Imperial College London

DrSonyaBabu-Narayan

Faculty of MedicineNational Heart & Lung Institute

Reader in Adult Congenital Heart Disease
 
 
 
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Contact

 

+44 (0)20 7351 8803s.babu-narayan

 
 
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Location

 

NIHR Cardiovascular Biomedical RChelsea WingRoyal Brompton Campus

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Summary

 

Publications

Publication Type
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176 results found

Ghonim S, Jenkins S, Smith GC, Mathew G, Gatzoulis MA, Gatehouse P, Babu-Narayan SVet al., 2018, 9 ECV and T1 mapping in repaired tetralogy of fallot – CMR diffuse fibrosis measurement needs the right method for the right ventricle?, Joint Meeting of the British-Society-of-Cardiovascular-Imaging/British-Society-of-Cardiovascular-CT, British-Society-of-Cardiovascular-Magnetic-Resonance and British-Nuclear-Cardiac-Society on British Cardiovascular Imaging

Introduction It is increasingly appreciated applying parametric mapping to the RV has inherent challenges.Methods We studied native LV and RV T1 mapping and ECV measures at 1.5 T in repaired tetralogy of Fallot (rTOF) patients (n=44, 24 male, 32±14 years, 35 (80%), NYHA class I). Single slices targeted perpendicular to to the LV septum or RV inferior wall using 11HB-MOLLI (6 mm slice thickness/TR 279 ms/TE 1.1 ms/Flip-angle 35°). Like image planes were repeated using ‘high sensitivity native T1’ 14HB-MOLLI (6 mm slice thickness/TR 300 ms/TE 1.1 ms/Flip-angle 5°) in attempt to improve sensitivity to tissue collagen. Haematocrit for ECV calculation was obtained within a few hours of CMR.Results RVECV correlated with LVECV (r=0.7; p<0.001). Nineteen (46%) had increased RVECV (>35%) and 3 (8%) increased LVECV (>30%). Associations with all standard risk factors in rTOF were tested. RVECV correlated with right atrial area, (r=0.4; p<0.05). ‘High-sensitivity native T1’ correlated with akinetic RVOT length (r=0.6; p<0.05), and left atrial area (r=0.3; p=0.07) and QRS duration (r=0.3; p=0.4). RVECV did not correlate with high-sensitivity’ native T1. No diffuse RV fibrosis measure correlated with ejection fraction.Conclusion Diffuse fibrosis was only associated with increased right atrial and RVOT akinetic area size which if true may relate to RV diastolic dysfunction. Given the lack of consistency of findings between techniques more data are needed, including determination of how the measures obtained relate to myocardial composition. Despite best efforts to obtain optimum RV T1 maps these findings suggest current approaches have limited use and dedicated RV sequence development is required.

Poster

Bokma JP, Mulder BJ, Hickey K, Babu-Narayan SV, Wald RM, Gatzoulis MA, Sleeper LA, Geva T, Valente AMet al., 2018, Prognostic Effects of Pulmonary Valve Replacement in Repaired Tetralogy of Fallot: Results From a Large Multicenter Study, Quality of Care and Outcomes Research Scientific Sessions, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Introduction: The prognostic impact and optimal timing of pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) remains a subject of debate. The objective of this study was to determine whether PVR was associated with beneficial outcome.Methods: In this multicenter, case-controlled study, clinical, ECG, cardiac magnetic resonance (CMR) and outcome data of rTOF patients were collected. A propensity score for PVR was created to adjust for baseline differences between PVR and non-PVR patients. A frailty failure time model was used to determine the association of PVR, with matching on propensity score and adjustment for other risk factors, on time to the composite of death/sustained ventricular tachycardia (VT). In addition, differential association between impact of PVR and outcome according to predefined ‘proactive’ and ‘conservative’ (see figure) criteria was studied.Results: A total of 440 PVR patients (age 24±13 years at baseline CMR, 58% male, RV ejection fraction (EF) 47±8%, QRS duration 148±27ms) and 537 non-PVR patients (age 29±15 years, 53% male, RV EF 51±8%, QRS duration 140±27ms) were included. During follow-up of 5.3±3.1 years, 41 events occurred (30 death, 11 sustained VT). The adjusted hazard ratio (HR) for death/sustained VT for patients with versus without PVR was 0.65 (95% CI: 0.31, 1.36, p=0.25). There was an increasing hazard of event across prespecified ordered PVR x criteria subgroups (Figure; P=0.044 for proactive, P=0.002 for conservative). There was a two-fold risk (hazard ratio=2.15, 95% CI: 1.34, 3.46) comparing each adjacent subgroup, suggesting benefit of PVR when conservative criteria were met, and lack of benefit of PVR when criteria were not met. A roughly similar pattern was seen according to proactive criteria.Conclusions: In this multicenter, 5-year follow-up study in patients with rTOF, conservative guidelines seemed useful in selecting patient

