383 results found
Nashat H, Kempny A, Harries C, et al., 2020, A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 299, Pages: 131-135, ISSN: 0167-5273
Yang H, Bouma BJ, Dimopoulos K, et al., 2020, Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 299, Pages: 123-130, ISSN: 0167-5273
Constantine A, Dimopoulos K, Rafiq I, et al., Sex differences in hypertrophic cardiomyopathy: time to tailor risk stratification and therapy?, European Journal of Preventive Cardiology, ISSN: 2047-4873
Tulloh R, Dimopoulos K, Condliffe R, et al., 2019, Management of Adults with Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK: Survey of Current Practice, Unmet Needs and Expert Commentary (vol 27, pg 1018, 2018), HEART LUNG AND CIRCULATION, Vol: 28, Pages: 1915-1915, ISSN: 1443-9506
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.
McCabe C, Vranesic II, Verdes MC, et al., 2019, Reply to Echocardiographic predictors of outcome in PAH, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 294, Pages: 58-58, ISSN: 0167-5273
McCabe C, Vranesic II, Verdes MC, et al., 2019, Right ventricular systolic to diastolic duration ratio: A novel predictor of outcome in adult idiopathic pulmonary arterial hypertension, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 293, Pages: 218-222, ISSN: 0167-5273
Constantine A, Tulloh R, Condliffe R, et al., 2019, Congenital heart disease, pulmonary arterial hypertension and the UK's Drivers and Vehicle Licensing Agency: controversial new guidance, Pulmonary Circulation, Vol: 9, Pages: 2045894019882627-2045894019882627, ISSN: 2045-8940
Constantine A, Dimopoulos K, 2019, Evaluating a strategy of PAH therapy pre-treatment in patients with atrial septal defects and pulmonary arterial hypertension to permit safe repair (“treat-and-repair”), International Journal of Cardiology, Vol: 291, Pages: 142-144, ISSN: 0167-5273
Cazzoli I, Gunturiz-Beltran C, Guarguagli S, et al., 2019, Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations, International Journal of Cardiology, 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.
Constantine A, Barradas-Pires A, Dimopoulos K, 2019, Modifiable risk factors in congenital heart disease: Education, transition, digital health and choice architecture, European Journal of Preventive Cardiology, Pages: 204748731987414-204748731987414, ISSN: 2047-4873
Guarguagli S, Kempny A, Cazzoli I, et al., 2019, Efficacy of catheter ablation for atrial fibrillation in patients with congenital heart disease, EUROPACE, Vol: 21, Pages: 1334-1344, ISSN: 1099-5129
D'Alto M, Constantine A, Balint OH, et al., 2019, The effects of parenteral prostacycline therapy as add-on treatment to oral compounds in Eisenmenger syndrome, European Respiratory Journal, Vol: 54, ISSN: 0903-1936
D'Alto M, Budts W, Diller GP, et al., 2019, Does gender affect the prognosis and risk of complications in patients with congenital heart disease in the modern era?, International Journal of Cardiology, Vol: 290, Pages: 156-161, ISSN: 0167-5273
Gender differences in the outcome of acquired cardiovascular disease are well known, but available literature on the influence of gender in congenital heart disease (CHD) is limited. Registries have provided valuable, albeit at times conflicting data. Higher mortality rates have been reported in older males with CHD, while sudden cardiac death is more prevalent in young males. However, mortality around surgery for CHD is higher in girls compared to boys, likely due to smaller body size. Women are at higher risk of developing pulmonary arterial hypertension, but at lower risk of adverse aortic outcomes, even though they are less likely to receive aortic surgery. Finally, women have a lower risk of presenting with infective endocarditis compared to men. The underlying reasons for gender differences in CHD can be attributed to genetic, hormonal, behavioural and other causes. The aim of the present paper is to provide an overview of available evidence on gender differences in CHD and their impact on outcome.
