Publications
515 results found
Dawes T, Dimopoulos K, Mccabe C, et al., 2021, Survival effects of pulmonary vasodilators in group 3 pulmonary hypertension, European-Respiratory-Society (ERS) International Congress, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 1
Klok FA, Toenges G, Mavromanoli AC, et al., 2021, Early switch to oral anticoagulation in patients with acute intermediate-risk pulmonary embolism (PEITHO-2) : a multinational, multicentre, single-arm, phase 4 trial, LANCET HAEMATOLOGY, Vol: 8, Pages: E627-E636, ISSN: 2352-3026
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- Citations: 8
Ladouceur M, Segura de la Cal T, Gaye B, et al., 2021, Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle, HEART, Vol: 107, Pages: 1384-1389, ISSN: 1355-6037
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- Citations: 7
Barradas-Pires A, de la Cal TS, Constantine A, et al., 2021, Systemic microangiopathy in Eisenmenger syndrome – The missing link?, International Journal of Cardiology, Vol: 337, Pages: 62-63, ISSN: 0167-5273
Barradas-Pires A, Constantine A, Dimopoulos K, 2021, Safety of physical sports and exercise in ACHD, International Journal of Cardiology Congenital Heart Disease, Vol: 4, Pages: 1-10, ISSN: 2666-6685
An active lifestyle, including regular physical activity, is an important component of long-term health and wellbeing, including in patients with adult congenital heart disease (ACHD). In the past, however, safety concerns regarding ACHD patients engaging in exercise have led to overly restrictive advice from specialists. Over the past few decades, it has become clear that the vast majority of patients with ACHD can exercise safely within their own limits; moreover, exercise capacity is highly variable between patients and should be measured objectively when possible.Cardiopulmonary exercise testing (CPET) is an essential part of ACHD surveillance and provides an integrative assessment of the cardiovascular, respiratory and muscular response to exercise. It allows an objective assessment of exercise tolerance, identification of the extent and mechanisms of exercise limitations, monitoring of disease progression and forms the basis of exercise prescription.Exercise prescription in ACHD patients ideally follows evaluation in an expert center, incorporating clinical assessment and exercise testing, to identify specific risk factors for adverse events during exercise and allow individualized recommendations of safe limits for exercise. In cases where specific risks exist, the intensity of exercise should be adjusted with respect to its dynamic and static components. The exercise prescription should then be reviewed regularly and amended as risk factors emerge or are successfully treated.This review highlights the benefits of physical activity in ACHD patients and discusses the main parameters that influence the risk of certain forms of exercise in some patients. CPET and exercise prescription for non-competitive sports are also discussed.
Constantine A, Costola G, Bianchi P, et al., 2021, Enhanced assessment of perioperative mortality risk in adults with congenital heart disease, Journal of the American College of Cardiology, Vol: 78, Pages: 234-242, ISSN: 0735-1097
BackgroundIn-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment.ObjectivesTo assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery.MethodsWe assessed perioperative survival in patients >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable derived PErioperative ACHd (PEACH) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. ResultsThe development cohort comprised 1782 procedures performed during the study period. Re-sternotomy was undertaken in 897(50.3%). There were 31(1.7%) in-hospital deaths. The PErioperative ACHd (PEACH) score showed excellent discrimination ability (AUC 0.88, 95%CI:0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC 0.69, 95%CI:0.6-0.78, p=0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC 0.75, 95%CI:0.72-0.77) and was well calibrated (Hosmer Lemishow 2 goodness-of-fit p=0.55). There was agreement in expected and observed perioperative mortality between cohorts.ConclusionsThe PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. Condensed abstract:The PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. In this study, we assesse
Chessa M, Brida M, Gatzoulis MA, et al., 2021, Emergency department management of patients with adult congenital heart disease: a consensus paper from the ESC Working Group on Adult Congenital Heart Disease, the European Society for Emergency Medicine (EUSEM), the European Association for Cardio-Thoracic Surgery (EACTS), and the Association for Acute Cardiovascular Care (ACVC), EUROPEAN HEART JOURNAL, Vol: 42, Pages: 2527-2535, ISSN: 0195-668X
