Publications
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Naqvi N, Babu-Narayan S, Krupickova S, et al., 2020, Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children, JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY, Vol: 33, Pages: 622-630, ISSN: 0894-7317
Martin-Garcia AC, Dimopoulos K, Boutsikou M, et al., 2020, Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement, HEART, Vol: 106, Pages: 455-461, ISSN: 1355-6037
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- Citations: 2
Fusco F, Shimada E, Scognamiglio G, et al., 2020, Restrictive ventricular septal defect resulting in systemic outflow obstruction in adults with Fontan circulation: challenging diagnosis of a serious and potentially fatal complication, JOURNAL OF CARDIOVASCULAR MEDICINE, Vol: 21, Pages: 276-279, ISSN: 1558-2027
Cazzoli I, Gunturiz-Beltran C, Guarguagli S, et al., 2020, Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations, International Journal of Cardiology, Vol: 301, Pages: 127-134, ISSN: 0167-5273
IntroductionArrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation.MethodsFive consecutive patients (3 female, mean age 47.8 ± 12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients.ResultsPatients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4–1375) from their last ablation. During a median follow up of 31 months (range 1–70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing.ConclusionsCatheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.
Diller G-P, Lammers AE, Babu-Narayan S, et al., 2019, Denoising and artefact removal for transthoracic echocardiographic imaging in congenital heart disease: utility of diagnosis specific deep learning algorithms, INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, Vol: 35, Pages: 2189-2196, ISSN: 1569-5794
Pushparajah K, Phuoc D, Mathur S, et al., 2019, EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE Cardiovascular MRI and CT in congenital heart disease, ECHO RESEARCH AND PRACTICE, Vol: 6, Pages: R121-R138, ISSN: 2055-0464
Li W, Yin Y, Dimopoulos K, et al., 2019, Early and late effects of cardiac resynchronization therapy in adult congenital heart disease, Journal of the American Heart Association, Vol: 8, ISSN: 2047-9980
Background: There are limited data regarding cardiac resynchronization therapy (CRT) in adult congenital heart disease (ACHD). We aimed to assess early and late outcomes of CRT amongst patients with ACHD.Methods: We retrospectively studied ACHD patients receiving CRT (2004-2017). Clinical and echocardiographic data were analyzed at baseline, early (1.8±0.8 years) and late (4.7±0.8 years) follow-up after CRT.Results: Fifty-four ACHD patients (median age 46 years, range 18-73 years, 74% male) had CRT (biventricular paced >90%) and were followed for 5.7±3.0 years. Compared to baseline, CRT was associated with significant improvement at early follow-up in NYHA functional class, QRS duration and cardiothoracic ratio (P<0.05 for all); improvement in NYHA class was sustained at late follow-up.Amongst patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction (LVEF) and reduction in LV end-systolic volume at early and late follow up (P<0.05 for both). For patients with a systemic right ventricle (RV; n=15), there was a significant early but not late reduction in systemic RV basal and longitudinal diameters.Eleven patients died and 2 had heart transplantation unrelated to systemic ventricular morphology. Thirty-five (65%) patients responded positively to CRT but only baseline QRS duration was predictive of a positive response.Conclusions: CRT results in sustained improvement in functional class, systemic LV size and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphology, were most likely to respond to CRT.