Martin-Garcia AC, Dimopoulos K, Boutsikou M, et al., 2019, Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement., Heart
OBJECTIVES: Cardiac surgery or catheter interventions are nowadays commonly performed to reduce volume loading of the right ventricle in adults with congenital heart disease. However, little is known, on the effect of such procedures on pre-existing tricuspid regurgitation (TR). We assessed the potential reduction in the severity of TR after atrial septal defect (ASD) closure and pulmonic valve replacement (PVR). METHODS: Demographics, clinical and echocardiographic characteristics of consecutive patients undergoing ASD closure or PVR between 2005 and 2014 at a single centre who had at least mild preoperative TR were collected and analysed. RESULTS: Overall, 162 patients (mean age at intervention 41.6±16.1 years, 38.3% male) were included: 101 after ASD closure (61 transcatheter vs 40 surgical) and 61 after PVR (3 transcatheter vs 58 surgical). Only 11.1% received concomitant tricuspid valve surgery (repair). There was significant reduction in the severity of TR in the overall population, from 38 (23.5%) patients having moderate or severe TR preoperatively to only 11 (6.8%) and 20 (12.3%) at 6 months and 12 months of follow-up, respectively (McNemar p<0.0001). There was a significant reduction in tricuspid valve annular diameter (p<0.0001), coaptation distance (p<0.0001) and systolic tenting area (p<0.0001). The reduction in TR was also observed in patients who did not have concomitant tricuspid valve (TV) repair (from 15.3% to 6.9% and 11.8% at 6 and 12 months, respectively, p<0.0001). On multivariable logistic regression including all univariable predictors of residual TR at 12 months, only RA area remained in the model (OR 1.2, 95% CI 1.04 to 1.37, p=0.01). CONCLUSIONS: ASD closure and PVR are associated with a significant reduction in tricuspid regurgitation, even among patients who do not undergo concomitant tricuspid valve surgery.
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.
Opotowsky AR, Carazo M, Singh MN, et al., 2019, Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: Results of the Boston Adult Congenital Heart Disease Biobank, AMERICAN HEART JOURNAL, Vol: 214, Pages: 142-155, ISSN: 0002-8703
Bax S, Jacob J, Ahmed R, et al., 2019, Right ventricle to left ventricle ratio at CTPA predicts mortality in interstitial lung disease, Chest, ISSN: 0012-3692
INTRODUCTION: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to ILD severity. Right ventricle to left ventricle diameter ratio (RV:LV) measured at CT pulmonary angiography (CTPA), has been shown to provide valuable information in pulmonary arterial hypertension patients and to predict death or deterioration in acute pulmonary embolism. METHODS: Demographics, ILD subtype, echocardiography and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterisation (RHC) at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored using CT criteria. RV:LV ratio at CTPA was evaluated by three different methods. Cox-proportional hazard analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. RESULTS: 92 patients were included: 64% male, mean age 65±11 years, with FVC 57±20% (predicted), TLCOc 22±8% (predicted) and KCOc 51±17% (predicted). PH was confirmed at RHC in 78%. Of all CTPA-derived measures, an RV:LV ratio ≥1.0 strongly predicted mortality or transplantation at univariate analysis (HR 3.26, 95%CI:1.49-7.13, p=0.003), whereas invasive haemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (HR: 3.19, CI:1.44-7.10, p=0.004), adjusting for an ILD diagnosis of IPF and CT derived ILD severity. CONCLUSION: An increased RV:LV ratio measured at CTPA provides a simple, non-invasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow up, more aggressive treatment and consideration of lung transplantation.
