Publications
503 results found
Barradas Pires A, Dimopoulos K, Constantine A, 2023, Repair of aortic regurgitation in young adults: sooner rather than later, Journal of the American Heart Association, ISSN: 2047-9980
AbstractObjectives: Establishing surgical criteria for aortic valve replacement (AVR) or repair in severe aortic regurgitation (AR) is challenging and evidence-based recommendations for young adults are lacking. We studied the indications for AVR in a cohort of young adults with severe AR from a tertiary centre, and the associated outcomes. The relation between pre-surgical echocardiographic parameters and post-operative left ventricular (LV) size and function, clinical events, and prosthetic valve-related complications was also investigated.Methods: Data were collected retrospectively on consecutive adult patients who underwent AVR or repair for severe AR between 2005 and 2019 in a tertiary cardiac centre. Results: One-hundred-and-seventy-two patients were included (age at surgery 29 [22-41] years, 81% male). One third were operated before meeting standard guideline indications. Normalization of LV size and function was achieved in 65% of patients. No significant change in LV ejection fraction (EF) from baseline to the latest follow-up (p=0.08) was observed. Pre-surgical LV end-systolic diameter (LVESD) was associated with lack of LV normalization post-surgery (OR per 1 cm increase 2.81, 95%CI:1.54 - 5.56, p<0.01). On ROC analysis, the cut-off for baseline LVESD that maximizes sensitivity and specificity for lack of LV normalization post-surgery was 43mm. Pre- and post-operative LV dimensions, and post-operative LVEF were predictors of clinical events during follow-up. Prosthesis-related complications were frequent (20.3%) during a follow-up of 5.6 [2.9-9.2] years. Freedom from aortic re-intervention was 98%, 96.5%, 85.4% at 1, 5 and 10 years, respectively.Conclusions: Patients with a history of cardiac surgery and/or increased baseline LVESD are less likely to achieve normalization of LV size and function following AVR. Clinicians must carefully balance the potential benefits of AVR on long-term outcomes against the risk of periprocedural complications and the
Seitler S, Dimopoulos K, Ernst S, et al., 2023, Medical Emergencies in Pulmonary Hypertension., Semin Respir Crit Care Med
The management of acute medical emergencies in patients with pulmonary hypertension (PH) can be challenging. Patients with preexisting PH can rapidly deteriorate due to right ventricular decompensation when faced with acute physiological challenges that would usually be considered low-risk scenarios. This review considers the assessment and management of acute medical emergencies in patients with PH, encompassing both pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), acknowledging these comprise the more severe groups of PH. Management protocols are described in a systems-based approach. Respiratory emergencies include pulmonary embolism, airways disease, and pneumonia; cardiac emergencies including arrhythmia and chest pain with acute myocardial infarction are discussed, alongside PH-specific emergencies such as pulmonary artery dissection and extrinsic coronary artery compression by a dilated proximal pulmonary artery. Other emergencies including sepsis, severe gastroenteritis with dehydration, syncope, and liver failure are also considered. We propose management recommendations for medical emergencies based on available evidence, international guidelines, and expert consensus. We aim to provide advice to the specialist alongside the generalist, and emergency doctors, nurses, and acute physicians in nonspecialist centers. A multidisciplinary team approach is essential in the management of patients with PH, and communication with local and specialist PH centers is paramount. Close hemodynamic monitoring during medical emergencies in patients with preexisting PH is vital, with early referral to critical care recommended given the frequent deterioration and high mortality in this setting.