Yin Y, Dimopoulos K, Shimada E, et al., 2019, Early and late effects of cardiac resynchronization therapy in adult congenital heart disease, Congress of the European-Society-of-Cardiology (ESC) / World Congress of Cardiology, Publisher: OXFORD UNIV PRESS, Pages: 1372-1372, ISSN: 0195-668X
Martin Garcia AC, Dimopoulos K, Boutsikou M, et al., 2019, Right ventricular volume off-loading following atrial septal defect closure or pulmonary valve replacement: impact on tricuspid regurgitation and mid-term remodeling, Congress of the European-Society-of-Cardiology (ESC) / World Congress of Cardiology, Publisher: OXFORD UNIV PRESS, Pages: 2258-2258, ISSN: 0195-668X
Ghonim S, Gatzoulis MA, Smith GC, et al., 2019, LGE CMR predicts sudden death and VT in adults with repaired tetralogy of Fallot - a prospective study with 3500 patient follow up years, Congress of the European-Society-of-Cardiology (ESC) / World Congress of Cardiology, Publisher: OXFORD UNIV PRESS, Pages: 1367-1367, ISSN: 0195-668X
Diller G-P, Babu-Narayan S, Li W, et al., 2019, Utility of machine learning algorithms in assessing patients with a systemic right ventricle, EHJ Cardiovascular Imaging / European Heart Journal - Cardiovascular Imaging, Vol: 20, Pages: 925-931, ISSN: 2047-2412
Aims: To investigate the utility of novel deep learning (DL) algorithms in recognizing transposition of the great arteries (TGA) after atrial switch procedure or congenitally corrected TGA (ccTGA) based on routine transthoracic echocardiograms. In addition, the ability of DL algorithms for delineation and segmentation of the systemic ventricle was evaluated. Methods and results: In total, 132 patients (92 TGA and atrial switch and 40 with ccTGA; 60% male, age 38.3 ± 12.1 years) and 67 normal controls (57% male, age 48.5 ± 17.9 years) with routine transthoracic examinations were included. Convolutional neural networks were trained to classify patients by underlying diagnosis and a U-Net design was used to automatically segment the systemic ventricle. Convolutional networks were build based on over 100 000 frames of an apical four-chamber or parasternal short-axis view to detect underlying diagnoses. The DL algorithm had an overall accuracy of 98.0% in detecting the correct diagnosis. The U-Net architecture model correctly identified the systemic ventricle in all individuals and achieved a high performance in segmenting the systemic right or left ventricle (Dice metric between 0.79 and 0.88 depending on diagnosis) when compared with human experts. Conclusion: Our study demonstrates the potential of machine learning algorithms, trained on routine echocardiographic datasets to detect underlying diagnosis in complex congenital heart disease. Automated delineation of the ventricular area was also feasible. These methods may in future allow for the longitudinal, objective, and automated assessment of ventricular function.
Lee MGY, Babu-Narayan SV, Kempny A, et al., 2019, Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta, HEART, Vol: 105, Pages: 1190-+, ISSN: 1355-6037
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- Citations: 4
Ghonim S, Gatehouse PD, Giblin G, et al., 2019, Can RV optimised native T1 mapping and ECV add clinical value in repaired tetralogy of Fallot?, Publisher: OXFORD UNIV PRESS, Pages: 204-205, ISSN: 2047-2404
Yin YY, Dimopoulos K, Shamada E, et al., 2019, Effect of cardiac resynchronization therapy on right ventricle in adult with congenital heart disease, Publisher: WILEY, Pages: 508-508, ISSN: 1388-9842
Diller G-P, Kempny A, Babu-Narayan SV, et al., 2019, Machine learning algorithms estimating prognosis and guiding therapy in adult congenital heart disease: data from a single tertiary centre including 10 019 patients, European Heart Journal, Vol: 40, Pages: 1069-1077, ISSN: 1522-9645
Aims: To assess the utility of machine learning algorithms on estimating prognosis and guiding therapy in a large cohort of patients with adult congenital heart disease (ACHD) or pulmonary hypertension at a single, tertiary centre. Methods and results: We included 10 019 adult patients (age 36.3 ± 17.3 years) under follow-up at our institution between 2000 and 2018. Clinical and demographic data, ECG parameters, cardiopulmonary exercise testing, and selected laboratory markers where collected and included in deep learning (DL) algorithms. Specific DL-models were built based on raw data to categorize diagnostic group, disease complexity, and New York Heart Association (NYHA) class. In addition, models were developed to estimate need for discussion at multidisciplinary team (MDT) meetings and to gauge prognosis of individual patients. Overall, the DL-algorithms-based on over 44 000 medical records-categorized diagnosis, disease complexity, and NYHA class with an accuracy of 91.1%, 97.0%, and 90.6%, respectively in the test sample. Similarly, patient presentation at MDT-meetings was predicted with a test sample accuracy of 90.2%. During a median follow-up time of 8 years, 785 patients died. The automatically derived disease severity-score derived from clinical information was related to survival on Cox analysis independently of demographic, exercise, laboratory, and ECG parameters. Conclusion: We present herewith the utility of machine learning algorithms trained on large datasets to estimate prognosis and potentially to guide therapy in ACHD. Due to the largely automated process involved, these DL-algorithms can easily be scaled to multi-institutional datasets to further improve accuracy and ultimately serve as online based decision-making tools.