Conference paper

Babu-Narayan SV, Voges I, Kilner PJ, 2018, Cardiovascular magnetic resonance, Diagnosis and Management of Adult Congenital Heart Disease: Third Edition, Pages: 88-100, ISBN: 9780702069291

Book chapter

Babu-Narayan SV, Gatzoulis MA, 2018, Tetralogy of fallot, Diagnosis and Management of Adult Congenital Heart Disease: Third Edition, Pages: 474-488, ISBN: 9780702069291

Book chapter

Lee MG, Babu-Narayan S, Montanaro C, Kempny A, Uebing A, d'Udekem Y, Gatzoulis Met al., 2017, Long-term Burden of Coarctation of the Aorta: A Contemporary Review of 842 Patients Up to the Seventh Decade of Life, Scientific Sessions of the American-Heart-Association / Resuscitation Science Symposium, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

Bokma JP, Geva T, Sleeper LA, Babu Narayan SV, Wald R, Hickey K, Jansen K, Wassall R, Lu M, Gatzoulis MA, Mulder BJ, Valente AMet al., 2017, A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot, Heart, Vol: 104, Pages: 738-744, ISSN: 1355-6037

OBJECTIVE: To determine the association of pulmonary valve replacement (PVR) with death and sustained ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTOF). METHODS: Subjects with rTOF and cardiac magnetic resonance from an international registry were included. A PVR propensity score was created to adjust for baseline differences. PVR consensus criteria were predefined as pulmonary regurgitation >25% and ≥2 of the following criteria: right ventricular (RV) end-diastolic volume >160 mL/m2, RV end-systolic volume >80 mL/m2, RV ejection fraction (EF) <47%, left ventricular EF <55% and QRS duration >160 ms. The primary outcome included (aborted) death and sustained VT. The secondary outcome included heart failure, non-sustained VT and sustained supraventricular tachycardia. RESULTS: In 977 rTOF subjects (age 26±15 years, 45% PVR, follow-up 5.3±3.1 years), the primary and secondary outcomes occurred in 41 and 88 subjects, respectively. The HR for subjects with versus without PVR (time-varying covariate) was 0.65 (95% CI 0.31 to 1.36; P=0.25) for the primary outcome and 1.43 (95% CI 0.83 to 2.46; P=0.19) for the secondary outcome after adjusting for propensity and other factors. In subjects (n=426) not meeting consensus criteria, the HR for subjects with (n=132) versus without (n=294) PVR was 2.53 (95% CI 0.79 to 8.06; P=0.12) for the primary outcome and 2.31 (95% CI 1.07 to 4.97; P=0.03) for the secondary outcome. CONCLUSION: In this large multicentre rTOF cohort, PVR was not associated with a reduced rate of death and sustained VT at an average follow-up of 5.3 years. Additionally, there were more events after PVR compared with no PVR in subjects not meeting consensus criteria.