Surkova E, Constantine A, Xu Z, et al., 2023, Prognostic significance of subpulmonary left ventricular size and function in patients with a systemic right ventricle, European Heart Journal - Cardiovascular Imaging, ISSN: 2047-2404
AimTo assess the additional prognostic significance of echocardiographic parameters of subpulmonary left ventricular (LV) size and function in patients with a systemic right ventricle (SRV).Methods and ResultsAll adults with a SRV who underwent transthoracic echocardiography in 2010-2018 at a large tertiary center were identified. Biventricular size and function were assessed at the most recent exam. The study endpoint was all-cause mortality or heart/heart-lung transplantation.We included 180 patients, 100(55.6%) male, mean age 42.4±12.3 years, of whom 103(57.2%) had undergone Mustard/Senning operations and 77(42.8%) had congenitally corrected transposition of great arteries.Over 4.9[3.8-5.7] years, 28(15.6%) patients died and 4(2.2%) underwent heart or heart-lung transplantation. Univariable predictors of the study endpoint included age, NYHA functional class III or IV, history of atrial arrhythmias, presence of pacemaker or cardioverter-defibrillator, high BNP, and echocardiographic markers of SRV and subpulmonary LV size and function. On multivariable Cox analysis of echocardiographic variables, indexed LV end-systolic diameter (ESDi; HR 2.77 [95%CI 1.35-5.68], p=0.01), LV fractional area change (FAC; HR 0.7 [95%CI 0.57-0.85], p=0.002), SRV basal diameter (HR 1.66 [95%CI 1.21-2.29], p=0.005), and SRV FAC (HR 0.65 [95%CI 0.49-0.87], p=0.008) remained predictive of mortality or transplantation. On ROC analysis, subpulmonary LV parameters performed better than SRV markers in predicting adverse events.ConclusionsSRV basal diameter, SRV FAC, LV ESDi, and LV FAC are significantly and independently associated with mortality and transplantation in adults with a SRV. Accurate echocardiographic assessment of both SRV and subpulmonary LV is therefore essential to inform risk stratification and management.
Chivers S, Cleary A, Knowles R, et al., 2023, COVID-19 in congenital heart disease (COaCHeD) study., Open Heart, Vol: 10, ISSN: 2053-3624
BACKGROUND: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care. OBJECTIVE: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes. METHODS: Multicentre UK study undertaken 1 March 2020-30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation. RESULTS: There were 405 reported cases (127 paediatric/278 adult). In children (age <16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p<0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p<0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)). CONCLUSIONS: Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD.
Dimopoulos K, Bouchard M, Cheryl Chong Z, et al., 2023, Transition to adult care of young people with congenital heart disease: Impact of a service on knowledge and self-care skills, and correlates of a successful transition, European Heart Journal - Quality of Care and Clinical Outcomes, Vol: 9, Pages: 351-357, ISSN: 2058-5225
AimsLess than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition.Methods and resultsOverall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P < 0.0001; describing: 14.4–42.7%, P < 0.0001; understanding: 26.1–60.7%, P < 0.0001), and from the second to the third (naming: 67.4%, P = 0.004, describing: 61.4%, P < 0.001, understanding: 71.1%, P = 0.02;). Patients with LD did not improve their disease knowledge over time (all P > 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition.ConclusionA transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.
Samaranayake CB, Kempny A, Naeije R, et al., 2023, Beta-blockade improves right ventricular diastolic function in exercising pulmonary arterial hypertension, European Respiratory Journal, Vol: 61, ISSN: 0903-1936
Krishnathasan K, Constantine A, Rafiq I, et al., 2023, Management of pulmonary arterial hypertension during pregnancy., Expert Rev Respir Med, Vol: 17, Pages: 413-423
INTRODUCTION: Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >20 mmHg and pulmonary vascular resistance >2 Wood Units (WU) on right-heart catheterization. Pregnancy is generally contraindicated in PAH, it is associated with high maternal mortality. Despite current recommendations, the number of women with PAH wishing to become pregnant is increasing. Specialist care is essential for preconception counseling, and the management of pregnancy and delivery in such patients. AREAS COVERED: We cover the physiology of pregnancy, and its effects on the cardiovascular system in PAH. We also discuss optimal management based on available evidence and guidance. EXPERT OPINION: Pregnancy should be avoided in most patients with PAH. Counseling on appropriate contraception should be offered routinely. Education of women with childbearing potential is essential and should start at the time of diagnosis of PAH, or the time of transition from pediatric to adult services in patients developing PAH in childhood. Women wishing to become pregnant should receive individualized risk assessment and optimization of PAH therapies via a dedicated specialist pre-pregnancy counseling service, to minimize risk and improve outcomes. Pregnant PAH patients should receive expert multidisciplinary management in a PH center, including close monitoring and early initiation of therapies.
Krishnathasan K, Dimopoulos K, Duncan N, et al., 2023, Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes, European Journal of Preventive Cardiology, Pages: 1-8, ISSN: 2047-4873
AimsPrevious studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021.Methods and resultsThis retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4–12) days, with a maximum dose of 120 (80–160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2–82.3%] and 43.3% (95% CI: 36–52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome.ConclusionAdult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.