Montanaro C, Merola A, Kempny A, et al., 2019, The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 280, Pages: 61-66, ISSN: 0167-5273
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- Citations: 4
Krupickova S, Li W, Gatzoulis MA, et al., 2019, Ramipril for left ventricular diastolic function in patients with pulmonary regurgitation after repair of tetralogy of Fallot, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 278, Pages: 93-93, ISSN: 0167-5273
van Doren S, Brida M, Gatzoulis MA, et al., 2019, Sex differences in publication volume and quality in congenital heart disease: are women disadvantaged?, OPEN HEART, Vol: 6, ISSN: 2053-3624
Krupickova S, Li W, Cheang MH, et al., 2018, Ramipril and left ventricular diastolic function in stable patients with pulmonary regurgitation after repair of tetralogy of Fallot, Scientific Sessions of the American-Heart-Association / Resuscitation Science Symposium, Publisher: ELSEVIER IRELAND LTD, Pages: 64-69, ISSN: 0167-5273
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- Citations: 6
Li W, West C, McGhie J, et al., 2018, Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD), International Journal of Cardiology, Vol: 272, Pages: 77-83, ISSN: 0167-5273
The population of adults with congenital heart disease (ACHD) is increasing constantly due to medical, surgical and interventional successes and the input from advanced cardiovascular imaging. ACHD patients are at continuing risk of residua and sequelae related to their CHD contributing to significant morbidity and mortality. Consequently, lifelong expert surveillance is recommended for most patients. Healthcare providers are still working out how best to achieve this objective, how to train enough experts to provide high quality care, and how to organize the delivery of care. Echocardiography is crucial to clinical surveillance providing a comprehensive assessment of cardiac morphology, physiology, pathophysiology, and function. Thus it contributes significantly to the overall clinical management of ACHD patients. The International Society for Adult Congenital Heart Disease (ISACHD; www.isachd.org) is the leading organization of professionals worldwide dedicated to the pursuit of excellence in the care of ACHD patients. Recognizing the critical role of imaging in the diagnosis and management of ACHD, ISACHD established a task force to provide guidance on echocardiographic studies and reporting. The rationale is that standardization of echocardiographic imaging and reporting carries the potential to improve the overall quality of these exams around the world and facilitate collaborative multicenter research. The standardized ACHD protocols provided by the ISACHD task force (found in the appendices) include specific recommendations for data acquisition and reporting for each of the major adult congenital heart lesions. These protocols give a comprehensive and structured approach in the evaluation of ACHD patients and help to ensure excellent patient care.
Geva T, Mulder B, Gavreau K, et al., 2018, Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in patients with repaired tetralogy of fallot enrolled in the INDICATOR Cohort, Circulation, Vol: 138, Pages: 2106-2115, ISSN: 0009-7322
Background -Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot (rTOF) before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia (VT) and death have not been identified. Methods -Patients with rTOF enrolled in the INDICATOR cohort-a 4-center international cohort study- who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Pre-procedural clinical, electrocardiogram, cardiovascular magnetic resonance (CMR), and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR CMR until the primary outcome-death, aborted sudden cardiac death, or sustained VT. Results -Of the 452 eligible patients (median age at PVR 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained VT) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular (RV) ejection fraction < 40% (hazard ratio [HR] 2.39; 95% confidence interval [CI] 1.18 to 4.85; P = 0.02), RV mass-to-volume ratio ≥ 0.45 g/mL (HR 4.08; 95%, CI 1.57 to 10.6; P = 0.004), and age at PVR ≥ 28 years (HR 3.10; 95% CI 1.42 to 6.78; P = 0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted RV systolic pressure ≥40 mm Hg was associated with the primary outcome (HR 3.42; 95% CI 1.09 to 10.7; P = 0.04). Preoperative predictors of a composite secondary outcome-postoperative arrhythmias and heart failure-included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index. Conclusions -In this observational investigation of patients with rTOF, an older age at PVR and pre-PVR RV hypertrophy and dysfunction were predictive of shorter time to postoperative death and sustained VT. These find
Di Salvo G, Miller O, Babu Narayan S, et al., 2018, Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI, EHJ Cardiovascular Imaging / European Heart Journal - Cardiovascular Imaging, Vol: 19, Pages: 1077-1098, ISSN: 2047-2412
Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.
