Publications
217 results found
Roehl M, Conway M, Ghonim S, et al., 2024, STEAM-SASHA: A novel approach for blood and fat suppressed native T1 measurement in the right ventricular myocardium, Magnetic Resonance Materials in Physics, Biology and Medicine, ISSN: 0968-5243
Objective:The excellent blood and fat suppression of stimulated echo acquisition mode (STEAM) can be combined with saturation recovery single-shot acquisition (SASHA) in a novel STEAM-SASHA sequence for right ventricular (RV) native T1 mapping.Materials and methods:STEAM-SASHA splits magnetization preparation over two cardiac cycles, nulling blood signal and allowing fat signal to decay. Breath-hold T1 mapping was performed in a T1 phantom and twice in 10 volunteers using STEAM-SASHA and a modified Look-Locker sequence at peak systole at 3T. T1 was measured in 3 RV regions, the septum and left ventricle (LV).Results:In phantoms, MOLLI under-estimated while STEAM-SASHA over-estimated T1, on average by 3.0% and 7.0% respectively, although at typical 3T myocardial T1 (T1 > 1200 ms) STEAM-SASHA was more accurate. In volunteers, T1 was higher using STEAM-SASHA than MOLLI in the LV and septum (p = 0.03, p = 0.006, respectively), but lower in RV regions (p > 0.05). Inter-study, inter-observer and intra-observer coefficients of variation in all regions were < 15%. Blood suppression was excellent with STEAM-SASHA and noise floor effects were minimal.Discussion:STEAM-SASHA provides accurate and reproducible T1 in the RV with excellent blood and fat suppression. STEAM-SASHA has potential to provide new insights into pathological changes in the RV in future studies.
Barradas-Pires A, Merás P, Constantine A, et al., 2023, Repair of aortic regurgitation in young adults: sooner rather than later, Journal of the American Heart Association, Vol: 12, Pages: 1-11, ISSN: 2047-9980
AbstractObjectives: Establishing surgical criteria for aortic valve replacement (AVR) or repair in severe aortic regurgitation (AR) is challenging and evidence-based recommendations for young adults are lacking. We studied the indications for AVR in a cohort of young adults with severe AR from a tertiary centre, and the associated outcomes. The relation between pre-surgical echocardiographic parameters and post-operative left ventricular (LV) size and function, clinical events, and prosthetic valve-related complications was also investigated.Methods: Data were collected retrospectively on consecutive adult patients who underwent AVR or repair for severe AR between 2005 and 2019 in a tertiary cardiac centre. Results: One-hundred-and-seventy-two patients were included (age at surgery 29 [22-41] years, 81% male). One third were operated before meeting standard guideline indications. Normalization of LV size and function was achieved in 65% of patients. No significant change in LV ejection fraction (EF) from baseline to the latest follow-up (p=0.08) was observed. Pre-surgical LV end-systolic diameter (LVESD) was associated with lack of LV normalization post-surgery (OR per 1 cm increase 2.81, 95%CI:1.54 - 5.56, p<0.01). On ROC analysis, the cut-off for baseline LVESD that maximizes sensitivity and specificity for lack of LV normalization post-surgery was 43mm. Pre- and post-operative LV dimensions, and post-operative LVEF were predictors of clinical events during follow-up. Prosthesis-related complications were frequent (20.3%) during a follow-up of 5.6 [2.9-9.2] years. Freedom from aortic re-intervention was 98%, 96.5%, 85.4% at 1, 5 and 10 years, respectively.Conclusions: Patients with a history of cardiac surgery and/or increased baseline LVESD are less likely to achieve normalization of LV size and function following AVR. Clinicians must carefully balance the potential benefits of AVR on long-term outcomes against the risk of periprocedural complications and the
Krishnathasan K, Dimopoulos K, Duncan N, et al., 2023, Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes, European Journal of Preventive Cardiology, Vol: 30, Pages: 1335-1342, ISSN: 2047-4873
AimsPrevious 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 resultsThis 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 <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4–12) days, with a maximum dose of 120 (80–160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2–82.3%] and 43.3% (95% CI: 36–52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome.ConclusionAdult 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.