Xu Z, Gatzoulis MA, Dimopoulos K, et al., 2021, Better Outcomes in Pulmonary Arterial Hypertension After Repair of Congenital Heart Disease, Compared With Idiopathic Pulmonary Arterial Hypertension., CJC Open, Vol: 3, Pages: 872-879
BACKGROUND: Pulmonary vascular changes in postoperative pulmonary artery hypertension (PAH) are similar to those seen in idiopathic PAH. Data are sparse on direct comparative midterm outcomes for these 2 high-risk populations. METHODS: Patients with idiopathic or postoperative PAH referred to a large tertiary hospital between June 2005 and July 2019 were retrospectively evaluated. RESULTS: A total of 364 consecutive patients were studied, including 201 postoperative PAH patients and 163 patients with idiopathic PAH, with a median age of 18.7 (interquartile range 10.0, 31.5) and 7.3 (IQR: 2.9, 18.3) years, respectively. PAH-specific drugs were used in 77.7% of patients; 31.4% received combination therapy. Patients with idiopathic PAH had a shorter 6-mintue walk distance, lower percutaneous oxygen saturation, and higher B-type natriuretic peptide levels than those with postoperative PAH at diagnosis (all P < 0.001), During a median follow-up time of 3.4 (interquartile range: 2.1, 5.8) years, 56 patients (15.4%) died, and one underwent bilateral lung transplantation. Patients with postoperative PAH had better survival than those with idiopathic PAH, according to age (hazard ratio [HR] 0.128, 95% confidence interval [CI]: 0.07-0.22, P < 0.0001); Kaplan-Meier survival estimates at 5 years for idiopathic and postoperative PAH patients were 74.3% and 92.6%, respectively. Patients in New York Heart Association functional class III-IV had an over 4-fold increased risk of death (HR 4.85, 95% CI: 2.61-9.00, P < 0.0001). Patients < 18 years of age at idiopathic PAH diagnosis had a worse survival compared to adult patients (HR 6.90, 95% CI: 4.19-15.56, P = 0.040). CONCLUSIONS: Postoperative-PAH patients had better midterm survival compared to patients with idiopathic PAH. Mortality was significant in both PAH groups, reinforcing the need for early diagnosis and optimal individualized management to improve outcomes.
Bergbaum C, Samaranayake CB, Pitcher A, et al., 2021, A case series on the use of steroids and mycophenolate mofetil in idiopathic and heritable pulmonary veno-occlusive disease: is there a role for immunosuppression?, EUROPEAN RESPIRATORY JOURNAL, Vol: 57, ISSN: 0903-1936
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- Citations: 5
Constantine A, Condliffe R, Clift P, et al., 2021, Palliative care in pulmonary hypertension associated with congenital heart disease: systematic review and expert opinion, ESC Heart Failure, Vol: 8, Pages: 1901-1904, ISSN: 2055-5822
AimsPulmonary arterial hypertension (PAH) is common amongst patients with congenital heart disease (CHD). It is a severe and complex condition that adversely affects quality of life and prognosis. While quality of life questionnaires are routinely used in clinical pulmonary hypertension practice, little is known on how to interpret their results and manage PAH‐CHD patients with evidence of impaired health‐related quality of life, especially those with advanced disease and palliative care needs.Methods and resultsWe performed a systematic review of studies concerning palliative care for people with PAH‐CHD, also reviewing the health‐related quality of life literature pertaining to these patients. Of 330 papers identified through initial screening, 17 were selected for inclusion. Underutilization of advance care planning and palliative care resources was common. Where palliative care input was sought, this was frequently late in the course of the disease. No studies provided evidence‐based clinical criteria for triggering referral to palliative care, a framework for providing tailored care in this patient group, or how to manage the risk of sudden cardiac death and implantable cardioverter defibrillators in advanced PAH‐CHD. We synthesize this information into eight important areas, including the impact of PAH‐CHD on quality of life, barriers to and benefits of palliative care involvement, advance care planning discussions, and end‐of‐life care issues in this complex patient group, and provide expert consensus on best practice in this field.ConclusionsThis paper presents the results of a systematic review and expert statements on the preferred palliative care strategy for patients with PAH‐CHD.
