Publications
494 results found
Dawes TJW, McCabe C, Dimopoulos K, et al., 2023, Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study, Respirology, Vol: 28, Pages: 262-272, ISSN: 1323-7799
Background and ObjectivePulmonary hypertension is a life-limiting complication of interstitial lung disease (ILD-PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD-PH was associated with improved survival.MethodsConsecutive incident patients with ILD-PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow-up and survival data modelled by Bayesian methods.ResultsThe diagnoses in 128 patients were idiopathic pulmonary fibrosis (n = 74, 58%), hypersensitivity pneumonitis (n = 17, 13%), non-specific interstitial pneumonia (n = 12, 9%), undifferentiated ILD (n = 8, 6%) and other lung diseases (n = 17, 13%). Final outcomes were death (n = 106, 83%), transplantation (n = 9, 7%) and censoring (n = 13, 10%). Patients treated with PDE5i (n = 50, 39%) had higher mean pulmonary artery pressure (median 38 mm Hg [interquartile range, IQR: 34, 43] vs. 35 mm Hg [IQR: 31, 38], p = 0.07) and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], p=0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18 years [95% CI: 1.43, 3.04] vs. 0.94 years [0.69, 1.51], p = 0.003) independent of all other prognostic markers by Bayesian joint-modelling (HR 0.39, 95% CI: 0.23, 0.59, p < 0.001) and propensity-matched analyses (HR 0.38, 95% CI: 0.22, 0.58, p < 0.001). Survival difference with treatment was significantly larger if right ventricular function was normal, rather than abnormal, at presentation (+2.55 years, 95% CI: −0.03, +3.97 vs. +0.98 years, 95% CI: +0.47, +2.00, p = 0.04).ConclusionPDE5i treatment in ILD-PH should be investigated by a prospective randomized trial.
Dimopoulos K, Bouchard M, Cheryl Chong Z, et al., 2023, Transition to adult care of young people with congenital heart disease: Impact of a service on knowledge and self-care skills, and correlates of a successful transition, European Heart Journal - Quality of Care and Clinical Outcomes, Pages: 1-7, ISSN: 2058-5225
AimsLess than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition.Methods and resultsOverall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P < 0.0001; describing: 14.4–42.7%, P < 0.0001; understanding: 26.1–60.7%, P < 0.0001), and from the second to the third (naming: 67.4%, P = 0.004, describing: 61.4%, P < 0.001, understanding: 71.1%, P = 0.02;). Patients with LD did not improve their disease knowledge over time (all P > 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition.ConclusionA transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.
Constantine A, Dimopoulos K, Heng EL, et al., 2023, Transcatheter pulmonary valve implantation: An option for the few becoming an option for the many, International Journal of Cardiology, Vol: 373, Pages: 44-46, ISSN: 0167-5273
Dimopoulos K, Constantine A, Clift P, et al., 2023, Cardiovascular complications of Down syndrome: Scoping review and expert consensus, Circulation, Vol: 147, Pages: 425-441, ISSN: 0009-7322
Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension, co-existent pulmonary, endocrine, and metabolic diseases, and risk factors for atherosclerotic disease. Moreover, disparities in the cardiovascular care of people with Down syndrome compared with the general population, which vary across different geographies and healthcare systems, further contribute to cardiovascular mortality; this issue is often overlooked by the wider medical community. This review focuses on the diagnosis, prevalence and management of cardiovascular disease encountered in people with Down syndrome. Specifically, this article seeks to summarize available evidence in 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to disparities in care in areas of differing resource availability. All specialists and non-specialist clinicians providing care for people with Down syndrome should be aware of best clinical practice in all aspects of care of this distinct population.