Journal article

Heng E, Gatzoulis M, Uebing A, Sethia B, Uemura H, Smith G, Diller G, McCarthy K, Ho SY, Li W, Wright P, Spadotto V, Kilner PJ, Oldershaw P, Pennell DJ, Shore DF, Babu-Narayan SVet al., 2017, Immediate and Midterm Cardiac Remodelling Following Surgical Pulmonary Valve Replacement in Adults with Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study, Circulation, Vol: 136, Pages: 1703-1713, ISSN: 0009-7322

Background: Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling.Methods: Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y).Results: There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m2; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m2; P<0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m2 and RVESVi ≤82 mL/m2. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P<0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm2/m2; P<0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (P=0.03) over a median of 9.5 years of follow-up.Conclusions: Significant right heart structural reverse remodeling takes

Journal article

Phuoc D, Coats L, O'Sullivan J, Crossland D, Haugk B, Babu-Narayan SV, Keegan J, Hudson M, Parry G, Manas D, Hasan Aet al., 2017, Combined heart-liver transplantation for failing Fontan circulation in a late survivor with single-ventricle physiology, ESC Heart Failure, Vol: 4, Pages: 675-678, ISSN: 2055-5822

Management of adults with failing Fontan physiology poses many challenges, especially as transplantation offers the only realistic alternative to palliative care. We present the first combined heart and liver transplant performed in Europe, for a late survivor of single ventricle palliation with the Fontan circulation. In addition to the conventional medical and surgical challenges posed, we highlight the management of the associated multi-organ failure with focus on the liver and novel strategies for assessment and optimization.

Journal article

Cazzoli I, Larsen SH, Kempny A, Dimopoulos K, Uebing A, Diller GP, West C, Senior R, Babu-Narayan SV, Gatzoulis MA, Li Wet al., 2017, Echocardiographic predictors of outcome in repaired Tetralogy of Fallot patients over 40 years old, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 149-149, ISSN: 0195-668X

Conference paper

Dimopoulos K, Alonso-Gonzalez R, Wort SJ, Swan L, Uebing A, Babu-Narayan SV, Gatzoulis MA, Kempny Aet al., 2017, The incidence of aortic dissection in tetralogy of Fallot, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 56-56, ISSN: 0195-668X

Conference paper

Cauldwell M, Quail M, Smith GS, Heng EL, Ghonim S, Uebing A, Swan L, Li W, Patel R, Pennell D, Steer P, Johnson M, Gatzoulis M, Babu-Narayan SVet al., 2017, Effect of pregnancy on ventricular and aortic dimensions in repaired tetralogy of Fallot, Journal of the American Heart Association, Vol: 6, ISSN: 2047-9980

Background: To assess whether the cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects haemodynamic stability; in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation or aortic root dilatation. Methods and Results: Retrospective cohort study of women with repaired TOF with paired Cardiovascular Magnetic Resonance (CMR) scans before and after their first pregnancy (baseline RV end systolic volume index 49mL/m2 and RV end diastolic volume index 118mL/m2) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline CMR scan, time between follow up of CMR scans, QRS duration, RV ejection fraction and indexed RV end systolic and diastolic volume at baseline. The effect of pregnancy and time on parameters was assessed using mixed effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions or exercise data. There was an effect of pregnancy observed in both LV EDV and LV SV, consistent with a sustained small increase in LV SV due to pregnancy (53ml/m2 to 55ml/m2).Conclusion: Women with repaired TOF and with mild to moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that pre pregnancy counselling is tailored to their individual clinical status.