Surkova E, Dimopoulos K, Li W, 2021, Reply to: "Systolic dysfunction of the subpulmonary left ventricle in the systemic right ventricle: late manifestation or subclinical predictor of heart failure?", INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 332, Pages: 69-69, ISSN: 0167-5273
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- Citations: 1
Bianchi P, Constantine A, Costola G, et al., 2021, Ultra fast track extubation in adult congenital heart surgery, Journal of the American Heart Association, Vol: 10, ISSN: 2047-9980
Background:In pediatric cardiac surgery, perioperative management has evolved from slow weaning of mechanical ventilation in the intensive care unit to “ultra‐fast‐track” anesthesia with early extubation (EE) in theater to promote a faster recovery. The strategy of EE has not been assessed in adults with congenital heart disease, a growing population of patients who often require surgery.Methods And Results:Data were collected retrospectively on all patients >16 years of age who underwent adult congenital heart surgery in our tertiary center between December 2012 and January 2020. Coarsened exact matching was performed for relevant baseline variables. Overall, 711 procedures were performed: 133 (18.7%) patients underwent EE and 578 (81.3%) patients received conventional extubation. After matching, patients who received EE required less inotropic or vasopressor support in the early postoperative period (median Vasoactive‐inotropic score 0.5 [0.0–2.0] versus 2.0 [0.0–3.5]; P<0.0001) and had a lower total net fluid balance than patients after conventional extubation (1168±723 versus 847±733 mL; P=0.0002). The overall reintubation rate was low at 0.3%. EE was associated with a significantly shorter postoperative length of stay in higher dependency care units before a “step‐down” to ward‐based care (48 [45–50] versus 50 [47–69] hours; P=0.004). Lower combined intensive care unit and high dependency unit costs were incurred by patients who received EE compared with patients who received conventional extubation (£3949 [3430–4222] versus £4166 [3893–5603]; P<0.0001).Conclusions:In adult patients undergoing surgery for congenital heart disease, EE is associated with a reduced need for postoperative hemodynamic support, a shorter intensive care unit stay, and lower health‐care‐related costs.
Barradas-Pires A, Boyalla V, Dimopoulos K, 2021, Gender, an additional cardiovascular risk factor?Y, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 331, Pages: 270-272, ISSN: 0167-5273
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- Citations: 1
Vidal MLB, Diller G-P, Kempny A, et al., 2021, UTILITY OF DEEP LEARNING ALGORITHMS IN DIAGNOSING AND AUTOMATIC PROGNOSTICATION OF PULMONARY ARTERIAL HYPERTENSION BASED ON ROUTINE ECHOCARDIOGRAPHIC IMAGING, 70th Annual Scientific Session and Expo of the American-College-of-Cardiology (ACC), Publisher: ELSEVIER SCIENCE INC, Pages: 1670-1670, ISSN: 0735-1097
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- Citations: 1
Barradas-Pires A, Constantine A, Dimopoulos K, 2021, Preventing disease progression in Eisenmenger syndrome, Expert Review of Cardiovascular Therapy, Vol: 19, Pages: 501-518, ISSN: 1744-8344
Introduction: Eisenmenger syndrome describes a condition in which a congenital heart defect has caused severe pulmonary vascular disease, resulting in reversed (right-left) or bidirectional shunting and chronic cyanosis.Areas covered: In this paper, the progression of congenital heart defects to Eisenmenger syndrome, including early screening, diagnosis and operability are covered. The mechanisms of disease progression in Eisenmenger syndrome and management strategies to combat this, including the role of pulmonary arterial hypertension therapies, are also discussed.Expert opinion/commentary: Patients with congenital heart disease (CHD) are at increased risk of developing pulmonary arterial hypertension with Eisenmenger syndrome being its extreme manifestation. All CHD patients should be regularly assessed for pulmonary hypertension. Once Eisenmenger syndrome develops, shunt closure should be avoided. The clinical manifestations of Eisenmenger syndrome are driven by the systemic effects of the pulmonary hypertension, congenital defect and long-standing cyanosis. Expert care is essential for avoiding pitfalls and preventing disease progression in this severe chronic condition, which is associated with significant morbidity and mortality. Pulmonary arterial hypertension therapies have been used alongside supportive care to improve the quality of life, exercise tolerance and the outcome of these patients, although the optimal timing for their introduction and escalation remains uncertain.