O'Connor M, Barbero U, Kramer DB, et al., 2023, Anatomic, histologic, and mechanical features of the right atrium: implications for leadless atrial pacemaker implantation, EUROPACE, Vol: 25, ISSN: 1099-5129
Chivers S, Cleary A, Knowles R, et al., 2023, COVID-19 in congenital heart disease (COaCHeD) study, OPEN HEART, Vol: 10, ISSN: 2053-3624
Bokma JP, Geva T, Sleeper LA, et al., 2023, Improved Outcomes After Pulmonary Valve Replacement in Repaired Tetralogy of Fallot, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 81, ISSN: 0735-1097
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- Citations: 5
Allen JJ, Keegan J, Mathew G, et al., 2023, Fully-modelled blood-focused variable inversion times for 3D late gadolinium-enhanced imaging, Magnetic Resonance Imaging, Vol: 98, Pages: 44-54, ISSN: 0730-725X
PurposeVariable heart rate during single-cycle inversion-recovery Late Gadolinium-Enhanced (LGE) scanning degrades image quality, which can be mitigated using Variable Inversion Times (VTIs) in real-time response to R-R interval changes. We investigate in vivo and in simulations an extension of a single-cycle VTI method previously applied in 3D LGE imaging, that now fully models the longitudinal magnetisation (fmVTI).MethodsThe VTI and fmVTI methods were used to perform 3D LGE scans for 28 3D LGE patients, with qualitative image quality scores assigned for left atrial wall clarity and total ghosting. Accompanying simulations of numerical phantom images were assessed in terms of ghosting of normal myocardium, blood, and myocardial scar.ResultsThe numerical simulations for fmVTI showed a significant decrease in blood ghosting (VTI: 410 ± 710, fmVTI: 68 ± 40, p < 0.0005) and scar ghosting (VTI: 830 ± 1300, fmVTI: 510 ± 730, p < 0.02). Despite this, there was no significant change in qualitative image quality scores, either for left atrial wall clarity (VTI: 2.0 ± 1.0, fmVTI: 1.8 ± 1.0, p > 0.1) or for total ghosting (VTI: 1.9 ± 1.0, fmVTI: 2.0 ± 1.0, p > 0.7).ConclusionsSimulations indicated reduced ghosting with the fmVTI method, due to reduced Mz variability in the blood signal. However, other sources of phase-encode ghosting and blurring appeared to dominate and obscure this finding in the patient studies available.
Xu Z, Radojevic J, Kempny A, et al., 2023, IMAGING PREDICTORS OF OUTCOME IN PATIENTS WITH TRANSPOSITION OF THE GREAT ARTERIES AND A SYSTEMIC RIGHT VENTRICLE: RESULTS OF A PROSPECTIVE FOLLOW-UP STUDY INCLUDING 1630 PATIENT-YEARS, 72nd Annual Scientific Session (ACC), Publisher: ELSEVIER SCIENCE INC, Pages: 1532-1532, ISSN: 0735-1097
Mayourian J, Sleeper L, Lee J, et al., 2023, DEVELOPMENT AND VALIDATION OF AN OUTCOME PREDICTION MODEL FOR REPAIRED TETRALOGY OF FALLOT: THE INDICATOR COHORT, 72nd Annual Scientific Session (ACC), Publisher: ELSEVIER SCIENCE INC, Pages: 1527-1527, ISSN: 0735-1097
Conway M, Vallespin SN, Ferreira P, et al., 2023, IN-VIVO DIFFUSION TENSOR CARDIOVASCULAR MAGNETIC RESONANCE DETECTS THE ARRANGEMENT AND DYNAMIC NATURE OF RIGHT VENTRICULAR MICROSTRUCTURE IN HEALTH AND DISEASE, 17th Annual Congress of the British-Society-of-Cardiovascular-Magnetic-Resonance (BSCMR), Publisher: BMJ PUBLISHING GROUP, Pages: A2-A3, ISSN: 1355-6037
Krishnathasan K, Dimopoulos K, Duncan N, et al., 2022, Renal dysfunction: a predictor of adverse outcomes in ACHD patients with acute decompensated heart failure, ESC Congress 2022, Publisher: Oxford University Press, Pages: 1819-1819, ISSN: 0195-668X
Thygesen JH, Tomlinson C, Hollings S, et al., 2022, COVID-19 trajectories among 57 million adults in England: a cohort study using electronic health records, LANCET DIGITAL HEALTH, Vol: 4, Pages: E542-E557
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- Citations: 17
Tortigue M, Nield LE, Karakachoff M, et al., 2022, Familial recurrence patterns in congenitally corrected transposition of the great arteries: an international study, Circulation: Genomic and Precision Medicine, Vol: 15, Pages: 1-11, ISSN: 2574-8300
Background:Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns.Methods:An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands.Results:Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia.Conclusions:ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.