Dawes TJW, McCabe C, Dimopoulos K, et al., 2023, Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study, Respirology, Vol: 28, Pages: 262-272, ISSN: 1323-7799
Background and ObjectivePulmonary hypertension is a life-limiting complication of interstitial lung disease (ILD-PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD-PH was associated with improved survival.MethodsConsecutive incident patients with ILD-PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow-up and survival data modelled by Bayesian methods.ResultsThe diagnoses in 128 patients were idiopathic pulmonary fibrosis (n = 74, 58%), hypersensitivity pneumonitis (n = 17, 13%), non-specific interstitial pneumonia (n = 12, 9%), undifferentiated ILD (n = 8, 6%) and other lung diseases (n = 17, 13%). Final outcomes were death (n = 106, 83%), transplantation (n = 9, 7%) and censoring (n = 13, 10%). Patients treated with PDE5i (n = 50, 39%) had higher mean pulmonary artery pressure (median 38 mm Hg [interquartile range, IQR: 34, 43] vs. 35 mm Hg [IQR: 31, 38], p = 0.07) and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], p=0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18 years [95% CI: 1.43, 3.04] vs. 0.94 years [0.69, 1.51], p = 0.003) independent of all other prognostic markers by Bayesian joint-modelling (HR 0.39, 95% CI: 0.23, 0.59, p < 0.001) and propensity-matched analyses (HR 0.38, 95% CI: 0.22, 0.58, p < 0.001). Survival difference with treatment was significantly larger if right ventricular function was normal, rather than abnormal, at presentation (+2.55 years, 95% CI: −0.03, +3.97 vs. +0.98 years, 95% CI: +0.47, +2.00, p = 0.04).ConclusionPDE5i treatment in ILD-PH should be investigated by a prospective randomized trial.
Constantine A, Dimopoulos K, Heng EL, et al., 2023, Transcatheter pulmonary valve implantation: An option for the few becoming an option for the many, International Journal of Cardiology, Vol: 373, Pages: 44-46, ISSN: 0167-5273
Dimopoulos K, Constantine A, Clift P, et al., 2023, Cardiovascular complications of Down syndrome: Scoping review and expert consensus, Circulation, Vol: 147, Pages: 425-441, ISSN: 0009-7322
Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension, co-existent pulmonary, endocrine, and metabolic diseases, and risk factors for atherosclerotic disease. Moreover, disparities in the cardiovascular care of people with Down syndrome compared with the general population, which vary across different geographies and healthcare systems, further contribute to cardiovascular mortality; this issue is often overlooked by the wider medical community. This review focuses on the diagnosis, prevalence and management of cardiovascular disease encountered in people with Down syndrome. Specifically, this article seeks to summarize available evidence in 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to disparities in care in areas of differing resource availability. All specialists and non-specialist clinicians providing care for people with Down syndrome should be aware of best clinical practice in all aspects of care of this distinct population.
Drakopoulou M, Dimopoulos K, Prapa S, et al., 2023, Adult Congenital Heart Disease, Pediatric Cardiac Surgery, Fifth Edition, Pages: 999-1054, ISBN: 9781119282310
The successful management and correction of congenital heart defects during infancy have resulted in an increasing number of adults with congenital heart disease (CHD). There are numerous issues to be considered when caring for adult CHD (ACHD) patients, many of which are unique to this population. Resternotomy in patients who have undergone prior palliative or corrective procedures carries an increased risk. The groin should be prepped and draped into the operative field should rapid access be required during resternotomy. Planning for an anesthetic procedure requires in-depth knowledge of the cardiac anatomy and physiology and the patient's medical history. On completion of the operation, the patient is transported to the intensive care unit. Coarctation of the aorta has a biphasic presentation. Initial presentation is shortly after birth when the ductus begins to close, resulting in the sequelae of decreased blood flow distal to the coarctation.