Price LC, Martinez G, Brame A, et al., 2021, Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement, BRITISH JOURNAL OF ANAESTHESIA, Vol: 126, Pages: 774-790, ISSN: 0007-0912
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- Citations: 21
Ntiloudi D, Dimopoulos K, Tzifa A, et al., 2021, Hospitalizations in adult patients with congenital heart disease: an emerging challenge, HEART FAILURE REVIEWS, Vol: 26, Pages: 347-353, ISSN: 1382-4147
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- Citations: 5
Lewis RA, Armstrong I, Bergbaum C, et al., 2021, EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study, EUROPEAN RESPIRATORY JOURNAL, Vol: 57, ISSN: 0903-1936
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- Citations: 21
Surkova E, Segura T, Dimopoulos K, et al., 2021, Systolic dysfunction of the subpulmonary left ventricle is associated with the severity of heart failure in patients with a systemic right ventricle, International Journal of Cardiology, Vol: 324, Pages: 66-71, ISSN: 0167-5273
BACKGROUND: The study aimed to assess the relation between echocardiographic parameters of subpulmonary left ventricular (LV) size and function, and the severity of heart failure in patients with a systemic right ventricle (SRV). METHODS AND RESULTS: A total of 157 patients (89 post Mustard/Senning operations, 68 with congenitally corrected transposition of great arteries [ccTGA]) were included. The size and function of the SRV and subpulmonary LV were assessed on the most recent echocardiographic exam. Clinical data were collected from the electronic records. The majority (133, 84.7%) were in NYHA functional class 1-2. Median BNP concentration was 79.5[38.3-173.3] ng/l, and 100 (63.7%) patients were receiving heart failure therapy. Both LV and SRV fractional area change (FAC) differed significantly between patients with NYHA class 1-2 vs 3-4 (48[41.5-52.8]% vs 34[28.6-38.6]%, p < 0.0001 and 29.5[23-35]% vs 22[20-27]%, p < 0.0001, respectively), but LV FAC had a higher discriminative power for functional class >2 than SRV FAC (AUC 0.90, p < 0.0001 vs 0.79; p < 0.0001, respectively). A LV FAC cut-off value <39.2% had the highest accuracy in identifying patients with NYHA class 3-4 (sensitivity 83% and specificity 88%). In multivariable logistic regression analysis, LV FAC and SRV FAC independently associated to NYHA class 3-4 (OR 0.80 [95%CI 0.72-0.88], p < 0.0001 and OR 0.85 [95%CI 0.76-0.96], p = 0.007, respectively). CONCLUSIONS: Subpulmonary LV systolic dysfunction is associated with NYHA functional class 3-4 in patients with ccTGA or after Mustard or Senning operation. Careful evaluation of the subpulmonary LV should be a part of the routine assessment of patients with a SRV.
Scognamiglio G, Fusco F, Hankel TC, et al., 2021, Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 322, Pages: 129-134, ISSN: 0167-5273
Barradas-Pires A, DiazNuila Alcazar M, Martinez-Rubio A, et al., 2021, Something has got to give: funding innovation in an era of rigid budgeting, and why physicians should care, EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY, Vol: 28, Pages: 44-46, ISSN: 2047-4873
Ferrero P, Chessa M, Piazza I, et al., 2021, COVID-19 in adults with congenital heart disease: early morbidity and mortality in two northern Italian specialist centers, ITALIAN JOURNAL OF MEDICINE, Vol: 15, Pages: 27-33, ISSN: 1877-9344
Arvanitaki A, Ntiloudi D, Giannakoulas G, et al., 2021, Prediction Models and Scores in Adult Congenital Heart Disease, CURRENT PHARMACEUTICAL DESIGN, Vol: 27, Pages: 1232-1244, ISSN: 1381-6128
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- Citations: 7
Dimopoulos K, Opotowsky AR, Constantine A, et al., 2020, Often transferred, rarely transitioned: The current state of transition for young people with congenital heart disease, International Journal of Cardiology, Vol: 329, Pages: 89-90, ISSN: 0167-5273
Opotowsky AR, Dimopoulos K, Buber J, 2020, Implications of Elevated Pulmonary Artery Pressure for Transcatheter Mitral Repair Time for Comprehensive Hemodynamic Investigation, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 76, Pages: 2607-2610, ISSN: 0735-1097
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- Citations: 3
Nashat H, Favoccia C, Constantine A, et al., 2020, The investigation and diagnosis of pulmonary hypertension in adults with congenital heart disease, Journal of Congenital Cardiology, Vol: 4, Pages: 1-9, ISSN: 2056-7251
Pulmonary hypertension is not uncommon in adult patients with congenital heart disease and can significantly affect their exercise capacity, quality of life and prognosis. Timely identification and management of pulmonary hypertension in these patients is important. Patients must be allocated to the correct diagnostic group and treatment decided upon, including offering pulmonary arterial hypertension therapies to those who are most likely to benefit.In this paper, we provide an overview of the diagnostic modalities that are used to investigate and diagnose pulmonary hypertension in modern adult congenital heart disease practice. Emphasis is placed on echocardiography, which is the modality of choice for raising the suspicion of PH, and cardiac catheterisation, which is fundamental in establishing the diagnosis. Other modalities, such as cardiac magnetic resonance imaging and computed tomography can provide complementary information on anatomy and physiology.Combining all this information into a clinical diagnosis and management plan requires clinical expertise and a multidisciplinary approach, especially when managing this rare and complex group of patients.
