Publications
516 results found
Mitropoulou P, Jenkins P, Camm CF, et al., 2024, The state of adult congenital heart disease training from the trainee perspective: A call for action., Eur Heart J Qual Care Clin Outcomes
Price LC, Kouranos V, Baughman R, et al., 2024, Use of pulmonary arterial hypertension therapies in patient swith sarcoidosis-associated pulmonary hypertension., Sarcoidosis Vasc Diffuse Lung Dis, Vol: 41
Daelman B, Van Bulck L, Luyckx K, et al., 2024, Frailty and Cognitive Function in Middle-Aged and Older Adults With Congenital Heart Disease., J Am Coll Cardiol, Vol: 83, Pages: 1149-1159
BACKGROUND: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential. OBJECTIVES: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits. METHODS: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment. RESULTS: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income. CONCLUSIONS: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.
Constantine A, Krishnathasan K, Dimopoulos K, 2024, Understanding the interface between physical and mental health in adults with congenital heart disease., Eur J Prev Cardiol
Constantine A, Ferrero P, Gribaudo E, et al., 2024, Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life, European Journal of Preventive Cardiology, ISSN: 2047-4873
Aims:To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking.Methods and results:Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35–48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0–15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4–10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04–1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75–10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23–7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05–70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis.Conclusion:Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for trans
Surkova E, Constantine A, Xu Z, et al., 2024, Prognostic significance of subpulmonary left ventricular size and function in patients with a systemic right ventricle, European Heart Journal - Cardiovascular Imaging, Vol: 25, Pages: 58-65, 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.
Wander G, Breviario S, Krishnathasan K, et al., 2023, Multifaceted Fontan patients and their response to pregnancy, JACC: Case Reports, Vol: 28, ISSN: 2666-0849
We present four patients with Fontan circulation who underwent successful pregnancies, albeit with complications that required close monitoring and timely intervention. Each Fontan patient presents with a unique clinical picture making risk stratification challenging but all the more important.
Constantine A, Dimopoulos K, Gerges C, et al., 2023, Peripheral pulmonary artery stenosis in adults: a novel type of pulmonary vascular disease with a strong genetic background, European Respiratory Journal, Vol: 62, ISSN: 0903-1936
Group 4 pulmonary hypertension (PH) includes several conditions associated with obstructions in the pulmonary arterial tree. Chronic thromboembolic PH (CTEPH), the most common type of group 4 PH in adults, has attracted the most attention to date. In this condition, the rise in pulmonary artery pressure results from a combination of the obstruction to flow caused by organised thrombus and pulmonary arterial hypertension (PAH)-like microvascular disease, which develops as a result of excessive blood flow and shear stress in unobstructed areas of the lung. For this reason, CTEPH is typically managed both through mechanical relief of the obstruction (pulmonary endarterectomy and/or balloon pulmonary angioplasty; BPA) and PAH therapies. International guidelines have only relatively recently recognised conditions other than CTEPH that may cause PH associated with pulmonary artery obstruction [1, 2]: congenital pulmonary arterial stenosis has been classified under subgroup 4.2. However, there is still limited understanding about the prevalence, pathophysiology and prognosis of peripheral pulmonary artery stenosis in adults.
Ganni E, Ho SY, Reddy S, et al., 2023, Tetralogy of Fallot Across the Lifespan: A Focus on the Right Ventricle., CJC Pediatr Congenit Heart Dis, Vol: 2, Pages: 283-300
Tetralogy of Fallot is a cyanotic congenital heart disease, for which various surgical techniques allow patients to survive to adulthood. Currently, the natural history of corrected tetralogy of Fallot is underlined by progressive right ventricular (RV) failure due to pulmonic regurgitation and other residual lesions. The underlying cellular mechanisms that lead to RV failure from chronic volume overload are characterized by microvascular and mitochondrial dysfunction through various regulatory molecules. On a clinical level, these cardiac alterations are commonly manifested as exercise intolerance. The degree of exercise intolerance can be objectified and aid in prognostication through cardiopulmonary exercise testing. The timing for reintervention on residual lesions contributing to RV volume overload remains controversial; however, interval assessment of cardiac function and volumes by echocardiography and magnetic resonance imaging may be helpful. In patients who develop clinically important RV failure, clinicians should aim to maintain a euvolemic state through the use of diuretics while paying particular attention to preload and kidney function. In patients who develop signs of cardiogenic shock from right heart failure, stabilization through the use of inotropes and pressor is indicated. In special circumstances, the use of mechanical support may be appropriate. However, cardiologists should pay particular attention to residual lesions that may impact the efficacy of the selected device.
Constantine A, Al-Sakini N, Dimopoulos K, 2023, Improved prognostication using the inferior vena cava collapsibility index: A tool validated for adults with congenital heart disease, International Journal of Cardiology, Pages: 131651-131651, ISSN: 0167-5273
Ferrero P, Krishnathasan K, Constantine A, et al., 2023, Pulmonary arterial hypertension in congenital heart disease, Heart, ISSN: 1355-6037
Clift P, Dimopoulos K, Angelini A, 2023, Univentricular Congenital Heart Defects and the Fontan Circulation Practical Manual for Patient Management, Publisher: Springer, ISBN: 9783031362071
This book provides a concise, practically applicable guide to the management of patients born with a univentricular heart defect.
Mavromanoli AC, Barco S, Ageno W, et al., 2023, Recovery of right ventricular function after intermediate-risk pulmonary embolism: results from the multicentre Pulmonary Embolism International Trial (PEITHO)-2, CLINICAL RESEARCH IN CARDIOLOGY, Vol: 112, Pages: 1372-1381, ISSN: 1861-0684
Barradas-Pires A, Merás P, Constantine A, et al., 2023, Repair of aortic regurgitation in young adults: sooner rather than later, Journal of the American Heart Association, Vol: 12, Pages: 1-11, 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
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, Vol: 30, Pages: 1335-1342, 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.
Seitler S, Dimopoulos K, Ernst S, et al., 2023, Medical Emergencies in Pulmonary Hypertension, SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE, ISSN: 1069-3424
Chivers S, Cleary A, Knowles R, et al., 2023, COVID-19 in congenital heart disease (COaCHeD) study, OPEN HEART, Vol: 10, ISSN: 2053-3624
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 REVIEW OF RESPIRATORY MEDICINE, Vol: 17, Pages: 413-423, ISSN: 1747-6348
Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al., 2023, Eisenmenger Syndrome: <i>JACC</i> State-of-the-Art Review (vol 79, pg 1183, 2022), JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 81, Pages: 1746-1746, ISSN: 0735-1097
Xu Z, Constantine A, Dimopoulos K, et al., 2023, DIFFERENTIAL BIVENTRICULAR ADAPTION TO PULMONARY ARTERIAL HYPERTENSION RELATED TO IDIOPATHIC/HERITABLE VERSUS CONGENITAL HEART DISEASE: A PROSPECTIVE CMR AND INVASIVE STUDY, 72nd Annual Scientific Session (ACC), Publisher: ELSEVIER SCIENCE INC, Pages: 1895-1895, ISSN: 0735-1097
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.
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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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
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