Publications
420 results found
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
Tutarel O, Alonso-Gonzalez R, Montanaro C, et al., 2018, Infective endocarditis in adults with congenital heart disease remains a lethal disease, Congress of the European-Society-of-Cardiology (ESC), Publisher: BMJ PUBLISHING GROUP, Pages: 161-+, ISSN: 1355-6037
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- Citations: 52
Boutsikou M, Uebing A, Kilner P, et al., 2015, Brock procedure: 52 years of effective palliation for Tetralogy of Fallot, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 199, Pages: 195-196, ISSN: 0167-5273
Wald RM, Valente AM, Gauvreau K, et al., 2015, Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair, HEART, Vol: 101, Pages: 1724-1730, ISSN: 1355-6037
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- Citations: 65
Heng EL, Gatzoulis MA, Babu-Narayan SV, 2015, Tailoring counselling after pulmonary valve surgery in repaired tetralogy of Fallot, HEART, Vol: 101, Pages: 1695-1696, ISSN: 1355-6037
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- Citations: 1
Gelson E, Curry R, Gatzoulis MA, et al., 2015, Maternal cardiac and obstetric performance in consecutive pregnancies in women with heart disease, BJOG-AN INTERNATIONAL JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Vol: 122, Pages: 1552-1559, ISSN: 1470-0328
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- Citations: 6
Babu-Narayan SV, Giannakoulas G, Valente AM, et al., 2015, Imaging of congenital heart disease in adults., European Heart Journal, Vol: 37, Pages: 1182-1195, ISSN: 1522-9645
Imaging is fundamental to the lifelong care of adult congenital heart disease (ACHD) patients. Echocardiography remains the first line imaging for inpatient, outpatient, or perioperative care. Cross-sectional imaging with cardiovascular magnetic resonance (CMR) or computed tomography (CT) provides complementary and invaluable information on cardiac and vascular anatomy and other intra-thoracic structures. Furthermore, CMR provides quantification of cardiac function and vascular flow. Cardiac catheterization is mostly reserved for assessment of pulmonary vascular resistance, ventricular end-diastolic pressure, and percutaneous interventions. There have been further advances in non-invasive imaging for ACHD including the application of advanced echocardiographic techniques, faster automated CMR imaging, and radiation dose reduction in CT. As a result ACHD, a heterogeneous population, benefit from appropriate application of multiple imaging modalities matched with tertiary ACHD expertise.
Orwat MI, Kempny A, Bauer U, et al., 2015, The importance of national and international collaboration in adult congenital heart disease: A network analysis of research output, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 195, Pages: 155-162, ISSN: 0167-5273
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- Citations: 12
Diller G-P, Kempny A, Alonso-Gonzalez R, et al., 2015, Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre, Circulation, Vol: 132, Pages: 2118-2125, ISSN: 0009-7322
Background—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.Methods and Results—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.Conclusions—ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
Diller G-P, Braeutigam A, Kempny A, et al., 2015, Depression requiring anti-depressant drug therapy in adult congenital heart disease: prevalence, risk factors, and prognostic value, European Heart Journal, Vol: 37, Pages: 771-782, ISSN: 1522-9645
Background Depression is prevalent in adults with congenital heart disease (ACHD), but limited data on the frequency of anti-depressant drug (ADD) therapy and its impact on outcome are available.Methods and results We identified all ACHD patients treated with ADDs between 2000 and 2011 at our centre. Of 6162 patients under follow-up, 204 (3.3%) patients were on ADD therapy. The majority of patients were treated with selective serotonin-reuptake inhibitors (67.4%), while only 17.0% of patients received tricyclic anti-depressants. Twice as many female patients used ADDs compared with males (4.4 vs. 2.2%, P < 0.0001). The percentage of patients on ADDs increased with disease complexity (P < 0.0001) and patient age (P < 0.0001). Over a median follow-up of 11.1 years, 507 (8.2%) patients died. After propensity score matching, ADD use was found to be significantly associated with worse outcome in male ACHD patients [hazard ratio 1.44 (95% confidence interval 1.17–1.84)]. There was no evidence that this excess mortality was directly related to ADD therapy, QT-prolongation, or malignant arrhythmias. However, males taking ADDs were also more likely to miss scheduled follow-up appointments compared with untreated counterparts, while no such difference in clinic attendance was seen in females.Conclusions The use of ADD therapy in ACHD relates to gender, age, and disease complexity. Although, twice as many female patients were on ADDs, it were their male counterparts, who were at increased mortality risk on therapy. Furthermore, males on ADDs had worse adherence to scheduled appointments suggesting the need for special medical attention and possibly psychosocial intervention for this group of patients.
