Publications
515 results found
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, 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, Vol: 206, Pages: 758-766, 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.
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
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
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.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al., 2022, Eisenmenger Syndrome <i>JACC</i> State-of-the-Art Review, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 79, Pages: 1183-1198, ISSN: 0735-1097
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- Citations: 11
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.
Barradas-Pires A, Constantine A, Dimopoulos K, 2022, Percutaneous Interventions in Adult Congenital Heart Disease, Patient Reported Outcomes and Quality of Life in Cardiovascular Interventions, Pages: 171-184, ISBN: 9783031098147
Congenital Heart Disease (CHD) is a growing field in cardiology, but strong scientific evidence is lacking to help guide the recommendations of care. Patient reported outcomes, or PROMs, focused on quality of life in CHD are a helpful tool to assess the quality of care provided and model the research to focus on patients’ priorities. This review focuses on quality of life in CHD after percutaneous procedures, such as percutaneous pulmonary valve implantation, atrial septal defect closure and patent foramen ovale closure. Despite heterogeneity and methodological differences between the studies reviewed, evidence suggests that quality of life after such procedures is generally good, and overall comparable to the general population. This is true across different age groups, making it particularly relevant in the decision-making process on intervention in older patients.
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, 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, Annual Scientific Sessions of the American-Heart-Association / Resuscitation Science Symposium, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
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, Annual Scientific Sessions of the American-Heart-Association / Resuscitation Science Symposium, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
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
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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- Citations: 9
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
Samaranayake CB, Warren C, Siewers K, et al., 2021, Impact of cyanosis on ventilatory responses during stair climb exercise in Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 341, Pages: 84-87, ISSN: 0167-5273
Constantine A, Dimopoulos K, Muthurangu V, et al., 2021, Twenty-year experience and outcomes in a national paediatric pulmonary hypertension service, European Society of Cardiology congress 2021, Publisher: OXFORD UNIV PRESS, Pages: 1972-1972, ISSN: 0195-668X
Constantine A, Dimopoulos K, 2021, Integrating patient-reported outcome measures in congenital heart disease care, European Heart Journal - Quality of Care and Clinical Outcomes, Vol: 7, Pages: 325-326, ISSN: 2058-5225
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
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
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
Gillesen MG, Fedchenko MF, Giang WGK, et al., 2021, Chronic kidney disease in patients with congenital heart disease - a nationwide cohort study, Publisher: OXFORD UNIV PRESS, Pages: 1846-1846, ISSN: 0195-668X
D'Alto M, Chessa M, Santoro G, et al., 2021, The adding value of fluid challenge and balloon occlusion tests in patients with atrial septal defect, European-Society-of-Cardiology (ESC) Congress / Session on New Insights into Cardiology, Publisher: OXFORD UNIV PRESS, Pages: 1840-1840, ISSN: 0195-668X
Wustmann K, Constantine A, Davies JE, et al., 2021, Prognostic implications of pulmonary wave reflection and reservoir pressure in patients with pulmonary hypertension, International Journal of Cardiology: Congenital Heart Disease, Vol: 5, Pages: 1-8, ISSN: 2666-6685
BackgroundRight ventricular (RV) coupling to the pulmonary circulation influences the response of the RV to the increased afterload caused by pulmonary hypertension (PH), which ultimately determines prognosis. A methodology that accounts for pulsatile flow is required when assessing ventriculo-arterial coupling. We applied wave intensity analysis (WIA) methods to assess the compliance of the main pulmonary artery (PA) in patients with or without PH and compared this to PA distensibility, RV function and clinical outcomes.MethodsHigh-fidelity blood pressure and Doppler flow velocity tracings were obtained simultaneously during cardiac catheterisation for suspected PH. RV volumes, main PA distensibility and ventriculo-arterial coupling (Emax/Ea) were analysed using cardiovascular magnetic resonance.ResultsThe study included 17 PH patients and 6 controls. Wave speed, reservoir and excess pressure were higher in PH patients compared to controls (p < 0.01 for all). Waveforms relating to RV ejection, microvascular wave reflection and late systolic proximal deceleration were higher in PH patients compared to controls (p < 0.01 for all) and related to echocardiographic findings, including PA Doppler notching and shortened acceleration time. Wave speed, reservoir pressure and excess pressure were strongly correlated to main PA distensibility, RV function and Emax/Ea. A higher total pressure integral was associated with an increased risk of death (all-cause mortality).ConclusionThe reservoir-excess pressure model, in combination with conventional clinical imaging, provides valuable information on the pathophysiology of PH that standard haemodynamic parameters do not. Future studies should further investigate the prognostic implications of WIA in PH, and its potential role in clinical practice.
Jansen K, Constantine A, Condliffe R, et al., 2021, Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation, Expert Review of Cardiovascular Therapy, Vol: 19, Pages: 837-855, ISSN: 1477-9072
Introduction:Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease. Areas covered:In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD. The indications, timing and the content of advanced heart failure assessment and transplant listing are discussed, along with a review of the types of transplant and other forms of available circulatory support in this group of patients. Finally, the integral role of advance care planning and palliative care is discussed. Expert opinion/commentary:All patients with PAH-CHD should be followed up in expert centers, where they can receive appropriate risk assessment, PAH therapy and supportive care. Referral for transplant assessment should be considered if there continue to be clinical high-risk features, persistent symptoms or acute heart failure decompensation despite appropriate PAH specific therapy. Expert management of PAH-CHD patients, therefore, requires vigilance for these features, along with a close relationship with local advanced heart failure services and a working knowledge of listing criteria, which may disadvantage congenital heart disease patients.
Samaranayake C, Luo Y, Siewers K, et al., 2021, Impact of cyanosis on ventilatory kinetics during stairclimbing in pulmonary arterial hypertension, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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