Mavromanoli AC, Barco S, Ageno W, et al., 2022, Recovery of right ventricular function after intermediate-risk pulmonary embolism: results from the multicentre Pulmonary Embolism International Trial (PEITHO)-2, CLINICAL RESEARCH IN CARDIOLOGY, ISSN: 1861-0684
Zhang C, Dimopoulos K, Li Q, et al., 2022, Long-term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension, PULMONARY CIRCULATION, Vol: 12, ISSN: 2045-8932
Constantine A, Condliffe R, Ciift P, et al., 2022, Macitentan for pulmonary arterial hypertension related to repaired congenital heart disease: real-world UK experience, Publisher: OXFORD UNIV PRESS, Pages: 1928-1928, ISSN: 0195-668X
Ricci P, Bouchard M, Zhiya CC, et al., 2022, Efficacy of congenital heart disease transition clinics in improving patient education, Publisher: OXFORD UNIV PRESS, Pages: 1853-1853, ISSN: 0195-668X
Constantine A, Dimopoulos K, Condliffe R, et al., 2022, Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort, Publisher: OXFORD UNIV PRESS, Pages: 1874-1874, ISSN: 0195-668X
Krishnathasan K, Dimopoulos K, Duncan N, et al., 2022, Renal dysfunction: a predictor of adverse outcomes in ACHD patients with acute decompensated heart failure, Publisher: OXFORD UNIV PRESS, Pages: 1819-1819, ISSN: 0195-668X
Samaranayake C, Niglas M, Kempny A, et al., 2022, Acute beat blockade improves right ventricular diastolic filling in pulmonary arterial hypertension: a rodent CMR and clinical human pressure-volume study, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Constantine A, Rhodes CJ, Ricci P, et al., 2022, Correlation between right ventricular dysfunction and plasma protein profile in pulmonary hypertension, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Samaranayake C, Kempny A, Price LC, et al., 2022, Metabolic modulation of the right ventricle and pulmonary circulation in pulmonary arterial hypertension: an interventional study using a Glucagon-like-peptide-1 (GLP-1) agonist, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Samaranayake C, Mcniven R, Kempny A, et al., 2022, Ventilatory limitation during cardiopulmonary exercise testing predicts survival in patients with pulmonary arterial hypertension with Eisenmenger physiology, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Gillesén M, Fedchenko M, Giang KW, et al., 2022, Chronic kidney disease in patients with congenital heart disease: a nationwide, register-based cohort study., Eur Heart J Open, Vol: 2
AIMS: To investigate the risk of chronic kidney disease (CKD) in young patients with congenital heart disease (CHD) (age 0-47 years) compared with age- and sex-matched controls without CHD. METHODS AND RESULTS: Using data from the Swedish National Patient Register and the Cause of Death Register, 71,936 patients with CHD (50.2% male) born between 1970 and 2017 were identified. Each patient with CHD was matched by sex and age to 10 controls without CHD (n = 714,457). Follow-up data were collected for patients with CHD and controls until 2017. During a median follow-up of 13.5 (5.8; 25.5) years, 379 (0.5%) patients with CHD and 679 (0.1%) controls developed CKD. The risk of CKD was 6.4 times higher in patients with CHD than controls [95% confidence interval (CI): 5.65-7.27] and was highest in patients with severe non-conotruncal defects [hazard ratio (HR): 11.31; 95% CI: 7.37-17.36]. Compared with matched controls, the absolute and relative risks of CKD were greater for CHD patients born between 1997 and 2017 (HR: 9.98; 95% CI: 8.05-13.37) (incidence 39.5 per 100 000 person-years). The risk of CKD remained significantly higher after adjusting for hypertension, acute kidney injury, and diabetes mellitus (HR: 4.37; 95% CI: 3.83-5.00). CONCLUSION: Although the absolute risk of CKD in young patients with CHD is relatively low, patients with CHD are six times more likely to develop CKD than non-CHD controls up to the age of 47 years. Further data are needed to inform guidelines on the prevention and follow-up of CKD in CHD patients.
Diller GP, Vidal MLB, Kempny A, et al., 2022, A framework of deep learning networks provides expert-level accuracy for the detection and prognostication of pulmonary arterial hypertension, EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, Vol: 23, Pages: 1447-1456, ISSN: 2047-2404
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Constantine A, Dimopoulos K, Haworth SG, et al., 2022, Twenty-year experience and outcomes in a National Pediatric Pulmonary Hypertension Service, American Journal of Respiratory and Critical Care Medicine, ISSN: 1073-449X
Rationale:Pediatric pulmonary hypertension is an important cause of childhood morbidity and mortality, but there are limited data on the range of associated diseases, contributions of different pulmonary hypertension subtypes, therapeutic strategies, and clinical outcomes in children. Objectives:To report the 20-year experience of a large UK National Pediatric Pulmonary Hypertension Service focusing on epidemiology and clinical outcomes.Methods:Consecutive patients presenting between 2001 and 2021 were included and survival analysis was performed for incident patients.Measurements and Main Results:Of 1353 patients assessed, a pulmonary hypertension diagnosis was made in 1101(81.4%) patients (51% female, median age 2.6[IQR 0.8-8.2] years). The most common form was pulmonary arterial hypertension in 48%, followed by 32.3% with pulmonary hypertension due to lung disease. Multiple contributory causes of pulmonary hypertension were common, with 16.9% displaying features of more than one diagnostic group. The annual incidence of childhood pulmonary hypertension was 3.5(95%CI 3.3-3.8)/million children, and the prevalence was 18.1(95%CI 15.8-20.4)/million. The incidence was highest for pulmonary hypertension due to lung disease in infancy (15.0[95%CI 12.7-17.2]/million/year). Overall, 82.4% patients received pulmonary arterial hypertension therapy and escalation to triple therapy during follow-up was required in 13.1%. In 970(88.1%) incident patients, transplant-free survival was 86.7%(95%CI:84.5-89%) at 1, and 68.6%(95%CI:64.7-72.6%) at 10 years. Pulmonary hypertension due to left heart disease had the lowest survival (hazard ratio 2.0, 95%CI:1.36-2.94, p<0.001).Conclusions:Clinical phenotypes of pediatric pulmonary hypertension are heterogenous and overlapping, with clinical phenotypes that evolve throughout childhood. Despite widespread use of pulmonary arterial hypertension therapy, the prognosis remains poor.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al., 2022, Eisenmenger Syndrome JACC State-of-the-Art Review, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 79, Pages: 1183-1198, ISSN: 0735-1097
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Constantine A, Rhodes CJ, Ricci P, et al., 2022, PLASMA PROTEIN PROFILE IN EISENMENGER SYNDROME AND OTHER FORMS OF PH: ASSOCIATION WITH MARKERS OF RV REMODELLING, ACC.22, Publisher: ELSEVIER SCIENCE INC, Pages: 1364-1364, ISSN: 0735-1097
Constantine A, Barradas-Pires A, Dimopoulos K, 2022, Cardiopulmonary exercise testing in congenital heart disease: Towards serial testing as part of long-term follow-up, European Journal of Preventive Cardiology, Vol: 29, Pages: 510-512, ISSN: 2047-4873
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.