Rydman R, Gatzoulis MA, Ho SY, et al., 2015, Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries, CIRCULATION-CARDIOVASCULAR IMAGING, Vol: 8, ISSN: 1941-9651
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- Citations: 38
Di Mario C, Kilic ID, Yeh JSM, et al., 2015, Exclusion of a giant aneurysm post-Kawasaki disease with novel polyurethane covered stents, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 184, Pages: 664-666, ISSN: 0167-5273
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- Citations: 7
Heng EL, Bolger AP, Kempny A, et al., 2015, Neurohormonal activation and its relation to outcomes late after repair of tetralogy of Fallot, HEART, Vol: 101, Pages: 447-454, ISSN: 1355-6037
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- Citations: 24
Kempny A, Diller G-P, Alonso-Gonzalez R, et al., 2015, Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease, Heart, Vol: 101, Pages: 699-705, ISSN: 1355-6037
Background In patients with acquired heart failure, hypoalbuminaemia is associated with increased risk of death. The prevalence of hypoproteinaemia and hypoalbuminaemia and their relation to outcome in adult patients with congenital heart disease (ACHD) remains, however, unknown.Methods Data on patients with ACHD who underwent blood testing in our centre within the last 14 years were collected. The relation between laboratory, clinical or demographic parameters at baseline and mortality was assessed using Cox proportional hazards regression analysis.Results A total of 2886 patients with ACHD were included. Mean age was 33.3 years (23.6–44.7) and 50.1% patients were men. Median plasma albumin concentration was 41.0 g/L (38.0–44.0), whereas hypoalbuminaemia (<35 g/L) was present in 13.9% of patients. The prevalence of hypoalbuminaemia was significantly higher in patients with great complexity ACHD (18.2%) compared with patients with moderate (11.3%) or simple ACHD lesions (12.1%, p<0.001). During a median follow-up of 5.7 years (3.3–9.6), 327 (11.3%) patients died. On univariable Cox regression analysis, hypoalbuminaemia was a strong predictor of outcome (HR 3.37, 95% CI 2.67 to 4.25, p<0.0001). On multivariable Cox regression, after adjusting for age, sodium and creatinine concentration, liver dysfunction, functional class and disease complexity, hypoalbuminaemia remained a significant predictor of death.Conclusions Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. Hypoalbuminaemia, therefore, should be included in risk-stratification algorithms as it may assist management decisions and timing of interventions in the growing ACHD population.
Bedair R, Babu-Narayan SV, Dimopoulos K, et al., 2015, Acceptance and psychological impact of implantable defibrillators amongst adults with congenital heart disease, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 181, Pages: 218-224, ISSN: 0167-5273
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- Citations: 22
Moceri P, Kempny A, Liodakis E, et al., 2015, Physiological differences between various types of Eisenmenger syndrome and relation to outcome, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 179, Pages: 455-460, ISSN: 0167-5273
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- Citations: 32
Gatzoulis MA, Beghetti M, Landzberg MJ, et al., 2014, Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 177, Pages: 340-347, ISSN: 0167-5273
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- Citations: 41
Simonneau G, Gatzoulis MA, Adatia I, et al., 2014, [Updated clinical classification of pulmonary hypertension]., Turk Kardiyol Dern Ars, Vol: 42 Suppl 1, Pages: 45-54, ISSN: 1016-5169
In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (YPPH) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1". Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.
