/L and a maximum daily inpatient loop diuretic requirement >160mg (furosemide equivalents). On multivariable Cox analysis, pulmonary hypertension, cyanosis, hyponatraemia and CKD were independent predictors of mortality; patients with ≥2 of these risk factors had an 8-fold higher mortality (figure). Conclusion ACHD patients hospitalised for decompensated HF have a poor outlook with a quarter of patients dying within a median Funding Acknowledgement Type of funding sources: None. Figure 1

Fifty-one consecutive adult congenital heart disease (ACHD) patients attended a single site, day-case facility -in the midst of the Covid pandemic-for cardiac assessment tailored to their individual needs. All patients responded positively to our one to one invitation to attend, despite Covid restrictions, and to our anonymized questionnaire. The latter showed an overwhelming support for personalized care and for more patient education, support and empowerment. A subset of patients have since been enrolled in a pilot App with HUMA on an ACHD education, self-reporting and self-managing tool, with potential worldwide implications for ACHD care.

This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA).To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF.Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers.The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V0We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Congenital heart disease represents the most prevalent birth defect, and surgical and interventional advances have translated to a burgeoning adult population of patients. Timely, tailored, and lesion-specific interventions are necessary to optimise long-term outcomes for this complex, heterogeneous patient cohort. We present a review of the most common defects which may be encountered in general adult cardiology. Particular focus is paid to the recommended interventional options and associated complications for each condition in line with European and American guidelines.

Objectives: Our objectives involved identifying whether repeated averaging in basal and mid left ventricular myocardial levels improves precision and correlation with collagen volume fraction for 11 heartbeat MOLLI T1 mapping versus assessment at a single ventricular level. Materials and methods: For assessment of T1 mapping precision, a cohort of 15 healthy volunteers underwent two CMR scans on separate days using an 11 heartbeat MOLLI with a 5(3)3 beat scheme to measure native T1 and a 4(1)3(1)2 beat post-contrast scheme to measure post-contrast T1, allowing calculation of partition coefficient and ECV. To assess correlation of T1 mapping with collagen volume fraction, a separate cohort of ten aortic stenosis patients scheduled to undergo surgery underwent one CMR scan with this 11 heartbeat MOLLI scheme, followed by intraoperative tru-cut myocardial biopsy. Six models of myocardial diffuse fibrosis assessment were established with incremental inclusion of imaging by averaging of the basal and mid-myocardial left ventricular levels, and each model was assessed for precision and correlation with collagen volume fraction. Results: A model using 11 heart beat MOLLI imaging of two basal and two mid ventricular level averaged T1 maps provided improved precision (Intraclass correlation 0.93 vs 0.84) and correlation with histology (R2 = 0.83 vs 0.36) for diffuse fibrosis compared to a single mid-ventricular level alone. ECV was more precise and correlated better than native T1 mapping. Conclusion: T1 mapping sequences with repeated averaging could be considered for applications of 11 heartbeat MOLLI, especially when small changes in native T1/ECV might affect clinical management.

Background Adults with repaired tetralogy of Fallot (rtoF) are at risk of ventricular arrhythmia and sudden cardiac death (SCD). Cross-sectional data suggest association of late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (CMR) with adverse clinical risk factors Purpose We sought to determine prognosis related to LGE CMR. Methods In this prospective cohort study the primary composite outcome comprised the first of cardiovascular death (SCD or heart failure-related), aborted SCD (successfully resuscitated cardiac arrest or appropriate AICD shock for ventricular fibrillation), and clinical sustained ventricular tachycardia (VT>30 seconds duration). Results In 531 rtoF patients (median age 32; 23–42, 296 (56%) male, NYHA≥II 17%) followed up after LGE CMR for median 5 (1.7–8.9) years, there were 39 primary composite outcomes: 10 SCD, 11 heart failure related deaths (2 perioperative RV failure), 2 aborted SCD and 16 clinical sustained VT events. At study end, there were 28 ventricular arrhythmic events in 28 rtoF patients (10 SCD, 16 clinical sustained VT, 2 aborted VF) that were significantly predicted by RV LGE extent (HR 1.45 CI: 1.3–1.6; P Univariable predictors of the primary outcome were RV LGE score; HR: 1.44 (1.31–1.57; p In multivariable analysis both RV LGE score and indexed right atrial area (RAAi) only, remained predictive of the primary outcome (HR 1.29 CI: 1.12–1.49; p Conclusions For every unit increase in CMR defined RV fibrosis score there is a 44% increased risk of sudden cardiac death and VT. LGE CMR and maximal right atrial area should therefore be incorporated into risk stratification for sudden death in adults with rTOF. Acknowledgement/Funding British heart foundation

