458 results found
Constantine A, Condliffe R, Clift P, et al., 2021, Cardiac disease in Down Syndrome: literature review and international expert consensus in collaboration with Down Syndrome International (DSi), Journal of Congenital Cardiology, Vol: 5, Pages: 1-3, ISSN: 2056-7251
BackgroundCongenital heart disease is common in patients with Down syndrome, yet clinical recommendations relating to its diagnosis and management in this patient group are lacking.Main bodyWe discuss the ongoing collaboration between an international panel of cardiovascular experts and expert stakeholders from Down Syndrome International, an international disabled people's organisation with membership of organisations and individuals from 136 countries worldwide. The aim of this collaboration is to describe best clinical practice, focusing on 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to the care of patients in areas of differing resource availability.ConclusionsThe planned expert consensus statement on cardiac disease in people with Down syndrome aims to foster communication between experts, direct future research and inform future practice guidelines for the diagnosis and management of cardiovascular disease in people with Down syndrome.
Constantine A, Dimopoulos K, 2021, Integrating patient-reported outcome measures in congenital heart disease care, European Heart Journal - Quality of Care and Clinical Outcomes, Vol: 7, Pages: 325-326, ISSN: 2058-5225
Wustmann K, Constantine A, Davies JE, et al., 2021, Prognostic implications of pulmonary wave reflection and reservoir pressure in patients with pulmonary hypertension, International Journal of Cardiology: Congenital Heart Disease, Vol: 5, Pages: 1-8, ISSN: 2666-6685
BackgroundRight ventricular (RV) coupling to the pulmonary circulation influences the response of the RV to the increased afterload caused by pulmonary hypertension (PH), which ultimately determines prognosis. A methodology that accounts for pulsatile flow is required when assessing ventriculo-arterial coupling. We applied wave intensity analysis (WIA) methods to assess the compliance of the main pulmonary artery (PA) in patients with or without PH and compared this to PA distensibility, RV function and clinical outcomes.MethodsHigh-fidelity blood pressure and Doppler flow velocity tracings were obtained simultaneously during cardiac catheterisation for suspected PH. RV volumes, main PA distensibility and ventriculo-arterial coupling (Emax/Ea) were analysed using cardiovascular magnetic resonance.ResultsThe study included 17 PH patients and 6 controls. Wave speed, reservoir and excess pressure were higher in PH patients compared to controls (p < 0.01 for all). Waveforms relating to RV ejection, microvascular wave reflection and late systolic proximal deceleration were higher in PH patients compared to controls (p < 0.01 for all) and related to echocardiographic findings, including PA Doppler notching and shortened acceleration time. Wave speed, reservoir pressure and excess pressure were strongly correlated to main PA distensibility, RV function and Emax/Ea. A higher total pressure integral was associated with an increased risk of death (all-cause mortality).ConclusionThe reservoir-excess pressure model, in combination with conventional clinical imaging, provides valuable information on the pathophysiology of PH that standard haemodynamic parameters do not. Future studies should further investigate the prognostic implications of WIA in PH, and its potential role in clinical practice.
