Publications
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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.
Constantine A, Dimopoulos K, Rafiq I, et al., 2020, Sex differences in hypertrophic cardiomyopathy: time to tailor risk stratification and therapy?, European Journal of Preventive Cardiology, Vol: 27, Pages: 1816-1818, ISSN: 2047-4873
Costola G, Constantine A, Bianchi P, et al., 2020, Early extubation following cardiac surgery in adult congenital heart disease improves post-operative haemodynamics and reduces costs, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2202-2202, ISSN: 0195-668X
Constantine A, Jenkins P, Oliver J, et al., 2020, Multicentre study on pulmonary arterial hypertension therapies in fontan patients: underutilised or of limited use?, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2220-2220, ISSN: 0195-668X
Krishnathasan K, Constantine A, Fitzsimmons S, et al., 2020, Transition for patients with congenital heart disease in the UK: need for a universal model with adequate training and support, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2203-2203, ISSN: 0195-668X
Gribaudo E, Constantine A, Costola G, et al., 2020, Patients with Fontan circulation beyond the fourth decade of life, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2221-2221, ISSN: 0195-668X
Gribaudo E, Constantine A, Pires A, et al., 2020, Long term follow-up after pregnancy in Fontan patients, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2212-2212, ISSN: 0195-668X
Meneguzzo G, Costola G, Constantine A, et al., 2020, Peak oxygen uptake on cardio pulmonary exercise testing predicts mortality in adult Fontan patients, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2178-2178, ISSN: 0195-668X
De Santis J, Constantine A, Ministeri M, et al., 2020, Strong association between cardio-pulmonary exercise parameters and mortality in adults with transposition of the great arteries and a systemic right ventricle, ESC Congress 2020, Publisher: OXFORD UNIV PRESS, Pages: 2219-2219, ISSN: 0195-668X
Ghonim S, Ernst S, Keegan J, et al., 2020, 3D late gadolinium enhancement cardiovascular magnetic resonance predicts inducibility of ventricular tachycardia in adults with repaired tetralogy of Fallot, Circulation: Arrhythmia and Electrophysiology, Vol: 13, Pages: 1331-1341, ISSN: 1941-3084
Background - Adults with repaired tetralogy of Fallot (rTOF) die prematurely from ventricular tachycardia (VT) and sudden cardiac death. Inducible VT predicts mortality. Ventricular scar, the key substrate for VT, can be non-invasively defined with late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) but whether this relates to inducible VT is unknown.Methods - Sixty-nine consecutive rTOF patients (43 male, mean 40{plus minus}15 years) clinically scheduled for invasive programmed VT-stimulation were prospectively recruited for prior 3D LGE CMR. Ventricular LGE was segmented and merged with reconstructed cardiac chambers and LGE volume measured.Results - VT was induced in 22(31%) patients. Univariable predictors of inducible VT included increased RV LGE (OR 1.15;p=0.001 per cm3), increased non-apical vent LV LGE (OR 1.09;p=0.008 per cm3), older age (OR 1.6;p=0.01 per decile), QRS duration ≥180ms (OR 3.5;p=0.02), history of non-sustained VT (OR 3.5; p=0.02) and previous clinical sustained VT (OR 12.8;p=0.003); only prior sustained VT (OR 8.02;p=0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR 1.14;p=0.003). An RV LGE volume of 25cm3 had 72% sensitivity and 81% specificity for predicting inducible VT (AUC 0.81;p<0.001). At the extreme cutoffs for 'ruling-out' and 'ruling-in' inducible VT, RV LGE >10cm3 was 100% sensitive and >36cm3 was 100% specific for predicting inducible VT.Conclusions - 3D LGE CMR-defined scar burden is independently associated with inducible VT and may help refine patient selection for programmed VT-stimulation when applied to an at least intermediate clinical risk cohort.