Journal article

Rydman R, Shiina Y, Diller GP, Niwa K, Li W, Uemura H, Uebing A, Barbero U, Bouzas B, Ernst S, Wong T, Pennell D, Gatzoulis M, Babu-Narayan SVet al., 2017, Major adverse events and atrial tachycardia in Ebstein’s anomaly by cardiovascular magnetic resonance, Heart, Vol: 104, Pages: 37-44, ISSN: 1468-201X

Objectives Patients with Ebstein’s anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. Methods Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACE: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT).Results CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06[95%CI 1.168-3.623],p=0.012 and HR 2.35[95%CI 1.348-4.082],p=0.003, respectively), LV stroke volume index (HR 2.82[95%CI 1.212-7.092],p=0.028) and cardiac index (HR 1.71[95%CI 1.002-1.366],p=0.037);all remained significant when tested solely for mortality. Prior history of AT (HR 11.16[95%CI 1.30-95.81],p=0.028) and NYHA-class >2 (HR 7.66[95%CI 1.54-38.20],p=0.013) were also associated with MACE; AT preceded all but one MACE events suggesting its potential role as an early marker of adverse outcome (p=0.011). CMR variables associated with first-onset AT (n=17;21.5%) included RVEF (HR 1.55[95%CI 1.103-2.160],p=0.011)],total R/L volume index (HR 1.18[95%CI 1.06-1.32],p=0.002), RV/LV end diastolic volume ratio (HR 1.55[95%CI 1.14-2.10],p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03[95%CI 1.00-1.07],p=0.041); the latter two combined enhanced risk prediction (HR 6.12[95% CI 1.67-22.56],p=0.007). Conclusion CMR-derived indices carry prognostic information regarding MACE and first-onset AT amongst adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Journal article

Ghonim S, Voges I, Gatehouse PD, Keegan J, Gatzoulis MA, Kilner PJ, Babu-Narayan SVet al., 2017, Myocardial Architecture, Mechanics, and Fibrosis in Congenital Heart Disease, Frontiers in Cardiovascular Medicine, Vol: 4, ISSN: 2297-055X

Congenital heart disease (CHD) is the most common category of birth defect, affecting1% of the population and requiring cardiovascular surgery in the first months of lifein many patients. Due to advances in congenital cardiovascular surgery and patientmanagement, most children with CHD now survive into adulthood. However, residualand postoperative defects are common resulting in abnormal hemodynamics, whichmay interact further with scar formation related to surgical procedures. Cardiovascularmagnetic resonance (CMR) has become an important diagnostic imaging modality inthe long-term management of CHD patients. It is the gold standard technique to assessventricular volumes and systolic function. Besides this, advanced CMR techniques allowthe acquisition of more detailed information about myocardial architecture, ventricularmechanics, and fibrosis. The left ventricle (LV) and right ventricle have unique myocardialarchitecture that underpins their mechanics; however, this becomes disorganized underconditions of volume and pressure overload. CMR diffusion tensor imaging is able tointerrogate non-invasively the principal alignments of microstructures in the left ventricularwall. Myocardial tissue tagging (displacement encoding using stimulated echoes) andfeature tracking are CMR techniques that can be used to examine the deformation andstrain of the myocardium in CHD, whereas 3D feature tracking can assess the twistingmotion of the LV chamber. Late gadolinium enhancement imaging and more recently T1mapping can help in detecting fibrotic myocardial changes and evolve our understandingof the pathophysiology of CHD patients. This review not only gives an overview aboutavailable or emerging CMR techniques for assessing myocardial mechanics and fibrosisbut it also describes their clinical value and how they can be used to detect abnormalitiesin myocardial architecture and mechanics in CHD patients.

Journal article

Giannakidis A, Kamnitsas K, Spadotto V, Keegan J, Smith G, Glocker B, Rueckert D, Ernst S, Gatzoulis MA, Pennell DJ, Babu-Narayan S, Firmin DNet al., 2017, Fast fully automatic segmentation of the severely abnormal human right ventricle from cardiovascular magnetic resonance images using a multi-scale 3D convolutional neural network, 12th International Conference on Signal-Image Technology and Internet-Based Systems (SITIS), Publisher: IEEE, Pages: 42-46

Cardiac magnetic resonance (CMR) is regarded as the reference examination for cardiac morphology in tetralogy of Fallot (ToF) patients allowing images of high spatial resolution and high contrast. The detailed knowledge of the right ventricular anatomy is critical in ToF management. The segmentation of the right ventricle (RV) in CMR images from ToF patients is a challenging task due to the high shape and image quality variability. In this paper we propose a fully automatic deep learning-based framework to segment the RV from CMR anatomical images of the whole heart. We adopt a 3D multi-scale deep convolutional neural network to identify pixels that belong to the RV. Our robust segmentation framework was tested on 26 ToF patients achieving a Dice similarity coefficient of 0.8281±0.1010 with reference to manual annotations performed by expert cardiologists. The proposed technique is also computationally efficient, which may further facilitate its adoption in the clinical routine.

