Publications
516 results found
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.
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
Ntelios D, Giannakoulas G, Dimopoulos K, 2019, Strength training in congenital heart disease: A way to boost respiratory function?, EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY, Vol: 26, Pages: 489-491, ISSN: 2047-4873
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- Citations: 1
Kempny A, McCabe C, Dimopoulos K, et al., 2019, Incidence, mortality and bleeding rates associated with pulmonary embolism in England between 1997 and 2015, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 277, Pages: 229-234, ISSN: 0167-5273
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- Citations: 14
Dimopoulos K, Ernst S, McCabe C, et al., 2019, Pulmonary Artery Denervation A New, Long-Awaited Interventional Treatment for Combined Pre- and Post-Capillary Pulmonary Hypertension?, JACC-CARDIOVASCULAR INTERVENTIONS, Vol: 12, Pages: 285-288, ISSN: 1936-8798
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- Citations: 4
Kempny A, Fraisse A, Dimopoulos K, 2019, Risk stratification in congenital heart disease - A call for protocolised assessment and multicentre collaboration, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 276, Pages: 114-115, ISSN: 0167-5273
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- Citations: 5
Price LC, Alonso-Gonzalez R, Alexander D, et al., 2019, Critical Care Management of the Adult with Eisenmenger Syndrome and Pulmonary Arterial Hypertension Related to Congenital Heart Disease, INTENSIVE CARE OF THE ADULT WITH CONGENITAL HEART DISEASE, Editors: DaCruz, Macrae, Webb, Publisher: SPRINGER INTERNATIONAL PUBLISHING AG, Pages: 273-297, ISBN: 978-3-319-94170-7
Favoccia C, Kempny A, Yorke J, et al., 2018, EmPHasis-10 score for the assessment of quality of life in various types of pulmonary hypertension and its relation to outcome., Eur J Prev Cardiol, Pages: 2047487318819161-2047487318819161
Dimopoulos K, Condliffe R, Tulloh RMR, et al., 2018, Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease <i>JACC</i> Review Topic of the Week, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 72, Pages: 2778-2788, ISSN: 0735-1097
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- Citations: 31
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: 11
Drakopoulou M, Nashat H, Kempny A, et al., 2018, Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension, Heart, Vol: 104, Pages: 1963-1969, ISSN: 1355-6037
OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain. METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis. RESULTS: A total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001). CONCLUSIONS: Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.
Nashat H, Barbosa J, Harries C, et al., 2018, EFFICACY OF MACITENTAN IN A LARGE, REAL LIFE POPULATION OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A76-A76, ISSN: 0040-6376
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- Citations: 2
Dimopoulos K, Favoccia C, Shaughnessy L, et al., 2018, Transition to adult care in adolescents with congenital heart disease, PROGRESS IN PEDIATRIC CARDIOLOGY, Vol: 51, Pages: 62-66, ISSN: 1058-9813
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- Citations: 4
Mouratoglou SA, Dimopoulos K, Kamperidis V, et al., 2018, Right Atrial Function Predicts Clinical Outcome in Patients with Precapillary Pulmonary Hypertension, JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY, Vol: 31, Pages: 1137-1145, ISSN: 0894-7317
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- Citations: 12
Condliffe R, Clift P, Dimopoulos K, et al., 2018, Management dilemmas in pulmonary arterial hypertension associated with congenital heart disease, PULMONARY CIRCULATION, Vol: 8, ISSN: 2045-8932
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- Citations: 13
Wort SJ, Favoccia C, Kempny A, et al., 2018, emPHasis-10 score predicts mortality in patients with pulmonary hypertension, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 3
Nashat H, Da Costa R, Barbosa J, et al., 2018, Clinical efficacy of Macitentan in patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Price L, Mccabe C, Herbert S, et al., 2018, Critical care admissions in patients with pulmonary arterial hypertension., 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Blanche C, Alonso-Gonzalez R, Uribarri A, et al., 2018, Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: ELSEVIER IRELAND LTD, Pages: 79-83, ISSN: 0167-5273
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- Citations: 15
Bax S, Jacobs J, Kouranos V, et al., 2018, 10 years' experience of referral of ILD patients with Suspected PH to a National PH Service: Demographics and Outcomes, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Vranesic II, Li W, Price L, et al., 2018, Increased right ventricular systolic: diastolic duration ratiopredicts mortality in idiopathic pulmonary arterial hypertension, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 1
Nashat H, Harries C, Parfitt L, et al., 2018, A single-centre, placebo-controlled, double-blinded, randomized, cross-over study of Iloprost in patients with Eisenmenger syndrome, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 1
Price L, Kempny A, Mccabe C, et al., 2018, Sildenafil in Patients with Severe Group 3 Pulmonary Hypertension, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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
Tulloh R, Dimopoulos K, Condliffe R, et al., 2018, Management of adults with congenital heart disease and pulmonary arterial hypertension in the UK: survey of current practice, unmet needs and expert commentary, Heart, Lung and Circulation, Vol: 27, Pages: 1018-1027, ISSN: 1443-9506
BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD. The questions related to referral pathways, screening, therapy and palliative care. RESULTS: 821 surveys were distributed and 106 were returned. Respondents included a broad mix of specialist physicians with many patients along with general cardiologists managing only a small number of PAH-CHD patients. Although 97% of respondents have access to a specialist PH centre, patients are still being managed in non-specialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols. Palliative care services are limited and general cardiologists rarely perform 6-minute walk tests (6MWT) or quality of life assessments. People with PAH-CHD are often undertreated, with 39% of respondents reporting that fewer than 25% of these patients were receiving PAH-specific therapies. CONCLUSIONS: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects.
Constantine AH, Kempny A, Swan L, et al., 2018, Pregnancy in adults with congenital heart disease in England between 1997 and 2015: Clinical outcomes and risk factors for the peri-partum period, European-Society-of-Cardiology Congress 2018, Publisher: OXFORD UNIV PRESS, Pages: 1281-1281, ISSN: 0195-668X
Constantine AH, Dimopoulos K, Alonso-Gonzalez R, et al., 2018, Sub-aortic stenosis in England between 1997 and 2015: reoperation rates and risk factors, European Society of Cardiology Congress 2018, Publisher: OXFORD UNIV PRESS, Pages: 464-464, ISSN: 0195-668X
Tyebally S, Khokhar A, Zuhair M, et al., 2018, Complex decision making in the management of adult congenital heart disease, European-Society-of-Cardiology Congress, Publisher: OXFORD UNIV PRESS, Pages: 110-110, ISSN: 0195-668X
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