Cardiac fibrosis is a final common pathology in inherited and acquired heart diseases that causes cardiac electrical and pump failure. Here, we use systems genetics to identify a pro-fibrotic gene network in the diseased heart and show that this network is regulated by the E3 ubiquitin ligase WWP2, specifically by the WWP2-N terminal isoform. Importantly, the WWP2-regulated pro-fibrotic gene network is conserved across different cardiac diseases characterized by fibrosis: human and murine dilated cardiomyopathy and repaired tetralogy of Fallot. Transgenic mice lacking the N-terminal region of the WWP2 protein show improved cardiac function and reduced myocardial fibrosis in response to pressure overload or myocardial infarction. In primary cardiac fibroblasts, WWP2 positively regulates the expression of pro-fibrotic markers and extracellular matrix genes. TGFβ1 stimulation promotes nuclear translocation of the WWP2 isoforms containing the N-terminal region and their interaction with SMAD2. WWP2 mediates the TGFβ1-induced nucleocytoplasmic shuttling and transcriptional activity of SMAD2.

Cardiac fibrosis is a final common pathology in inherited and acquired heart diseases that causes cardiac electrical and pump failure. Here, we use systems genetics to identify a pro-fibrotic gene network in the diseased heart and show that this network is regulated by the E3 ubiquitin ligase WWP2, specifically by the WWP2-N terminal isoform. Importantly, the WWP2-regulated pro-fibrotic gene network is conserved across different cardiac diseases characterized by fibrosis: human and murine dilated cardiomyopathy and repaired tetralogy of Fallot. Transgenic mice lacking the N-terminal region of the WWP2 protein show improved cardiac function and reduced myocardial fibrosis in response to pressure overload or myocardial infarction. In primary cardiac fibroblasts, WWP2 positively regulates the expression of pro-fibrotic markers and extracellular matrix genes. TGFβ1 stimulation promotes nuclear translocation of the WWP2 isoforms containing the N-terminal region and their interaction with SMAD2. WWP2 mediates the TGFβ1-induced nucleocytoplasmic shuttling and transcriptional activity of SMAD2., Pathological cardiac fibrosis is a hallmark of diseases leading to heart failure. Here, the authors used systems genetics to identify a pro-fibrotic gene network regulated by WWP2, a E3 ubiquitin ligase, which orchestrates the nucleocytoplasmic shuttling and transcriptional activity of SMAD2 in the diseased heart.

Introduction There is growing recognition of Fontan-associated liver disease (FALD), which encompasses a spectrum ranging from liver congestion to cirrhosis and hepatocellular carcinoma. Diagnostic pathways remain poorly defined, with no consensus on optimal screening and management modalities. Aim of this study was to assess prevalence and severity of FALD by implementation of a structured screening programme. Methods A cohort of 93 Fontan patients aged ≥ 16 years underwent a screening protocol for liver disease at our tertiary Adult Congenital Heart Disease Centre, including blood tests, liver ultrasound with elastography, and fibroscan. Results were discussed in a MDM and patients categorised according to the risk of having liver fibrosis. MRI, CT scan and/or endoscopy were also organised when appropriate. Patients were followed up yearly if the initial tests were reassuring, or started on 6-montly HCC surveillance if the initial screening suggested advanced fibrosis. Results The study cohort comprised 55 (59%) males, mean age 25.4±6.2 years, and median 18 years since Fontan surgery. Most patients were asymptomatic (79%), with good haemodynamics. Liver abnormalities on ultrasonography were evident in 51% (41 of 80 scans), including coarse echotexture (41%), hepatomegaly (12%), hyper-echoic nodules (6%) and signs of portal hypertension (15%). Liver stiffness was elevated on both ultrasound elastography (mean 7.8±3.3 kPa, Philips US ElasPQ) and fibroscan (median 20.0±6.7 kPa). Liver MRIs and triple phase liver CTs, performed in a subset of the cohort, further characterised liver abnormalities, most notably focal nodular hyperplasia (FNH) in 65% (13 of 20 MRIs). Most patients had normal biochemical liver profiles, with mean values for bilirubin 18.2 ± 10.6umol/L, ALT 26.5 ± 10.6IU/L, albumin 47.5 ± 5.3g/L and platelets 169 ±55 x109, despite hepatic imaging of parenchymal abnormalities. Two patients were found to have hepatocellular carcinomas (HCC), BCLC stage B and C at diagnosis. There was a total of 4 deaths over a median follow-up of 2.8 years, of which 2 deaths were liver-related. Conclusions Elevated hepatic stiffness and ultrasound appearance in keeping with severe fibrosis are common in adult Fontan patients, despite normal liver enzymes and synthetic function. FNH is the most common abnormality on imaging in high risk patients. The incidence of HCC justifies surveillance in this group of patients, however the optimal surveillance protocol remains to be established.

