Publications
515 results found
Bredy C, Ministeri M, Kempny A, et al., 2017, NYHA classification in adults with congenital heart disease: Relation to objective measures of exercise and outcome., European Heart Journal - Quality of Care and Clinical Outcomes, Vol: 4, Pages: 51-58, ISSN: 2058-5225
AimsThe New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.Methods and resultsThis study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of ‘asymptomatic’ (NYHA class 1) patients did not achieve a ‘normal’ peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26–14.35, P < 0.0001).ConclusionDespite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.
Constantine AH, Kempny A, Swan L, et al., 2017, Pregnancy in adults with congenital heart disease in England: birth rate and delivery practices between 1997 and 2014, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 212-212, ISSN: 0195-668X
Brida M, Dimopoulos K, Kempny A, et al., 2017, Body mass index in adult congenital heart disease, HEART, Vol: 103, Pages: 1250-1257, ISSN: 1355-6037
- Author Web Link
- Cite
- Citations: 40
Brida M, Dimopoulos KD, McCabe CM, et al., 2017, Breathing reserve in adults with congenital heart disease and its relation to exercise intolerance, Publisher: OXFORD UNIV PRESS, Pages: 1351-1351, ISSN: 0195-668X
Kempny A, Dimopoulos K, Price LC, et al., 2017, Incidence of pulmonary embolism, associated mortality and bleeding risk in england between 1998-2015, Publisher: OXFORD UNIV PRESS, Pages: 865-866, ISSN: 0195-668X
Drakopoulou M, Nashat H, Kempny A, et al., 2017, Incidence of arrhythmia and relation to mortality in a contemporary cohort of adults with pulmonary arterial hypertension associated with congenital heart disease, Publisher: OXFORD UNIV PRESS, Pages: 954-954, ISSN: 0195-668X
Martin Garcia AC, Arachchillage DRJ, Kempny A, et al., 2017, Low platelet count and its relation to death in adults with eisenmenger syndrome, Publisher: OXFORD UNIV PRESS, Pages: 953-954, ISSN: 0195-668X
Martin Garcia AC, Arachchillage DRJ, Kempny A, et al., 2017, Abnormalities in platelet volume and count as predictors of thrombosis and bleeding in patients with Eisenmenger syndrome, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 146-146, ISSN: 0195-668X
Larsen SH, Dimopoulos K, Gatzoulis MA, et al., 2017, Pulmonary valve replacement in England: trends and outcome over the past two decades, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 56-56, ISSN: 0195-668X
Cazzoli I, Larsen SH, Kempny A, et al., 2017, Echocardiographic predictors of outcome in repaired Tetralogy of Fallot patients over 40 years old, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 149-149, ISSN: 0195-668X
Dimopoulos K, Alonso-Gonzalez R, Wort SJ, et al., 2017, The incidence of aortic dissection in tetralogy of Fallot, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 56-56, ISSN: 0195-668X
Martin-Garcia AC, Arachchillage DR, Kempny A, et al., 2017, Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome, Heart, Vol: 104, Pages: 45-50, ISSN: 1355-6037
OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years. RESULTS: Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×10⁹/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×10⁹/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively). CONCLUSIONS: Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.