Li Q, Dimopoulos K, Liu T, et al., 2019, Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease, EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY, Vol: 26, Pages: 1067-1076, ISSN: 2047-4873
Masding A, Preston SD, Toshner M, et al., 2019, Chronic thromboembolic pulmonary hypertension following long-term peripherally inserted central venous catheter use, Pulmonary Circulation, Vol: 9, ISSN: 2045-8940
A 36-year-old woman presented with recurrent pulmonary emboli (PE) despite oral anticoagulation. She was a type I diabetic with severe gastroparesis requiring insertion of multiple long-term peripherally inserted central catheters (PICC) over a 10-year period. Imaging at presentation demonstrated a PICC-associated mobile mass in the right atrium and signs of pulmonary hypertension (PH). She was thrombolyzed and fully anticoagulated, and diabetic management without PICC strongly recommended. PH persisted, however, and she developed chronic thromboembolic pulmonary hypertension (CTEPH), for which successful pulmonary endarterectomy (PEA) surgery led to symptomatic and hemodynamic improvement. This was the first case of CTEPH reported related to long-term PICC use outside the setting of malignant disease, and a novel observation that the PEA specimen contained multiple plastic fragments. Long-term PICC placement increases the risk of CTEPH, a life-threatening, albeit treatable, complication.
Favoccia C, Constantine AH, Wort SJ, et al., Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease, Expert Review of Cardiovascular Therapy, Vol: 17, Pages: 449-459, ISSN: 1477-9072
Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers, with emphasis on co-morbidities and complications is described. Expert opinion: PAH-CHD is a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.
Larsen SH, Dimopoulos K, Gatzoulis MA, et al., 2019, Surgical and percutaneous pulmonary valve replacement in England over the past two decades, HEART, Vol: 105, Pages: 932-937, ISSN: 1355-6037
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
Kempny A, Dimopoulos K, Fraisse A, et al., 2019, Blood viscosity and its relevance to the diagnosis and management of pulmonary hypertension, Journal of the American College of Cardiology, Vol: 73, Pages: 2640-2642, ISSN: 0735-1097
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
Bhatti Y, Rice AJ, Kempny A, et al., 2019, Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing, Pulmonary Circulation, Vol: 9, Pages: 1-4, ISSN: 2045-8940
Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.
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
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.
Dimopoulos K, Muthiah K, Alonso-Gonzalez R, et al., 2019, Heart or heart-lung transplantation for patients with congenital heart disease in England, Heart, Vol: 105, Pages: 596-602, ISSN: 1355-6037
BACKGROUND: Increased longevity in patients with congenital heart disease (CHD) is associated with late complications, mainly heart failure, which may not be amenable to redo surgery and become refractory to medical therapy and so, trigger referral for transplantation. We assessed the current role and future prospects of heart and heart-lung transplantation for patients with CHD in England. METHODS: We performed a retrospective analysis of hospital episodes for England for 1997-2015, identifying patients with a CHD code (ICD-10 'Q2xx.x'), who underwent heart or heart-lung transplantation. RESULTS: In total, 469 transplants (82.2% heart and 17.8% heart-lung) were performed in 444 patients. Half of patients transplanted had mild or moderate CHD complexity, this percentage increased with time (p=0.001). While overall, more transplantations were performed over the years, the proportion of heart-lung transplants declined (p<0.0001), whereas the proportion of transplants performed in adults remained static. Mortality was high during the first year, especially after heart-lung transplantation, but remained relatively low thereafter. Older age and heart-lung transplantation were strong predictors of death. While an increase in CHD transplants is anticipated, actual numbers in England seem to lag behind the increase in CHD patients with advanced heart failure. CONCLUSIONS: The current and future predicted increase in the numbers of CHD transplants does not appear to parallel the expansion of the CHD population, especially in adults. Further investment and changes in policy should be made to enhance the number of donors and increase CHD transplant capacity to address the increasing numbers of potential CHD recipients and optimise transplantation outcomes in this growing population.
Rajpal S, Carazo M, Singh M, et al., 2019, CREATININE VERSUS CYSTATIN C TO ESTIMATE GLOMERULAR FILTRATION RATE IN ADULTS WITH CONGENITAL HEART DISEASE, 68th Annual Scientific Session and Expo of the American-College-of-Cardiology (ACC), Publisher: ELSEVIER SCIENCE INC, Pages: 565-565, ISSN: 0735-1097
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