Barracano R, Nashat H, Constantine A, et al., 2020, Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication, Journal of Congenital Cardiology, Vol: 4
<jats:title>Abstract</jats:title><jats:sec> <jats:title>Background</jats:title> <jats:p>Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality.</jats:p> </jats:sec><jats:sec> <jats:title>Case presentation</jats:title> <jats:p>We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant.</jats:p> </jats:sec><jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.</jats:p> </jats:sec>
Brida M, Favoccia C, Constantine A, et al., 2020, Pulmonary arterial hypertension associated with congenital heart disease after defect repair: the effect of pregnancy, Journal of Congenital Cardiology, Vol: 4, Pages: 1-4
BackgroundAn increasing number of patients with previously repaired congenital heart disease (CHD) present with pulmonary arterial hypertension (PAH). This can occur immediately after repair (residual PAH) or years later.Case presentationWe present the case of a young woman who underwent repair of a ventricular septal defect in later childhood. Three years after repair, she was found to have significant residual PAH. She remained stable on PAH therapies, but a decade later decided to become pregnant against medical advice. She deteriorated during pregnancy and required escalation of PAH therapies and eventual admission to the intensive care unit, with an uneventful delivery at 32 weeks. Despite successful delivery, she remained symptomatic post-partum, with evidence of disease progression at right heart catheterisation.ConclusionsAll patients with repaired CHD should undergo routine screening for PAH. Early diagnosis and expert management, including the use of PAH therapies, is recommended to optimise outcome. Pregnancy is contraindicated in PAH patients, including patients with CHD, and requires an expert multidisciplinary approach to reduce morbidity and mortality when patients opt to proceed.
Nashat H, Constantine A, Dimopoulos K, 2020, Advanced therapies in Eisenmenger syndrome, Journal of Congenital Cardiology, Vol: 4, Pages: 1-5, ISSN: 2056-7251
BackgroundThis is a case report of a patient diagnosed with Eisenmenger syndrome in adult life.Case presentationThe patient had a large secundum atrial septal defect diagnosed incidentally in her twenties, with established pulmonary vascular disease and thus the defect was not closed. Over several years the patient showed signs of progressive disease with premature right ventricular dysfunction, preceding any significant symptomatic decline. Her medical therapy was escalated with the addition of intravenous epoprostenol, resulting in both objective and symptomatic improvement.ConclusionsEarly medical intervention and treatment can positively impact on the outcome of patients with pulmonary arterial hypertension associated with congenital heart disease.
Constantine A, Tulloh RMR, Turquet R, et al., 2020, PAH-CHD: transition to adulthood, Journal of Congenital Cardiology, Vol: 4, Pages: 1-4, ISSN: 2056-7251
BackgroundA structured transition provides a framework of care that bridges the gap between paediatric and adult medicine. It is essential for achieving continuity of care and providing support and education around the challenging period of adolescence for young people with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).PresentationIn this review of transition care in PAH-CHD, we evaluate the evidence supporting a structured programme of transition care and review the current principles and ‘best practice’ standards for transition in the UK. In the second part of the review, we highlight some important areas of education that are relevant to adolescents with PAH-CHD, including health education, exercise and participation in sports, pregnancy and contraception, employment, and driving.ConclusionsAs the number of young people embarking on transition continues to increase, the challenge is set to continue to improve the quality of care for our patients within the framework of available resources.
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