Kempny A, Dimopoulos K, Uebing A, et al., 2017, Outcome of cardiac surgery in patients with congenital heart disease in England between 1997 and 2015, PLOS One, Vol: 12, ISSN: 1932-6203
BackgroundThe number of patients with congenital heart disease (CHD) is increasing worldwide and most of them will require cardiac surgery, once or more, during their lifetime. The total volume of cardiac surgery in CHD patients at a national level and the associated mortality and predictors of death associated with surgery are not known. We aimed to investigate the surgical volume and associated mortality in CHD patients in England.MethodsUsing a national hospital episode statistics database, we identified all CHD patients undergoing cardiac surgery in England between 1997 and 2015.ResultsWe evaluated 57,293 patients (median age 11.9years, 46.7% being adult, 56.7% female). There was a linear increase in the number of operations performed per year from 1,717 in 1997 to 5,299 performed in 2014. The most common intervention at the last surgical event was an aortic valve procedure (9,276; 16.2%), followed by repair of atrial septal defect (9,154; 16.0%), ventricular septal defect (7,746; 13.5%), tetralogy of Fallot (3,523; 6.1%) and atrioventricular septal defect (3,330; 5.8%) repair. Associated mortality remained raised up to six months following cardiac surgery. Several parameters were predictive of post-operative mortality, including age, complexity of surgery, need for emergency surgery and socioeconomic status. The relationship of age with mortality was “U”-shaped, and mortality was highest amongst youngest children and adults above 60 years of age.ConclusionsThe number of cardiac operations performed in CHD patients in England has been increasing, particularly in adults. Mortality remains raised up to 6-months after surgery and was highest amongst young children and seniors.
Kempny A, Dimopoulos K, Gatzoulis MA, 2017, Declining incidence and prevalence of Eisenmenger Syndrome in the developed world – a triumph of modern medicine, Heart, Vol: 103, Pages: 1313-1314, ISSN: 1468-201X
Hjortshoj CMS, Kempny A, Jensen AS, et al., 2017, Past and current cause-specific mortality in Eisenmenger syndrome, European Heart Journal, Vol: 38, Pages: 2060-2067, ISSN: 0195-668X
Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015.Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, ‘early’ and ‘late’, respectively. Over a median follow-up of 6.1 years (interquartile range 2.1–21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the ‘late’ relative to the ‘early’ era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the ‘late’ vs. ‘early’ era (median survival 52.3 vs. 35.2 years, P < 0.001).Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the ‘late’ era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.
Midha D, Chen Z, Jones DG, et al., 2017, Pacing in congenital heart disease - A four-decade experience in a single tertiary centre, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 241, Pages: 177-181, ISSN: 0167-5273
Nashat H, Kempny A, McCabe C, et al., 2017, Eisenmenger syndrome: current perspectives, Research Reports in Clinical Cardiology, Vol: 8, Pages: 1-12, ISSN: 1179-8475
Abstract: Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and co
Dimopoulos K, Alonso-Gonzalez R, D’Alto M, 2017, Heart Failure, Exercise Intolerance, and Physical Training, Diagnosis and Management of Adult Congenital Heart Disease, Pages: 77-87, ISBN: 9780702069321
Bax S, Bredy C, Kempny A, et al., 2017, A Composite Echocardiography Score Predicts Pulmonary Hypertension In Patients With Interstitial Lung Disease, International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Sithamparanathan S, Nair A, Thirugnanasothy L, et al., 2016, Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry, JOURNAL OF HEART AND LUNG TRANSPLANTATION, Vol: 36, Pages: 770-779, ISSN: 1053-2498
Moceri P, Bouvier P, Baudouy D, et al., 2016, Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain., Eur Heart J Cardiovasc Imaging
AIMS: Survival in pulmonary arterial hypertension (PAH) and Eisenmenger syndrome (ES) relates to right ventricular (RV) function. Little is known about differences of ventricular function between ES patients and those suffering from other PAH aetiologies. In this study, we compared global ventricular function assessed by speckle-tracking in adult patients with ES, other PAH aetiologies, or healthy controls; and assessed the relationship between ventricular function and survival. METHODS AND RESULTS: We performed a prospective cohort study recruiting 83 adult PAH patients (43 ES and 40 other PAH aetiologies patients) and 37 controls between March 2011 and June 2015. Patients with complex congenital heart disease were excluded. Fifty-three patients (63.9%) were in NYHA functional class ≥III at baseline and 60 (72.3%) were on advanced therapies. Mean RV peak longitudinal strain was -16.3 ± 7% in ES, lower compared with healthy controls (P < 0.001) but similar to other PAH aetiologies (P = 0.6). Mean RV peak transverse strain was +26.1 ± 17% in ES, lower than in controls (P < 0.001) but higher than in other PAH aetiologies (P < 0.001). No difference was observed between ES and other PAH in LV circumferential and longitudinal strain. Over a median follow-up of 22.6 months (3.3-32.2), 22 (26.5%) patients died all from cardio-pulmonary causes. ES and RV peak transverse strain were independent predictors of survival. RV peak transverse strain ≤22% identified patients with a 14-fold increased risk of death. CONCLUSION: Right ventricular remodelling differs between adults with ES and other PAH aetiologies. ES and increased RV free wall transverse strain are associated with better survival.