Mavromanoli AC, Barco S, Ageno W, et al., 2022, Recovery of right ventricular function after intermediate-risk pulmonary embolism: results from the multicentre Pulmonary Embolism International Trial (PEITHO)-2, CLINICAL RESEARCH IN CARDIOLOGY, ISSN: 1861-0684
Zhang C, Dimopoulos K, Li Q, et al., 2022, Long-term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension, PULMONARY CIRCULATION, Vol: 12, ISSN: 2045-8932
Krishnathasan K, Dimopoulos K, Duncan N, et al., 2022, Renal dysfunction: a predictor of adverse outcomes in ACHD patients with acute decompensated heart failure, ESC Congress 2022, Publisher: Oxford University Press, Pages: 1819-1819, ISSN: 0195-668X
Constantine A, Condliffe R, Ciift P, et al., 2022, Macitentan for pulmonary arterial hypertension related to repaired congenital heart disease: real-world UK experience, ESC Congress 2022, Publisher: Oxford University Press, Pages: 1928-1928, ISSN: 0195-668X
Ricci P, Bouchard M, Zhiya CC, et al., 2022, Efficacy of congenital heart disease transition clinics in improving patient education, ESC Congress 2022, Publisher: Oxford University Press, Pages: 1853-1853, ISSN: 0195-668X
Constantine A, Dimopoulos K, Condliffe R, et al., 2022, Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort, ESC Congress 2022, Publisher: Oxford University Press, Pages: 1874-1874, ISSN: 0195-668X
Constantine A, Dimopoulos K, Haworth SG, et al., 2022, Twenty-year experience and outcomes in a National Pediatric Pulmonary Hypertension Service, American Journal of Respiratory and Critical Care Medicine, Vol: 206, Pages: 758-766, ISSN: 1073-449X
Rationale:Pediatric pulmonary hypertension is an important cause of childhood morbidity and mortality, but there are limited data on the range of associated diseases, contributions of different pulmonary hypertension subtypes, therapeutic strategies, and clinical outcomes in children. Objectives:To report the 20-year experience of a large UK National Pediatric Pulmonary Hypertension Service focusing on epidemiology and clinical outcomes.Methods:Consecutive patients presenting between 2001 and 2021 were included and survival analysis was performed for incident patients.Measurements and Main Results:Of 1353 patients assessed, a pulmonary hypertension diagnosis was made in 1101(81.4%) patients (51% female, median age 2.6[IQR 0.8-8.2] years). The most common form was pulmonary arterial hypertension in 48%, followed by 32.3% with pulmonary hypertension due to lung disease. Multiple contributory causes of pulmonary hypertension were common, with 16.9% displaying features of more than one diagnostic group. The annual incidence of childhood pulmonary hypertension was 3.5(95%CI 3.3-3.8)/million children, and the prevalence was 18.1(95%CI 15.8-20.4)/million. The incidence was highest for pulmonary hypertension due to lung disease in infancy (15.0[95%CI 12.7-17.2]/million/year). Overall, 82.4% patients received pulmonary arterial hypertension therapy and escalation to triple therapy during follow-up was required in 13.1%. In 970(88.1%) incident patients, transplant-free survival was 86.7%(95%CI:84.5-89%) at 1, and 68.6%(95%CI:64.7-72.6%) at 10 years. Pulmonary hypertension due to left heart disease had the lowest survival (hazard ratio 2.0, 95%CI:1.36-2.94, p<0.001).Conclusions:Clinical phenotypes of pediatric pulmonary hypertension are heterogenous and overlapping, with clinical phenotypes that evolve throughout childhood. Despite widespread use of pulmonary arterial hypertension therapy, the prognosis remains poor.
Samaranayake C, Niglas M, Kempny A, et al., 2022, Acute beat blockade improves right ventricular diastolic filling in pulmonary arterial hypertension: a rodent CMR and clinical human pressure-volume study, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Samaranayake C, Mcniven R, Kempny A, et al., 2022, Ventilatory limitation during cardiopulmonary exercise testing predicts survival in patients with pulmonary arterial hypertension with Eisenmenger physiology, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Constantine A, Rhodes CJ, Ricci P, et al., 2022, Correlation between right ventricular dysfunction and plasma protein profile in pulmonary hypertension, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Samaranayake C, Kempny A, Price LC, et al., 2022, Metabolic modulation of the right ventricle and pulmonary circulation in pulmonary arterial hypertension: an interventional study using a Glucagon-like-peptide-1 (GLP-1) agonist, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Gillesén M, Fedchenko M, Giang KW, et al., 2022, Chronic kidney disease in patients with congenital heart disease: a nationwide, register-based cohort study., Eur Heart J Open, Vol: 2
AIMS: To investigate the risk of chronic kidney disease (CKD) in young patients with congenital heart disease (CHD) (age 0-47 years) compared with age- and sex-matched controls without CHD. METHODS AND RESULTS: Using data from the Swedish National Patient Register and the Cause of Death Register, 71,936 patients with CHD (50.2% male) born between 1970 and 2017 were identified. Each patient with CHD was matched by sex and age to 10 controls without CHD (n = 714,457). Follow-up data were collected for patients with CHD and controls until 2017. During a median follow-up of 13.5 (5.8; 25.5) years, 379 (0.5%) patients with CHD and 679 (0.1%) controls developed CKD. The risk of CKD was 6.4 times higher in patients with CHD than controls [95% confidence interval (CI): 5.65-7.27] and was highest in patients with severe non-conotruncal defects [hazard ratio (HR): 11.31; 95% CI: 7.37-17.36]. Compared with matched controls, the absolute and relative risks of CKD were greater for CHD patients born between 1997 and 2017 (HR: 9.98; 95% CI: 8.05-13.37) (incidence 39.5 per 100 000 person-years). The risk of CKD remained significantly higher after adjusting for hypertension, acute kidney injury, and diabetes mellitus (HR: 4.37; 95% CI: 3.83-5.00). CONCLUSION: Although the absolute risk of CKD in young patients with CHD is relatively low, patients with CHD are six times more likely to develop CKD than non-CHD controls up to the age of 47 years. Further data are needed to inform guidelines on the prevention and follow-up of CKD in CHD patients.