Author(s): Falahatpisheh, Ahmad; Rickers, Carsten; Gabbert, Dominik; Heng, Ee Ling; Stalder, Aurelien; Kramer, Hans-Heiner; Kilner, Philip J; Kheradvar, Arash | Abstract: PurposeTo determine whether neglecting the flow unsteadiness in simplified Bernoulli's equation significantly affects the pulmonary transvalvular pressure drop estimation.Materials and methods3.0T magnetic resonance imaging (MRI) 4D velocity mapping was performed on four healthy volunteers, seven patients with repaired tetralogy of Fallot, and thirteen patients with transposition of the great arteries repaired by arterial switch. Pulmonary transvalvular pressure drop was estimated based on two methods: General Bernoulli's Equation (GBE), ie, the most complete form; and Simplified Bernoulli's Equation (SBE), known as 4V(2) . More than 2300 individual pressure drop measurements were used to compare the simplified and the general Bernoulli's methods. A linear mixed-effects model was employed for statistical analyses, fully accounting for clustering of observations among the methods and systolic phases.ResultsThe simplified Bernoulli's method systematically underestimated the pressure drop compared to general Bernoulli's method during the entire systolic phase (P l 0.05), including the peak systole, where on average ΔpSBE/ΔpGBE=78%.ConclusionThe simplified Bernoulli method underestimated the pressure drop during all systolic phases in all the studied subjects. Therefore, it is necessary to take into account the flow unsteadiness for more accurate estimation of the pressure drop. J. Magn. Reson. Imaging 2016;43:1313-1319.

We thank the authors for their interest in our recent publication in which we reported on significant right heart structural reverse remodeling seen in the immediate postsurgical pulmonary valve replacement (PVR) period in patients with repaired tetralogy of Fallot, which was followed by further time-related biological remodeling exemplified by further reduction in right ventricular (RV) end-systolic volume.1 Patients put forth for PVR following multidisciplinary review were opportunistically and serially approached by the research team (S.V.B.-N.) for study inclusion if they fulfilled the study inclusion criteria. The patients in …

Objectives Our objectives involved identifying whether repeated averaging in basal and mid left ventricular myocardial levels improves precision and correlation with collagen volume fraction for 11 heartbeat MOLLI T 1 mapping versus assessment at a single ventricular level. Materials and methods For assessment of T 1 mapping precision, a cohort of 15 healthy volunteers underwent two CMR scans on separate days using an 11 heartbeat MOLLI with a 5(3)3 beat scheme to measure native T 1 and a 4(1)3(1)2 beat post-contrast scheme to measure post-contrast T 1, allowing calculation of partition coefficient and ECV. To assess correlation of T 1 mapping with collagen volume fraction, a separate cohort of ten aortic stenosis patients scheduled to undergo surgery underwent one CMR scan with this 11 heartbeat MOLLI scheme, followed by intraoperative tru-cut myocardial biopsy. Six models of myocardial diffuse fibrosis assessment were established with incremental inclusion of imaging by averaging of the basal and mid-myocardial left ventricular levels, and each model was assessed for precision and correlation with collagen volume fraction. Results A model using 11 heart beat MOLLI imaging of two basal and two mid ventricular level averaged T 1 maps provided improved precision (Intraclass correlation 0.93 vs 0.84) and correlation with histology (R 2 = 0.83 vs 0.36) for diffuse fibrosis compared to a single mid-ventricular level alone. ECV was more precise and correlated better than native T 1 mapping. Conclusion T 1 mapping sequences with repeated averaging could be considered for applications of 11 heartbeat MOLLI, especially when small changes in native T 1/ECV might affect clinical management.