Kempny A, Hjortshøj CS, Gu H, et al., 2016, Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicentre study, Circulation, Vol: 135, Pages: 1432-1440, ISSN: 0009-7322
BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters. RESULTS: -We studied 1098 patients (median age 34.4years, range 16.1-84.4years, 65.1% female, 31.9% with Down syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis only age (HR 1.41/10years, 95%CI 1.24-1.59, P<0.001), pre-tricuspid shunt (HR 1.56, 95%CI 1.02-2.39, P=0.041), oxygen saturation at rest (HR 0.53/10%, 95%CI 0.43-0.65, P<0.001), presence of sinus rhythm (HR 0.53, 95%CI 0.32-0.88, P=0.013) and presence of pericardial effusion (HR 2.41, 95%CI 1.59-3.66, P<0.001) remained significant predictors of death. CONCLUSIONS: -There is significant premature mortality amongst contemporary adults with ES. We report, herewith a multivariable mortality risk stratification model based on five simple, non-invasive predictors of death in this population.
Garfield BE, Shao D, Parfitt L, et al., 2016, LOW SKELETAL MUSCLE STRENGTH AND PHYSICAL ACTIVITY ARE ASSOCIATED WITH POOR OUTCOMES IN PULMONARY ARTERIAL HYPERTENSION, THORAX, Vol: 71, Pages: A64-A64, ISSN: 0040-6376
Dimopoulos K, Muthiah K, Alonso-Gonzalez R, et al., 2016, Heart or heart and lung transplantation for patients with congenital heart disease in England: outcomes and future predictions, Quality of Care and Outcomes Research Scientific Sessions, Publisher: American Heart Association, ISSN: 0009-7322
Bax S, Dimopoulos K, Kier G, et al., 2016, Longitudinal change in non-invasive markers in interstitial lung disease associated pulmonary hypertension and correlation with invasive pulmonary haemodynamics, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Tyebally S, Sturdy A, Khokhar A, et al., 2016, Intravenous prostacyclin for pulmonary hypertension: Patient's perspective on complications, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Vescovo GM, Kempny A, Alonso-Gonzalez R, et al., 2016, Does the ECG still carry prognostic power in contemporary patients with repaired tetralogy of Fallot?, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 763-763, ISSN: 0195-668X
Wustmann K, Davies JE, Borgia F, et al., 2016, Pulmonary wave intensity analysis parameters correlate with ventriculo-arterial coupling in patients with or without pulmonary hypertension, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 1150-1151, ISSN: 0195-668X
Kempny A, Diller GP, Gatzoulis MA, et al., 2016, Mortality after surgery in patients with congenital heart disease. analysis of 57293 cardiac surgeries in England between 1997 and 2015, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 201-201, ISSN: 0195-668X
Nashat H, Harries C, Parfitt L, et al., 2016, Factors prohibiting progression to prostanoid therapy in patients with pulmonary arterial hypertension and CTEPH who remain in functional class III or above despite maximal oral therapy, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 565-565, ISSN: 0195-668X
This data is extracted from the Web of Science and reproduced under a licence from Thomson Reuters. You may not copy or re-distribute this data in whole or in part without the written consent of the Science business of Thomson Reuters.