Pires AB, Boyalla V, Meras-Colunga P, et al., 2020, Free dental care for patients at high risk of developing infective endocarditis - a cost-effectiveness study, European-Society-of-Cardiology (ESC) Congress, Publisher: OXFORD UNIV PRESS, Pages: 3536-3536, ISSN: 0195-668X
Ladouceur M, Hobbs K, De Gonneville A, et al., 2020, Long-term outcomes amongst adults with anatomic repair for transposition of the great arteries: not as perfect as we would have hoped?, European-Society-of-Cardiology (ESC) Congress, Publisher: OXFORD UNIV PRESS, Pages: 2181-2181, ISSN: 0195-668X
Price L, Brame A, Martinez G, et al., 2020, Perioperative management of patients with Pulmonary Hypertension undergoing Non-Cardiac Surgery: A Systemic Review and UK Consensus Statement, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Price LC, Kouranos V, Dimopoulos K, et al., 2020, Sarcoidosis-associated Pulmonary Hypertension: A London Cohort, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Lewis R, Armstrong I, Bergbaum C, et al., 2020, 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 multi-centre study, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 3
Constantine A, Dimopoulos K, Opotowsky A, 2020, Congenital heart disease and pulmonary hypertension, Cardiology Clinics, Vol: 38, Pages: 445-456, ISSN: 0733-8651
Pulmonary hypertension is common in adults with congenital heart disease and carries fundamental implications for management and prognosis. A high index of suspicion, combined with knowledge of the pathogenesis and pathophysiology of PH is required to achieve a timely, accurate diagnosis, and appropriate classification and treatment. Herein, we provide a guide on how to approach the adult with congenital heart disease and suspected PH of different types, including current management.
Constantine A, Barradas-Pires A, Dimopoulos K, 2020, Modifiable risk factors in congenital heart disease: Education, transition, digital health and choice architecture, European Journal of Preventive Cardiology, Vol: 27, Pages: 1074-1076, ISSN: 2047-4873
Stalikas N, Doundoulakis I, Karagiannidis E, et al., 2020, Non-Vitamin K Oral Anticoagulants in Adults with Congenital Heart Disease: A Systematic Review, JOURNAL OF CLINICAL MEDICINE, Vol: 9
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- Citations: 13
Rafiq I, Khokar AA, Alonso-Gonzalez R, et al., 2020, Severe left ventricular outflow tract obstruction immediately after surgical repair of Ebstein anomaly, Jacc Case Reports, ISSN: 2666-0849
Constantine A, Dimopoulos K, 2020, Pulmonary artery denervation for pulmonary arterial hypertension, Trends in Cardiovascular Medicine, Vol: 31, Pages: 252-260, ISSN: 1050-1738
Pulmonary arterial hypertension remains a progressive, life-limiting disease despite optimal medical therapy. Pulmonary artery denervation has arisen as a novel intervention in the treatment of pulmonary arterial hypertension, and other forms of pulmonary hypertension, with the aim of reducing the sympathetic activity of the pulmonary circulation. Pre-clinical studies and initial clinical trials have demonstrated that the technique can be performed safely with some positive effects on clinical, haemodynamic and echocardiographic markers of disease. The scope of the technique in current practice remains limited given the absence of well-designed, large-scale, international randomised controlled clinical trials. This review provides an overview of this exciting new treatment modality, including pathophysiology, technical innovations and recent trial results.
Angelini A, di Gioia C, Doran H, et al., 2020, Autopsy in adults with congenital heart disease (ACHD)., Virchows Archiv: an international journal of pathology, Vol: 476, Pages: 797-820, ISSN: 0945-6317
The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients.
McCabe C, Dimopoulos K, Pitcher A, et al., 2020, Chronic thromboembolic disease following pulmonary embolism: time for a fresh look at old clot, EUROPEAN RESPIRATORY JOURNAL, Vol: 55, ISSN: 0903-1936
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- Citations: 7
Martin-Garcia AC, Dimopoulos K, Boutsikou M, et al., 2020, Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement, HEART, Vol: 106, Pages: 455-461, ISSN: 1355-6037
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- Citations: 8
Cazzoli I, Gunturiz-Beltran C, Guarguagli S, et al., 2020, Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations, International Journal of Cardiology, Vol: 301, Pages: 127-134, ISSN: 0167-5273
IntroductionArrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation.MethodsFive consecutive patients (3 female, mean age 47.8 ± 12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients.ResultsPatients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4–1375) from their last ablation. During a median follow up of 31 months (range 1–70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing.ConclusionsCatheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.