Constantine A, Barradas-Pires A, Dimopoulos K, 2021, Cardiopulmonary exercise testing in congenital heart disease: Towards serial testing as part of long-term follow-up, European Journal of Preventive Cardiology, ISSN: 2047-4873
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
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
Khan U, Shaw T, Kempny A, et al., 2021, The clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond., Int J Cardiol
BACKGROUND: Aortic coarctation (AoCoa) is a congenital stenosis of aorta, which often co-exists with other congenital heart defects. Many studies have shown the importance of regular follow-up in these patients however there is scarcity of knowledge relating to the impact of left ventricle inflow lesions (LVIT) and left ventricle outflow track lesions (LVOT). The aim of this study is to evaluate the impact of isolated AoCoa with LVIT and/or LVOT on haemodynamic, morbidity and mortality. METHODS: We have retrospectively analysed clinical data of all adult AoCoa patients who underwent echocardiography between 2010-2018 in our centre. Outcome measures included death, number of hospitalisations for cardiac causes, development of cardiac arrhythmia, new prescription of HF medication. RESULTS: A total of 406 AoCoa patients were included and were followed for a median 4.2 years. At baseline, 38% patients had AoCoa alone, 54% patients had LVOT, 3% patients had LVIT, and 5% patients had mixed LVIT and LVOT, including patients with Shone syndrome. Patients with mixed LVIT and LVOT had the highest mortality of the four groups and the highest heart failure-related morbidity. Moreover, they were the most prone to have a higher indexed LA volume compared to patients with no LVOT (p=0.0001). During follow-up, 13 patients died, of which 21% patients were from the mixed LVIT and LVOT group. CONCLUSIONS: AoCoa patients with a combination of LVIT and LVOT including Shone complex are associated with a significantly higher morbidity and mortality compared to AoCoa alone.
Samaranayake CB, Warren C, Siewers K, et al., 2021, Impact of cyanosis on ventilatory responses during stair climb exercise in Eisenmenger syndrome and idiopathic pulmonary arterial hypertension., Int J Cardiol
Studies assessing exercise ventilatory responses during real-life exercise in pulmonary arterial hypertension (PAH) which include patients with cyanotic congenital heart disease are scarce. We assessed the ventilatory response to stairclimbing in patients with idiopathic PAH (IPAH) and congenital heart disease-associated PAH with Eisenmenger (EIS) physiology compared to healthy controls. Fifteen adults with IPAH, six EIS and 15 age and body mass index (BMI) matched controls were prospectively recruited. Participants completed spirometry and a self-paced stair-climb (48 steps) with portable cardiopulmonary exercise testing (CPET) equipment in-situ. Borg dyspnoea scores were measured at rest and on stair-climb cessation. Both IPAH and EIS groups had amplified ventilatory responses compared to Controls. The rate of increase in minute ventilation (VE) was exaggerated in EIS driven by an early increase in tidal volume (Tv) and more gradual increase in respiratory rate (RR). Peak Tv, RR, Tv: forced vital capacity (FVC) ratio, VE/VCO2 slope and stairclimb duration were significantly higher in EIS and IPAH compared to controls despite similar baseline spirometry and change in oxygen uptake on exercise. A decline in end-tidal carbon dioxide (CO2) and arterial oxygen saturations in early exercise distinguished EIS and IPAH patients. Significant correlations were observed between peak exercise Borg score and stair-climb time (r = 0.73, p = 0.002), peak end-tidal CO2 (r = -0.73, p = 0.001), peak VE (r = 0.53, p = 0.008), peak RR (r = 0.42, p = 0.011) and VE/VCO2 slope (r = 0.54, p = 0.001). Patients with IPAH and EIS have exaggerated ventilatory responses to stair-climbing compared to the controls with more severe levels of dyspnoea perception in Eisenmenger syndrome for equivalent oxygen uptake and work.