Morrish A, Ahmad M, 2022, Machine Learning to Predict Outcomes in Repaired Tetralogy of Fallot, JACC: Cardiovascular Imaging, Vol: 15, Pages: 954-955, ISSN: 1936-878X
Ghonim S, Babu-Narayan SV, 2022, Reproducibility of CMR in Patients With Cardiac Implantable Electrical Devices REPLY, JACC-CARDIOVASCULAR IMAGING, Vol: 15, Pages: 955-956, ISSN: 1936-878X
Majeed A, Geva T, Sleeper LA, et al., 2022, Cardiac MRI predictors of good long-term outcomes in patients with repaired TOF, AMERICAN HEART JOURNAL, Vol: 245, Pages: 70-77, ISSN: 0002-8703
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- Citations: 2
Ghonim S, Gatzoulis M, Ernst S, et al., 2022, Predicting survival in repaired tetralogy of Fallot- a lesion specific and personalised approach, JACC: Cardiovascular Imaging, Vol: 15, Pages: 257-268, ISSN: 1876-7591
Objective: We sought to identifyrepaired tetralogy of Fallot (rTOF) patients at high-risk of death and malignant ventricular arrhythmia (VA). Background: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. Methods: Consecutive patients were prospectively recruited for late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) to define right and left ventricular (RV,LV) fibrosisin addition to proven risk markers. Results: The primary end-point was all-cause mortality. Of the 550 patients, (median age 32 years, 56% male), 27 died (mean follow-up 6.4 (±5.8); total 3512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction (EF) ≤47%, LVEF ≤55%, B-type natriuretic peptide (BNP) ≥127ng/L, peak exercise oxygen uptake (V02) ≤17ml/kg/min, prior sustained atrial arrhythmia and age ≥50 years. The weighted scores for each of the above independent predictors differentiated a high-risk sub-group patients with a 4.4%, annual risk of mortality (AUC 0.87,P<0.001). The secondary end-point (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tractakinetic length ≥55mm and RVsystolic pressure ≥47mm Hg identified high-risk patients with a 3.7%, annual risk ofVA (AUC 0.79,P<0.001) RVLGE was heavily weighted in both risk scores due to its strong relative prognostic value. Conclusion: We present ascore integrating multiple appropriately weighted risk factors to identify the sub-group of rTOF patients that are at highannual risk of death who may benefit from targeted therapy.
Krishnathasan K, Constantine A, Kempny A, et al., 2021, Predictors of adverse clinical outcome after hospitalisation for decompensated heart failure in ACHD patients, European Society of Cardiology congress 2021, Publisher: OXFORD UNIV PRESS, Pages: 1854-1854, ISSN: 0195-668X
Ladouceur M, Segura de la Cal T, Gaye B, et al., 2021, Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle, HEART, Vol: 107, Pages: 1384-1389, ISSN: 1355-6037
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- Citations: 7
Hatipoglu S, Almogheer B, Mahon C, et al., 2021, Clinical Significance of Partial Anomalous Pulmonary Venous Connections (Isolated and Atrial Septal Defect Associated) Determined by Cardiovascular Magnetic Resonance, CIRCULATION-CARDIOVASCULAR IMAGING, Vol: 14, ISSN: 1941-9651
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- Citations: 4
Constantine A, Costola G, Bianchi P, et al., 2021, Enhanced assessment of perioperative mortality risk in adults with congenital heart disease, Journal of the American College of Cardiology, Vol: 78, Pages: 234-242, ISSN: 0735-1097
BackgroundIn-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment.ObjectivesTo assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery.MethodsWe assessed perioperative survival in patients >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable derived PErioperative ACHd (PEACH) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. ResultsThe development cohort comprised 1782 procedures performed during the study period. Re-sternotomy was undertaken in 897(50.3%). There were 31(1.7%) in-hospital deaths. The PErioperative ACHd (PEACH) score showed excellent discrimination ability (AUC 0.88, 95%CI:0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC 0.69, 95%CI:0.6-0.78, p=0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC 0.75, 95%CI:0.72-0.77) and was well calibrated (Hosmer Lemishow 2 goodness-of-fit p=0.55). There was agreement in expected and observed perioperative mortality between cohorts.ConclusionsThe PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. Condensed abstract:The PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. In this study, we assesse