Nashat H, Kempny A, Harries C, et al., 2020, A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 299, Pages: 131-135, ISSN: 0167-5273
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- Citations: 10
Yang H, Bouma BJ, Dimopoulos K, et al., 2020, Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 299, Pages: 123-130, ISSN: 0167-5273
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- Citations: 40
Bax S, Jacob J, Ahmed R, et al., 2020, Right ventricle to left ventricle ratio at CTPA predicts mortality in interstitial lung disease, Chest, Vol: 157, Pages: 89-98, ISSN: 0012-3692
INTRODUCTION: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to ILD severity. Right ventricle to left ventricle diameter ratio (RV:LV) measured at CT pulmonary angiography (CTPA), has been shown to provide valuable information in pulmonary arterial hypertension patients and to predict death or deterioration in acute pulmonary embolism. METHODS: Demographics, ILD subtype, echocardiography and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterisation (RHC) at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored using CT criteria. RV:LV ratio at CTPA was evaluated by three different methods. Cox-proportional hazard analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. RESULTS: 92 patients were included: 64% male, mean age 65±11 years, with FVC 57±20% (predicted), TLCOc 22±8% (predicted) and KCOc 51±17% (predicted). PH was confirmed at RHC in 78%. Of all CTPA-derived measures, an RV:LV ratio ≥1.0 strongly predicted mortality or transplantation at univariate analysis (HR 3.26, 95%CI:1.49-7.13, p=0.003), whereas invasive haemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (HR: 3.19, CI:1.44-7.10, p=0.004), adjusting for an ILD diagnosis of IPF and CT derived ILD severity. CONCLUSION: An increased RV:LV ratio measured at CTPA provides a simple, non-invasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow up, more aggressive treatment and consideration of lung transplantation.
Tulloh R, Dimopoulos K, Condliffe R, et al., 2019, Management of Adults with Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK: Survey of Current Practice, Unmet Needs and Expert Commentary (vol 27, pg 1018, 2018), HEART LUNG AND CIRCULATION, Vol: 28, Pages: 1915-1915, ISSN: 1443-9506
Sathianandan S, McCabe C, Dimopoulos K, et al., 2019, SILDENAFIL IN THE TREATMENT OF GROUP 3 PULMONARY HYPERTENSION, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A152-A152, ISSN: 0040-6376
Li W, Yin Y, Dimopoulos K, et al., 2019, Early and late effects of cardiac resynchronization therapy in adult congenital heart disease, Journal of the American Heart Association, Vol: 8, ISSN: 2047-9980
Background: There are limited data regarding cardiac resynchronization therapy (CRT) in adult congenital heart disease (ACHD). We aimed to assess early and late outcomes of CRT amongst patients with ACHD.Methods: We retrospectively studied ACHD patients receiving CRT (2004-2017). Clinical and echocardiographic data were analyzed at baseline, early (1.8±0.8 years) and late (4.7±0.8 years) follow-up after CRT.Results: Fifty-four ACHD patients (median age 46 years, range 18-73 years, 74% male) had CRT (biventricular paced >90%) and were followed for 5.7±3.0 years. Compared to baseline, CRT was associated with significant improvement at early follow-up in NYHA functional class, QRS duration and cardiothoracic ratio (P<0.05 for all); improvement in NYHA class was sustained at late follow-up.Amongst patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction (LVEF) and reduction in LV end-systolic volume at early and late follow up (P<0.05 for both). For patients with a systemic right ventricle (RV; n=15), there was a significant early but not late reduction in systemic RV basal and longitudinal diameters.Eleven patients died and 2 had heart transplantation unrelated to systemic ventricular morphology. Thirty-five (65%) patients responded positively to CRT but only baseline QRS duration was predictive of a positive response.Conclusions: CRT results in sustained improvement in functional class, systemic LV size and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphology, were most likely to respond to CRT.
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