Egidy Assenza G, Dimopoulos K, Budts W, et al., 2021, Management of acute cardiovascular complications in pregnancy., Eur Heart J
The growing population of women with heart disease of reproductive age has been associated with an increasing number of high-risk pregnancies. Pregnant women with heart disease are a very heterogeneous population, with different risks for maternal cardiovascular, obstetric, and foetal complications. Adverse cardiovascular events during pregnancy pose significant clinical challenges, with uncertainties regarding diagnostic and therapeutic approaches potentially compromising maternal and foetal health. This review summarizes best practice for the treatment of common cardiovascular complications during pregnancy, based on expert opinion, current guidelines, and available evidence. Topics covered include heart failure (HF), arrhythmias, coronary artery disease, aortic and thromboembolic events, and the management of mechanical heart valves during pregnancy. Cardiovascular pathology is the leading cause of non-obstetric morbidity and mortality during pregnancy in developed countries. For women with pre-existing cardiac conditions, preconception counselling and structured follow-up during pregnancy are important measures for reducing the risk of acute cardiovascular complications during gestation and at the time of delivery. However, many women do not receive pre-pregnancy counselling often due to gaps in what should be lifelong care, and physicians are increasingly encountering pregnant women who present acutely with cardiac complications, including HF, arrhythmias, aortic events, coronary syndromes, and bleeding or thrombotic events. This review provides a summary of recommendations on the management of acute cardiovascular complication during pregnancy, based on available literature and expert opinion. This article covers the diagnosis, risk stratification, and therapy and is organized according to the clinical presentation and the type of complication, providing a reference for the practicing cardiologist, obstetrician, and acute medicine specialist, while highlightin
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
D'Alto M, Constantine A, Chessa M, et al., 2021, Fluid challenge and balloon occlusion testing in patients with atrial septal defects, Heart, ISSN: 1355-6037
IntroductionCareful, stepwise assessment is required in all atrial septal defect (ASD) patients to exclude pulmonary vascular or left ventricular (LV) disease. Fluid challenge and balloon occlusion may unmask LV disease and post-capillary pulmonary hypertension, but their role in the evaluation of patients with ‘operable’ ASDs is not well established. MethodsWe conducted a prospective study in 3 Italian specialist centres between 2018 and 2020. Patients selected for percutaneous ASD closure underwent assessment at baseline and after fluid challenge, balloon occlusion and both. ResultsFifty patients (46[38.2,57.8] years, 72% female) were included. All had a shunt fraction >1.5, pulmonary vascular resistance (PVR) <5 WU and pulmonary arterial wedge pressure (PAWP) <15 mmHg. Individuals with a PVR ≥2 WU at baseline (higher PVR group) were older, more symptomatic, with a higher baseline systemic vascular resistance (SVR) than the lower PVR group (all p<0.0001). Individuals with a higher PVR experienced smaller increases in pulmonary blood flow following fluid challenge (0.3[-0.1,0.5] vs. 2.0[1.5,2.8] L/min, p<0.0001). Balloon occlusion led to a more marked fall in SVR (p<0.0001) and a larger increase in systemic blood flow (p=0.024) in the higher PVR group. No difference was observed in PAWP following fluid challenge and/or balloon occlusion between groups; 4(8%) patients reached a PAWP≥18 mmHg following the addition of fluid challenge to balloon occlusion testing.ConclusionsIn adults with ASD without overt LV disease, even small rises in PVR may have significant implications on cardiovascular haemodynamics. Fluid challenge may provide additional information to balloon occlusion in this setting.
Jansen K, Constantine A, Condliffe R, et al., 2021, Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation, Expert Review of Cardiovascular Therapy, ISSN: 1477-9072
Introduction:Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease. Areas covered:In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD. The indications, timing and the content of advanced heart failure assessment and transplant listing are discussed, along with a review of the types of transplant and other forms of available circulatory support in this group of patients. Finally, the integral role of advance care planning and palliative care is discussed. Expert opinion/commentary:All patients with PAH-CHD should be followed up in expert centers, where they can receive appropriate risk assessment, PAH therapy and supportive care. Referral for transplant assessment should be considered if there continue to be clinical high-risk features, persistent symptoms or acute heart failure decompensation despite appropriate PAH specific therapy. Expert management of PAH-CHD patients, therefore, requires vigilance for these features, along with a close relationship with local advanced heart failure services and a working knowledge of listing criteria, which may disadvantage congenital heart disease patients.
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.
Ghonim S, Gatzoulis M, Ernst S, et al., 2021, Predicting survival in repaired tetralogy of Fallot- a lesion specific and personalised approach, JACC: Cardiovascular Imaging, 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.
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.
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.
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?", Int J Cardiol, Vol: 332
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
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.
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
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
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
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.
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
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
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
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
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