Conference paper

Bonello B, Shore DF, Uebing A, Diller GP, Keegan J, Burman ED, Shiina Y, Swan L, Pennell DJ, Kilner PJ, Beurtheret S, Gatzoulis MA, Babu-Narayan SVet al., 2017, Aortic Dilatation in Repaired Tetralogy of Fallot., JACC: Cardiovascular Imaging, Vol: 11, Pages: 150-152, ISSN: 1936-878X

Journal article

Bonello B, Shore D, Uebing A, Diller G, Keegan J, Burman E, Shiina Y, Swan L, Pennell D, Kilner P, Beurtheret S, Gatzoulis M, Babu-narayan Set al., 2017, Aortic dilatation in repaired tetralogy of Fallot: features, determinants and progression, Journal of the American College of Cardiology, ISSN: 1558-3597

Journal article

Giannakidis A, Oktay O, Keegan J, Spadotto V, Voges I, Smith G, Pierce I, Bai W, Rueckert D, Ernst S, Gatzoulis MA, Pennell DJ, Babu-Narayan S, Firmin DNet al., 2017, Super-resolution Reconstruction of Late Gadolinium Cardiovascular Magnetic Resonance Images using a Residual Convolutional Neural Network, The 25th Scientific Meeting of the International Society for Magnetic Resonance in Medicine (ISMRM 2017)

Conference paper

Voges I, Franklin RC, Wage R, Babu-Narayan SVet al., 2017, Fatal severe coronary artery stenosis in Williams syndrome: decision making using late gadolinium enhancement cardiovascular MRI., Cardiology in the Young, Vol: 27, Pages: 1398-1401, ISSN: 1047-9511

Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.

Journal article

Beurtheret S, Tutarel O, Diller GP, West C, Ntalarizou E, Resseguier N, Papaioannou V, Jabbour R, Simpkin V, Bastin AJ, Babu-Narayan SV, Bonello B, Li W, Sethia B, Uemura H, Gatzoulis MA, Shore Det al., 2017, Contemporary cardiac surgery for adults with congenital heart disease, Heart, Vol: 103, Pages: 1194-1202, ISSN: 1355-6037

Objective Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery.Methods This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified.Results Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline.Conclusions Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only.

Journal article

Midha D, Chen Z, Jones DG, Williams HJ, Lascelles K, Jarman J, Clague J, Till J, Dimopoulos K, Babu-Narayan SV, Markides V, Gatzoulis MA, Wong Tet al., 2017, Pacing in congenital heart disease - A four-decade experience in a single tertiary centre, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 241, Pages: 177-181, ISSN: 0167-5273

Journal article

Pennell DJ, Baksi AJ, Prasad SK, Mohiaddin RH, Alpendurada F, Babu-Narayan SV, Schneider JE, Firmin DNet al., 2016, Review of Journal of Cardiovascular Magnetic Resonance 2015, Journal of Cardiovascular Magnetic Resonance, Vol: 18, Pages: 86-86, ISSN: 1097-6647