Brain natriuretic peptide (BNP) levels are elevated in patients with repaired Tetralogy of Fallot (rTOF), the clinical significance of which remains uncertain.Ninety consecutive adults (≥ 16 years) with rTOF (mean age 32.7 ± 11.3 years, 64% men) were prospectively recruited from a single tertiary centre, together with 15 age-matched and gender-matched controls. Patients with rTOF had elevated BNP (8.9 (5.9-14.6) vs 5.4 (2.2-7.5) pmol/L; p0.01), and BNP activation was common even in asymptomatic patients. Also, atrial natriuretic peptide (6.9 (4.0-9.9) vs 3.3 (1.0-4.0) pmol/L; p0.01), endothelin-1 (1.14 (0.94-1.48) vs 0.75 (0.44-0.93) pmol/L; p0.01) and renin (55.0 (45.5-66.5) vs 18.6 (12.0-22.7) pmol/L; p0.01) were elevated at baseline compared with controls. Interactions between BNP with endothelin-1, cardiothoracic ratio and right atrial area were evident. Eight deaths occurred over a median follow-up of 10 years. On Cox regression analysis, BNP emerged as a strong predictor of death (HR 1.16 per 10 pmol/L, 95% CI 1.05 to 1.29; p0.01). Survival receiver operating curve analysis revealed an optimum cut-off of BNP ≥ 15 pmol/L (=52 pg/mL), above which BNP was related to significantly increased mortality (HR 5.40, 95% CI 1.29 to 22.6; p0.01); absolute mortality at 5 years 19% vs 3% in patients with BNP ≤ 15 pmol/L. BNP was also a predictor of sustained arrhythmia (HR 2.06 per 10 pmol/L, 95% CI 1.32 to 3.21; p0.05).Neurohormonal activation is present in adults with rTOF including asymptomatic patients. BNP level ≥ 15 pmol/L is associated with a fivefold increased risk of death. These data suggest that BNP measurement in patients with rTOF should be incorporated in the periodic risk stratification assessment of these patients under lifelong follow-up.

Fragmented QRS (fQRS) results from localised disruption of conduction due to myocardial fibrosis and scar and is evident on 12-lead ECG. Inhomogeneous myocardial electrical activation consequently manifests as spikes within the QRS complex. fQRS has been shown to be associated with increased mortality, ventricular arrhythmias and major adverse coronary events across a spectrum of pathologies ranging from coronary artery disease, cardiomyopathies (dilated cardiomyopathy, hypertrophic cardiomyopathy and left ventricular non-compaction) to acute pulmonary embolism.1 fQRS has also been defined as a marker of arrhythmogenic right ventricular (RV) dysplasia, long QT and Brugada syndrome. fQRS morphologies are not only confined to acquired and hereditary cardiac conditions, but are also present on the surface ECGs of patients with congenital heart disease—including tetralogy of Fallot (TOF) and Ebstein's anomaly. fQRS has been reported to occur predominantly in the right and mid-praecordial leads in patients with repaired TOF (rTOF), with fragmented intracardiac electrograms previously demonstrated through invasive endocardial mapping.2 Until now, two cross-sectional studies of adult patients with rTOF have elucidated associations between fQRS and the extent of RV dilatation, dysfunction and myocardial fibrosis burden.3 ,4 RV fibrosis as quantified by late gadolinium enhancement (LGE) cardiovascular MR showed a strong correlation between the extent of fQRS (measured by the number of ECG leads in which fQRS was detectable) and the RV LGE score. …

Background: Magnetic resonance imaging (MRI) phantoms are routinely used for quality assurance in MRI centres; however their long term stability for verification of myocardial T1/ extracellular volume fraction (ECV) mapping has never been investigated. Methods: Nickel-chloride agarose gel phantoms were formulated in a reproducible laboratory procedure to mimic blood and myocardial T1 and T2 values, native and late after Gadolinium administration as used in T1/ECV mapping. The phantoms were imaged weekly with an 11 heart beat MOLLI sequence for T1 and long TR spin-echo sequences for T2, in a carefully controlled reproducible manner for 12 months. Results: There were only small relative changes seen in all the native and post gadolinium T1 values (up to 9.0 % maximal relative change in T1 values) or phantom ECV (up to 8.3 % maximal relative change of ECV, up to 2.2 % maximal absolute change in ECV) during this period. All native and post gadolinium T2 values remained stable over time with

Histological validation of a new CMR T1-mappingbased protocol to improve accuracy for fibrosis assessment in patients with aortic stenosis Vassilis Vassiliou, Katharina Wassilew, George Asimakopoulos, Anthony de Souza, Cesare Quarto, Ee Ling Heng, Claire E Raphael, Bruce S Spottiswoode, Andreas Greiser, Evangelia Nyktari, Francisco Alpendurada, David Firmin, Andrew Jabbour, John Pepper, Dudley J Pennell, Peter Gatehouse, Sanjay Prasad