Constantine A, Dimopoulos K, Jenkins P, et al., 2021, Use of pulmonary arterial hypertension therapies in Fontan patients: current practice across the United Kingdom, Journal of the American Heart Association, Vol: 11, Pages: 1-19, ISSN: 2047-9980
Background: The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in congenital heart disease (CHD) patients with single ventricle physiology. ‘Fontan failure’ is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult Fontan patients receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the UK.Methods and Results: We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist CHD centers in England and Scotland between 2009 and 2019. Patients on PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 Fontan patients followed in specialist centers, only 76 (4.9%) received PAH therapies during follow-up. The vast majority (90.8%) were treated with a phosphodiesterase-5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, history of protein losing enteropathy, or receive loop diuretics (p<0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median 12[11-15] months, functional class was more likely to improve in the treated group (p=0.01), with no other changes in clinical parameters or safety issues. Conclusions: PAH therapies are used in adult Fontan patients followed in specialist centers, targeting individuals with very advanced disease or complications. Follow-up suggests stabilization of the clinical status after 12 months of therapy.
Constantine A, Condliffe R, Clift P, et al., 2021, Cardiac disease in Down Syndrome: literature review and international expert consensus in collaboration with Down Syndrome International (DSi), Journal of Congenital Cardiology, Vol: 5, Pages: 1-3, ISSN: 2056-7251
BackgroundCongenital heart disease is common in patients with Down syndrome, yet clinical recommendations relating to its diagnosis and management in this patient group are lacking.Main bodyWe discuss the ongoing collaboration between an international panel of cardiovascular experts and expert stakeholders from Down Syndrome International, an international disabled people's organisation with membership of organisations and individuals from 136 countries worldwide. The aim of this collaboration is to describe best clinical practice, focusing on 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to the care of patients in areas of differing resource availability.ConclusionsThe planned expert consensus statement on cardiac disease in people with Down syndrome aims to foster communication between experts, direct future research and inform future practice guidelines for the diagnosis and management of cardiovascular disease in people with Down syndrome.
Vidal MLB, Kempny A, Li W, et al., 2021, Deep Learning Networks Trained on Routine Echocardiography Images Provide Expert Level Prognostication in Patients With Pulmonary Hypertension: A Study on 408 Patients From an Expert Centre, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
Vidal MLB, Kempny A, Li W, et al., 2021, Utility of a Novel Ensemble Based Deep Learning Network for the Automatic Detection of Pulmonary Hypertension and Right Ventricular Dilatation: A Study Based on Data From 783 Individuals From Two Tertiary Centres, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
Assenza GE, Dimopoulos K, Budts W, et al., 2021, Management of acute cardiovascular complications in pregnancy, EUROPEAN HEART JOURNAL, Vol: 42, Pages: 4224-4240, ISSN: 0195-668X
Assenza GE, Krieger E, Baumgartner H, et al., 2021, AHA/ACC vs ESC Guidelines for Management of Adults With Congenital Heart Disease JACC Guideline Comparison, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 78, Pages: 1904-1918, ISSN: 0735-1097
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Khan U, Shaw T, Kempny A, et al., 2021, The clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 343, ISSN: 0167-5273
Constantine A, Dimopoulos K, Condliffe R, et al., 2021, Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time, European Society of Cardiology congress 2021, Publisher: OXFORD UNIV PRESS, Pages: 1857-1857, ISSN: 0195-668X
Nashat H, Rocha L, Constantine A, et al., 2021, Cardiovascular outcomes in women with the highest classification of maternal cardiovascular risk in pregnancy, European Society of Cardiology congress 2021, Publisher: OXFORD UNIV PRESS, Pages: 2903-2903, ISSN: 0195-668X
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