There were 116 articles published in the Journal of Cardiovascular Magnetic Resonance (JCMR) in 2015, which is a 14 % increase on the 102 articles published in 2014. The quality of the submissions continues to increase. The 2015 JCMR Impact Factor (which is published in June 2016) rose to 5.75 from 4.72 for 2014 (as published in June 2015), which is the highest impact factor ever recorded for JCMR. The 2015 impact factor means that the JCMR papers that were published in 2013 and 2014 were cited on average 5.75 times in 2015. The impact factor undergoes natural variation according to citation rates of papers in the 2 years following publication, and is significantly influenced by highly cited papers such as official reports. However, the progress of the journal's impact over the last 5 years has been impressive. Our acceptance rate is < 25 % and has been falling because the number of articles being submitted has been increasing. In accordance with Open-Access publishing, the JCMR articles go on-line as they are accepted with no collating of the articles into sections or special thematic issues. For this reason, the Editors have felt that it is useful once per calendar year to summarize the papers for the readership into broad areas of interest or theme, so that areas of interest can be reviewed in a single article in relation to each other and other recent JCMR articles. The papers are presented in broad themes and set in context with related literature and previously published JCMR papers to guide continuity of thought in the journal. We hope that you find the open-access system increases wider reading and citation of your papers, and that you will continue to send your quality papers to JCMR for publication.

Journal article

Vassiliou VS, Heng EL, Gatehouse PD, Donovan J, Raphael CE, Giri S, Babu-Narayan SV, Gatzoulis MA, Pennell DJ, Prasad SK, Firmin DNet al., 2016, Magnetic resonance imaging phantoms for quality-control of myocardial T1 and ECV mapping: specific formulation, long-term stability and variation with heart rate and temperature, Journal of Cardiovascular Magnetic Resonance, Vol: 18, ISSN: 1532-429X

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 <2 % change. Temperature sensitivity testing showed MOLLI T1 values in the long T1 phantoms increasing by 23.9 ms per degree increase and short T1 phantoms increasing by 0.3 ms per degree increase. There was a small absolute increase in ECV of 0.069 % (~0.22 % relative increase in ECV) per degree increase. Variation in heart rate testing showed a 0.13 % absolute increase in ECV (~0.45 % relative increase in ECV) per 10 heart rate increase. CONCLUSIONS: These are the first phantoms reported in the literature modeling T1 and T2 values for blood and myocardium specifically for the T1mapping/ECV mapping application, with stability tested rigorously over a 12 month period. This work has significant implications for the utility of such phantoms in improving the accuracy of serial scans for myocardial tissue characterisation by T1 mapping methods and in multicentre work.

Journal article

Vescovo GM, Kempny A, Alonso-Gonzalez R, Swan L, Marchewka D, Gruebler MR, Uebing A, Babu-Narayan S, Gatzoulis MA, Dimopoulos Ket al., 2016, Does the ECG still carry prognostic power in contemporary patients with repaired tetralogy of Fallot?, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 763-763, ISSN: 0195-668X

Conference paper

Montanaro C, Alvarez-Alvarez B, Ministeri M, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan S, Gatzoulis M, Dimopoulos Ket al., 2016, Pulmonary hypertension in patients with transposition of great arteries or pulmonary atresia, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 158-158, ISSN: 0195-668X

Conference paper

Spadotto V, Voges I, Kilner PJ, Yacoub MH, Ernst S, Ho SY, Babu-Narayan SVet al., 2016, Juxtaposition of the atrial appendages: A nidus for thrombus in atriopulmonary Fontan?, Global Cardiology Science & Practice, Vol: 2016, ISSN: 2305-7823

Juxtaposition of atrial appendages is a rare cardiac congenital anomaly, usually associated with other cardiac malformations. Until now, it has not been linked to any significant clinical implications. We report cardiovascular magnetic resonance (CMR) findings of two adult patients who underwent atriopulmonary Fontan operation in the setting of left juxtaposition of the atrial appendages. The patients were in sinus rhythm at the time of the CMR study. Both patients had episodes of sustained atrial tachyarrhythmia requiring electrical cardioversion and were anticoagulated with warfarin with target INR 2-3. CMR images showed a thrombus located in the enlarged and juxtaposed right appendage in both patients. Blood flow frequently appears slow or sluggish in the dilated right atrium following atriopulmonary Fontan surgery. In addition, cine CMR suggested that blood flow reaches very low velocities in the massively dilated juxtaposed right atrial appendage cul-de-sac, thus potentially creating a substrate for clot formation. These findings propose that juxtaposed atrial appendages in atriopulmonary Fontan is an additional risk factor for clot formation, specifically in the dilated right atrial appendage on the left side juxtaposed with the left atrial appendage and that prophylactic anticoagulation is highly justified in these patients.