Nashat H, Montanaro C, Li W, et al., 2018, Atrial septal defects and pulmonary arterial hypertension, JOURNAL OF THORACIC DISEASE, Vol: 10, Pages: S2953-S2965, ISSN: 2072-1439
Fraisse A, di Salvo G, Janssen JC, et al., 2018, Patent foramen ovale after cryptogenic stroke: when is it justifiable to close it?, International Journal of Cardiology, Vol: 266, Pages: 81-82, ISSN: 0167-5273
Cazzoli I, Guarguagli S, Dimopoulos K, et al., 2018, Post-incisional arrhythmias in patients with transposition of the great arteries (TGA) after atrial switch procedure: always pulmonary venous atrium (PVA) reentry?, European-Society-of-Cardiology Congress, Publisher: OXFORD UNIV PRESS, Pages: 1229-1230, ISSN: 0195-668X
Ghonim S, Gatehouse PD, Gatzoulis MA, et al., 2018, Is cardiovascular magnetic resonance measurement of diffuse fibrosis ready for clinical use in the systemic RV?, International Journal of Cardiology, Vol: 271, Pages: 66-67, ISSN: 0167-5273
Boutsikou M, Shore D, Li W, et al., 2018, Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: varied clinical presentation, therapeutic approach and outcome, International Journal of Cardiology, Vol: 261, Pages: 49-53, ISSN: 0167-5273
IntroductionThe diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death.CasesWe present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome.ConclusionALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy.
Heng EL, Diller G-P, Gatzoulis MA, et al., 2018, Response by Heng et al to letter regarding article, "Immediate and midterm cardiac remodeling after surgical pulmonary valve replacement in adults with repaired tetralogy of fallot: a prospective cardiovascular magnetic resonance and clinical study"., Circulation, Vol: 137, Pages: 2186-2187, ISSN: 0009-7322
Cordina R, Ministeri M, Babu-Narayan SV, et al., 2018, Evaluation of the relationship between ventricular end-diastolic pressure and echocardiographic measures of diastolic function in adults with a Fontan circulation, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 259, Pages: 71-75, ISSN: 0167-5273
BackgroundEchocardiographic assessment of diastolic function in the setting of Fontan physiology is not well validated. We recently demonstrated that atrioventricular systolic to diastolic duration ratio (AVV S/D ratio) independently predicts mortality in Fontan-adults and that a value >1.1 was associated with poor prognosis.PurposeTo correlate echocardiographic measures of diastolic function with direct measurement of ventricular end-diastolic pressure (VEDP).MethodsA retrospective analysis was undertaken of Fontan-adults who had transthoracic echocardiography (TTE) within 12 months of direct measurement of VEDP during cardiac catheterisation.ResultsFifteen Fontan adults (3 males, mean age 29 ± 9 years) were evaluated. Thirteen patients had dominant morphologic left ventricle and 2 had morphologic right ventricle. Four had atriopulmonary connection and 11 had total cavopulmonary connection. Twelve patients were NYHA Class I/II and 3 were Class III. Time between TTE and cardiac catheter was 46 ± 113 days; VEDP was 8 ± 5 mmHg. Ten patients had preserved ventricular function, 3 had mild and 2 had moderate systolic impairment by subjective TTE assessment. AVV S/D ratio had the strongest correlation with VEDP (r = 0.8, p = 0.001). AVV S/D ratio ≥ 1.1 had 100% positive predictive value and 92% negative predictive value for detecting VEDP >10 mmHg. The only conventional echocardiographic measure of diastolic function that correlated with VEDP was pulmonary vein A wave - atrioventricular A wave duration difference (r = 0.8, p = 0.02).ConclusionsTTE measures reflect VEDP in adults with a Fontan circulation. AVV S/D ratio is a simple parameter yet to enter standard practice that can be used to identify elevated VEDP.
Ghonim S, Jenkins S, Smith GC, et 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.
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