Budts W, Miller O, Babu-Narayan S, et al., 2021, Imaging the adult with simple shunt lesions: position paper from the EACVI and the ESC WG on ACHD. Endorsed by AEPC (Association for European Paediatric and Congenital Cardiology), EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, Vol: 22, Pages: E58-E70, ISSN: 2047-2404
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- Citations: 5
Babu-Narayan S, Ray S, 2021, Women in cardiology: no progress in the pace of change, HEART, Vol: 107, Pages: 860-861, ISSN: 1355-6037
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- Citations: 2
Baumgartner H, De Backer J, Babu-Narayan S, et al., 2021, <i>Respaldado por: Association for European Paediatric and Congenital Cardiology</i> (AEPC), <i>International Society for Adult Congenital Heart Disease</i> (ISACHD), REVISTA ESPANOLA DE CARDIOLOGIA, Vol: 74, ISSN: 1885-5857
Baumgartner H, De Backer J, Babu-Narayan S, et al., 2021, 2020 ESC Guidelines for the management of adult congenital heart disease The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD), EUROPEAN HEART JOURNAL, Vol: 42, Pages: 563-645, ISSN: 0195-668X
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- Citations: 255
Surkova E, Segura T, Dimopoulos K, et al., 2021, Systolic dysfunction of the subpulmonary left ventricle is associated with the severity of heart failure in patients with a systemic right ventricle, International Journal of Cardiology, Vol: 324, Pages: 66-71, ISSN: 0167-5273
BACKGROUND: The study aimed to assess the relation between echocardiographic parameters of subpulmonary left ventricular (LV) size and function, and the severity of heart failure in patients with a systemic right ventricle (SRV). METHODS AND RESULTS: A total of 157 patients (89 post Mustard/Senning operations, 68 with congenitally corrected transposition of great arteries [ccTGA]) were included. The size and function of the SRV and subpulmonary LV were assessed on the most recent echocardiographic exam. Clinical data were collected from the electronic records. The majority (133, 84.7%) were in NYHA functional class 1-2. Median BNP concentration was 79.5[38.3-173.3] ng/l, and 100 (63.7%) patients were receiving heart failure therapy. Both LV and SRV fractional area change (FAC) differed significantly between patients with NYHA class 1-2 vs 3-4 (48[41.5-52.8]% vs 34[28.6-38.6]%, p < 0.0001 and 29.5[23-35]% vs 22[20-27]%, p < 0.0001, respectively), but LV FAC had a higher discriminative power for functional class >2 than SRV FAC (AUC 0.90, p < 0.0001 vs 0.79; p < 0.0001, respectively). A LV FAC cut-off value <39.2% had the highest accuracy in identifying patients with NYHA class 3-4 (sensitivity 83% and specificity 88%). In multivariable logistic regression analysis, LV FAC and SRV FAC independently associated to NYHA class 3-4 (OR 0.80 [95%CI 0.72-0.88], p < 0.0001 and OR 0.85 [95%CI 0.76-0.96], p = 0.007, respectively). CONCLUSIONS: Subpulmonary LV systolic dysfunction is associated with NYHA functional class 3-4 in patients with ccTGA or after Mustard or Senning operation. Careful evaluation of the subpulmonary LV should be a part of the routine assessment of patients with a SRV.
Babu-Narayan SV, Budts W, Chessa M, et al., 2021, Ghidul esc 2020 pentru managementul bolilor cardiovasculare congenitale la adult, Revista Romana de Cardiologie, Vol: 31, Pages: 395-493, ISSN: 1220-658X
Crossland DS, Ferguson R, Magee A, et al., 2021, Consultant staffing in UK congenital cardiac services: a 10-year survey of leavers and joiners, OPEN HEART, Vol: 8, ISSN: 2053-3624
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- Citations: 4
Liu Y, Chen S, Zuhlke L, et al., 2020, Global prevalence of congenital heart disease in school-age children: a meta-analysis and systematic review, BMC CARDIOVASCULAR DISORDERS, Vol: 20, ISSN: 1471-2261
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- Citations: 15
Majeed A, Geva T, Graf J, et al., 2020, Cardiac MRI Predictors of Good Long-term Outcomes in Patients With Repaired TOF, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
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