Journal article

Babu-Narayan SV, Prati D, Rydman R, Dimopoulos K, Diller GP, Uebing A, Henein MY, Kilner PJ, Gatzoulis MA, Li Wet al., 2016, Dyssynchrony and Electromechanical delay are associated with focal fibrosis in the Systemic Right Ventricle - Insights from Echocardiography, International Journal of Cardiology, Vol: 220, Pages: 382-388, ISSN: 1874-1754

BackgroundSystemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity.Methods and resultsTwenty-two consecutive patients, mean age 28 ± 8 years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P = 0.002 & P = 0.049), prolonged systemic RV isovolumic contraction time (P = 0.024) and reduced systemic RV free wall and septal excursion (P = 0.027 & P = 0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r = 0.73 & P = 0.004 and r = 0.62 & P = 0.041, respectively, and QRS duration r = 0.68, P < 0.01) and was inversely related to systolic RV free wall shortening velocity (r = − 0.59 & P = 0.042). The presence of LGE was also related to lower exercise capacity, ≥ mild tricuspid regurgitation and more arrhythmia (P = 0.008, P = 0.014 and P = 0.040). RV free wall excursion and systolic tissue Doppler velocity were related to CMR derived RV ejection fraction (r = 0.51, P = 0.015, and r = 0.77, P = < 0.001, respectively).ConclusionPost Mustard repair, myocardial fibrosis is related to dyssynchrony, RV long axis dysfunction and tricuspid regurgitation. Echocardiographic measurements of systemic RV function can be confidently used in serial follow-up following Mustard operation.

Journal article

Cordina R, von Klemperer K, Kempny A, West C, Senior R, Celermajer D, Gatzoulis MA, Babu-Narayan SV, Li Wet al., 2016, Echocardiographic predictors of mortality in adults with a fontan circulation, Journal of the American College of Cardiology: Cardiovascular Imaging, Vol: 10, Pages: 212-213, ISSN: 1936-878X

Journal article

Rydman R, Shiina Y, Diller G-P, Niwa K, Li W, Uemura H, Uebing A, Ernst S, Wong T, Pennell DJ, Gatzoulis MA, Babu-Narayan SVet al., 2016, MORTALITY AND VT IN EBSTEIN'S ANOMALY OF THE TRICUSPID VALVE: A PROSPECTIVE CARDIOVASCULAR MAGNETIC RESONANCE STUDY, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A27-A27, ISSN: 1355-6037

Conference paper

Heng EL, Gatzoulis MA, Uebing A, Sethia B, Uemura H, Smith GC, Diller G-P, McCarthy KP, Ho SY, Li W, Wright P, Spadotto V, Kilner PJ, Oldershaw P, Pennell DJ, Shore DF, Babu-Narayan SVet al., 2016, EARLY CARDIAC REMODELLING AFTER PULMONARY VALVE REPLACEMENT IN PATIENTS WITH REPAIRED TETRALOGY OF FALLOT, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A26-A26, ISSN: 1355-6037

Conference paper

Bonello B, Shore DF, Uebing A, Diller G-P, Keegan J, Burman E, Shiina Y, Swan L, Pennell D, Kilner PJ, Beurtheret S, Gatzoulis MA, Babu-Narayan SVet al., 2016, AORTIC DILATATION IN REPAIRED TETRALOGY OF FALLOT: FEATURES, DETERMINANTS AND PROGRESSION, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A2-A3, ISSN: 1355-6037

Conference paper

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