To determine whether neglecting the flow unsteadiness in simplified Bernoulli's equation significantly affects the pulmonary transvalvular pressure drop estimation.3.0T magnetic resonance imaging (MRI) 4D velocity mapping was performed on four healthy volunteers, seven patients with repaired tetralogy of Fallot, and thirteen patients with transposition of the great arteries repaired by arterial switch. Pulmonary transvalvular pressure drop was estimated based on two methods: General Bernoulli's Equation (GBE), ie, the most complete form; and Simplified Bernoulli's Equation (SBE), known as 4V(2) . More than 2300 individual pressure drop measurements were used to compare the simplified and the general Bernoulli's methods. A linear mixed-effects model was employed for statistical analyses, fully accounting for clustering of observations among the methods and systolic phases.The simplified Bernoulli's method systematically underestimated the pressure drop compared to general Bernoulli's method during the entire systolic phase (P0.05), including the peak systole, where on average ΔpSBE/ΔpGBE=78%.The simplified Bernoulli method underestimated the pressure drop during all systolic phases in all the studied subjects. Therefore, it is necessary to take into account the flow unsteadiness for more accurate estimation of the pressure drop. J. Magn. Reson. Imaging 2016;43:1313-1319.

The success of cardiac surgery over the past six decades has led to a paradigm shift in the management of patients with repaired tetralogy of Fallot (rTOF) towards addressing late sequelae in adulthood. Although surgical techniques have evolved, late morbidity and mortality have not been abolished in this patient cohort. Significant knowledge gaps remain in mediating adverse outcomes of arrhythmias, heart failure and sudden cardiac death. Surgical repair of TOF almost inevitably results in pulmonary regurgitation (PR) during the relief of RV outflow tract obstruction. While pulmonary valve replacement (PVR) represents the current standard of care for treating the haemodynamic consequences of pulmonary dysfunction, its survival benefit and impact on long-term morbidity remains largely unproven. Contemporary guidelines advocate PVR in the presence of symptoms, significant or progressive RV dilatation or dysfunction.1 However, decision-making favouring intervention is less straightforward in the asymptomatic cohort that forms the majority of patients with rTOF with significant PR. Irreversible ventricular impairment may ensue when intervening too late with chronic volume loading, while operating prematurely may increase the lifetime cumulative number of procedures and confer surgical risk, which may increase with successive sternotomies. Ascertaining the optimal timing of PVR therefore remains elusive. Outcomes in patients with rTOF following PVR are …

Background Ventricular fibrosis in adult congenital heart disease (ACHD) has been shown to relate to adverse clinical markers.[1,2] Novel free-breathing, motion-corrected late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) has been shown to be advantageous in quantifying LV LGE, but has not been evaluated for RV LGE. [3] It may therefore also be beneficial in ACHD patients, particularly with RV LGE patterns. We sought to investigate its clinical utility in ACHD patients undergoing LGE CMR.

A 51-year-old male with known osteogenesis imperfecta (OI) (type 1) presented with symptoms and signs of infective endocarditis. Transthoracic echocardiography showed chordal rupture and free mitral regurgitation, resulting in an emergency mitral valve repair. The surgical procedure was largely uneventful but subsequent clinical course on the intensive care unit was complicated by bowel perforation requiring two laparatomies for a colonic resection and loop ileostomy formation. Histology of the excised tissue demonstrated absent musculature with no evidence of ischemia. Spontaneous non-ischemic bowel perforation as a complication of osteogenesis imperfecta is to date unreported. Our case highlights the need for a high index of suspicion of non-ischemic bowel perforation in patients with connective tissue disorders. Keywords Osteogenesis imperfecta; Bowel perforation; Collagen; Non-ischemic; Connective tissue disorders; Pathogenesis; Collagen vascular disorder; Acute abdomen