Diller GP, Vidal MLB, Kempny A, et al., 2022, A framework of deep learning networks provides expert-level accuracy for the detection and prognostication of pulmonary arterial hypertension, EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, Vol: 23, Pages: 1447-1456, ISSN: 2047-2404
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Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al., 2022, Eisenmenger Syndrome JACC State-of-the-Art Review, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 79, Pages: 1183-1198, ISSN: 0735-1097
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Constantine A, Rhodes CJ, Ricci P, et al., 2022, PLASMA PROTEIN PROFILE IN EISENMENGER SYNDROME AND OTHER FORMS OF PH: ASSOCIATION WITH MARKERS OF RV REMODELLING, ACC.22, Publisher: ELSEVIER SCIENCE INC, Pages: 1364-1364, ISSN: 0735-1097
Constantine A, Barradas-Pires A, Dimopoulos K, 2022, Cardiopulmonary exercise testing in congenital heart disease: Towards serial testing as part of long-term follow-up, European Journal of Preventive Cardiology, Vol: 29, Pages: 510-512, ISSN: 2047-4873
Ghonim S, Gatzoulis M, Ernst S, et al., 2022, Predicting survival in repaired tetralogy of Fallot- a lesion specific and personalised approach, JACC: Cardiovascular Imaging, Vol: 15, Pages: 257-268, ISSN: 1876-7591
Objective: We sought to identifyrepaired tetralogy of Fallot (rTOF) patients at high-risk of death and malignant ventricular arrhythmia (VA). Background: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. Methods: Consecutive patients were prospectively recruited for late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) to define right and left ventricular (RV,LV) fibrosisin addition to proven risk markers. Results: The primary end-point was all-cause mortality. Of the 550 patients, (median age 32 years, 56% male), 27 died (mean follow-up 6.4 (±5.8); total 3512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction (EF) ≤47%, LVEF ≤55%, B-type natriuretic peptide (BNP) ≥127ng/L, peak exercise oxygen uptake (V02) ≤17ml/kg/min, prior sustained atrial arrhythmia and age ≥50 years. The weighted scores for each of the above independent predictors differentiated a high-risk sub-group patients with a 4.4%, annual risk of mortality (AUC 0.87,P<0.001). The secondary end-point (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tractakinetic length ≥55mm and RVsystolic pressure ≥47mm Hg identified high-risk patients with a 3.7%, annual risk ofVA (AUC 0.79,P<0.001) RVLGE was heavily weighted in both risk scores due to its strong relative prognostic value. Conclusion: We present ascore integrating multiple appropriately weighted risk factors to identify the sub-group of rTOF patients that are at highannual risk of death who may benefit from targeted therapy.
Barradas-Pires A, Constantine A, Dimopoulos K, 2022, Percutaneous Interventions in Adult Congenital Heart Disease, Patient Reported Outcomes and Quality of Life in Cardiovascular Interventions, Pages: 171-184, ISBN: 9783031098147
Congenital Heart Disease (CHD) is a growing field in cardiology, but strong scientific evidence is lacking to help guide the recommendations of care. Patient reported outcomes, or PROMs, focused on quality of life in CHD are a helpful tool to assess the quality of care provided and model the research to focus on patients’ priorities. This review focuses on quality of life in CHD after percutaneous procedures, such as percutaneous pulmonary valve implantation, atrial septal defect closure and patent foramen ovale closure. Despite heterogeneity and methodological differences between the studies reviewed, evidence suggests that quality of life after such procedures is generally good, and overall comparable to the general population. This is true across different age groups, making it particularly relevant in the decision-making process on intervention in older patients.
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