Kempny A, Diller GP, Dimopoulos K, et al., 2014, Attendance of outpatient clinic appointments amongst adults with congenital heart disease: predictors and relation to outcome, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 517-518, ISSN: 0195-668X
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- Citations: 1
Kempny A, Diller GP, Alonso-Gonzalez R, et al., 2014, Hypoalbuminemia predicts outcome in adult patients with congenital heart disease, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 516-516, ISSN: 0195-668X
Diller GP, Braeutigam A, Kempny A, et al., 2014, Depressive symptoms requiring antidepressant drug therapy in adult congenital heart disease, relate to gender and disease severity and are independently associated with higher mortality, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 517-517, ISSN: 0195-668X
Radke RM, Giardini A, Buys R, et al., 2014, Predicting survival of patients with cyanotic congenital heart disease: The value of cardiopulmonary exercise testing. Results from an international multicentre study, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 517-517, ISSN: 0195-668X
Diller GP, Kempny A, Inuzuka R, et al., 2014, Poor survival prospects of treatment naive Eisenmenger patients in the current era - a systematic review and report of own experience, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 44-44, ISSN: 0195-668X
Kempny A, Dimopoulos K, Gatzoulis MA, 2014, Single-ventricle physiology in the UK: an ongoing challenge of growing numbers and of growing complexity of congenital heart disease, HEART, Vol: 100, Pages: 1315-1316, ISSN: 1355-6037
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- Citations: 5
Diller G-P, Kempny A, Inuzuka R, et al., 2014, Survival prospects of treatment naive patients with Eisenmenger: a systematic review of the literature and report of own experience, HEART, Vol: 100, Pages: 1366-1372, ISSN: 1355-6037
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- Citations: 61
Scognamiglio G, Kempny A, Price LC, et al., 2014, C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value, HEART, Vol: 100, Pages: 1335-1341, ISSN: 1355-6037
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- Citations: 21
Corte TJ, Keir GJ, Dimopoulos K, et al., 2014, Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia, American Journal of Respiratory and Critical Care Medicine, Vol: 190, Pages: 208-217, ISSN: 1073-449X
Rationale: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality.Objectives: To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group.Methods: In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20). The primary study endpoint was a fall from baseline pulmonary vascular resistance index (PVRi) of 20% or more over 16 weeks.Measurements and Main Results: Sixty patients (42 men; mean age, 66.6 ± 9.2 yr), with a mean pulmonary artery pressure of 36.0 (± 8.9) mm Hg, PVRi 13.0 (± 6.7) Wood Units/m2 and reduced cardiac index of 2.21 (± 0.5) L/min/m2 were recruited to the study. Accounting for deaths and withdrawals, paired right heart catheter data were available for analysis in 39 patients (bosentan = 25, placebo = 14). No difference in the primary outcome was detected, with seven (28.0%) patients receiving bosentan, and four (28.6%) receiving placebo achieving a reduction in PVRi of greater than or equal to 20% (P = 0.97) at 16 weeks. There was no change in functional capacity or symptoms between the two groups at 16 weeks, nor any difference in rates of serious adverse events or deaths (three deaths in each group).Conclusions: This study shows no difference in invasive pulmonary hemodynamics, functional capacity, or symptoms between the bosentan and placebo groups over 16 weeks. Our data do not support the use of the dual endothelin-1 receptor antagonist, bosentan, in patients with PH and fibrotic IIP.
Prapa S, Mccarthy KP, Krexi D, et al., 2014, The aortic root phenotype in bicuspid aortic valve disease: evidence of shared Smad2 activation in aortic regions of distinct embryologic origin, 3rd Congress of the ESC-Council-on-Basic-Cardiovascular-Science on Frontiers in Cardio Vascular Biology, Publisher: OXFORD UNIV PRESS, ISSN: 0008-6363
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- Citations: 6
Kahr PC, Alonso-Gonzalez R, Kempny A, et al., 2014, Long-term natural history and postoperative outcome of double-chambered right ventricle-Experience from two tertiary adult congenital heart centres and review of the literature, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 174, Pages: 662-668, ISSN: 0167-5273
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- Citations: 31
Giannakoulas G, Mouratoglou S-A, Gatzoulis MA, et al., 2014, Blood biomarkers and their potential role in pulmonary arterial hypertension associated with congenital heart disease. A systematic review, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 174, Pages: 618-623, ISSN: 0167-5273
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- Citations: 45
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