The anatomy of tetralogy of Fallot (TOF), together with the pathophysiological consequences, was first described by Etienne-Louis Fallot in 1888. The tetrad of overriding aorta, right ventricular (RV) outflow tract obstruction, ventricular septal defect (VSD) and consequent RV hypertrophy is all due to antero-cephalad deviation of the outlet septum during fetal development. TOF is the most common cyanotic congenital heart disease (CHD), accounting for 10 % of CHD patients and occurring in 1 in 3,600 births (Shinebourne et al., Heart 92:1353–1359, 2006). It carries a recurrence risk of 3 % in siblings. There is genetic microdeletion in 22q11 in 15–25 % of TOF patients in whom TOF is part of DiGeorge syndrome. There is a spectrum of morphology, despite the four features that comprise ‘tetralogy’, and the severity of the RV outflow tract (RVOT) obstruction is the major determinant of first clinical presentation. Surgical palliation was achieved using the Blalock–Taussig shunt (subclavian artery to ipsilateral pulmonary artery connection) in 1944, a landmark event as these patients underwent the first cardiac surgery. Subsequently, surgical repair was described in the 1950s with the advent of cardiopulmonary bypass. Primary surgical repair involves patch closure of the VSD and intervention to the RVOT to relieve obstruction. RV muscle bundles are resected. Depending on the size of the outflow tract, pulmonary valve and pulmonary arteries; an RVOT patch, transannular patch and/or pulmonary artery (PA) patch may be required for RVOT reconstruction. In the variants with anomalous coronary arteries or in pulmonary atresia-type Fallot, an RV to PA conduit may be used.

Background Whilst pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) is shown to provide symptomatic benefit and reduction of right ventricular (RV) volumes, there are scarce data on the rate of ventricular mechanical and biological adaptation. We aimed to assess early and late post-PVR volumetric and functional changes. Methods and Results Patients with rTOF (≥16 years) for PVR were prospectively recruited for Cardiovascular Magnetic Resonance (CMR): pre-PVR (pPVR), early post-PVR at median 6 days (ePVR) and late post-PVR at median 3 years (mPVR). Fifty-seven patients with rTOF (mean age 35.8 ± 10.1 years, 38 male) were included. There was an acute reduction in indexed RV end-diastolic (EDVi), end-systolic (ESVi) volumes and mass early post-PVR, which was sustained at latest time point (RVEDVi pPVR 156.1 ± 41.9ml/m² vs ePVR 104.9 ± 28.4ml/m² vs mPVR 104.2 ± 34.4ml/m 2 and RVESVi pPVR 74.9 ± 26.2ml/m² vs ePVR 57.4 ± 22.7ml/m² vs mPVR 50.5 ± 21.7ml/m 2 ;p 2 and RVESVi≤82ml/m 2 . PVR produced a stepwise reduction in RVESVi (load independent variable) together with an improvement in corrected RVEF after restoring valve competency (which is load dependent). There was also a modest but significant improvement of LVEF, as well as reverse right atrial remodelling. Conclusions Cardiac remodelling is generally regarded as a gradual process post-PVR. We demonstrate for the first time that the major improvement in RV volumes seen at midterm follow-up have already taken place within days after surgery. This occurs with an apparent transient impairment of RVEF, although corrected RVEF more easily illustrates the immediate effect of PVR. However, RVESVi may be a more appropriate, load-independent marker that better reflects the early and sustained benefit of PVR on RV contractility.

Methods 15 healthy volunteers (31±5 years, 8 males) with no known medical conditions, on no medication, underwent CMR scans (Avanto, Siemens, 1.5T) on two separate attendances with an 11 heart beat MOLLI 5(3)3. Following frequency adjustment, native T1 maps were obtained twice on a basal and a mid-ventricular slice respectively. 15 mins following GBCA administration (0.1 mmol/kg, Gadobutrol, Bayer, Germany) both image planes were acquired again twice. Pixel-wise T1 maps were analyzed offline by 2 independent blinded operators. A Region Of Interest was manually drawn in the septum for myocardium and blood in native and post gad images as shown in fig 1.

Background Heart failure with preserved ejection fraction (HF-PEF) is common and has a poor prognosis with a 3 year mortality rate of 23%. There are currently no effective therapies for this condition. HF-PEF is characterized by symptoms of heart failure, normal or a mildly impaired ejection fraction and evidence of adverse cardiac and vascular remodelling. A feature of all heart failure is a heightened sympathetic nervous system (SNS), which can now be abrogated using renal denervation (RD). Methods We are conducting a study of patients with HF-PEF who will be randomised (2:1) to receive RD or open control. As a phase II mechanistic study we are primarily investigating the effect of RD on patient symptoms (questionnaire), cardio-pulmonary exercise function, B-type natriuretic peptide levels, left ventricular filling pressures, left ventricular mass and left atrial volume. We are also assessing macrovascular function using aorta imaging to calculate aorta distensibility, pulse wave velocity and aortic flow. Finally