Takotsubo syndrome is a condition characterised by temporary acute left ventricular dysfunction with regional wall abnormalities extending beyond a single coronary artery territory. Initially thought to be benign, this condition, which is challenging to distinguish from acute coronary syndrome, has substantial morbidity and mortality. The mechanism behind this condition remains elusive, but multiple theories have been proposed. Although beta blockers and angiotensin-converting enzyme inhibitors are used as treatments for left ventricular dysfunction, currently, there are no randomised controlled trials to support their use. In this paper, we review the latest evidence regarding aetiologies, pathophysiology, diagnostic criteria, prognosis, complications and management of Takotsubo syndrome.

UNASSIGNED: Selected patients with advanced heart failure ineligible for heart transplantation could benefit from left ventricular assist device therapy as \'destination therapy\'. There is evidence of the efficacy of destination therapy; however, it is not currently commissioned within the United Kingdom National Health Service due to the lack of economic evidence.
UNASSIGNED: What is the clinical and cost-effectiveness of a left ventricular assist device compared to medical management for patients with advanced heart failure ineligible for heart transplantation (destination therapy)?
UNASSIGNED: A systematic review of evidence on the clinical and cost-effectiveness of left ventricular assist devices as destination therapy was undertaken including, where feasible, a network meta-analysis to provide an indirect estimate of the relative effectiveness of currently available left ventricular assist devices compared to medical management. For the systematic reviews, data sources searched (up to 11 January 2022) were Cochrane CENTRAL, MEDLINE and EMBASE via Ovid for primary studies, and Epistemonikos and Cochrane Database of Systematic Reviews for relevant systematic reviews. Trial registers were also searched, along with data and reports from intervention-specific registries. Economic studies were identified in EconLit, CEA registry and the NHS Economic Evaluation Database (NHS EED). The searches were supplemented by checking reference lists of included studies. An economic model (Markov) was developed to estimate the cost-effectiveness of left ventricular assist devices compared to medical management from the United Kingdom National Health Service/personal social service perspective. Deterministic and probabilistic sensitivity analyses were conducted to explore uncertainties. Where possible, all analyses focused on the only currently available left ventricular assist device (HeartMate 3TM, Abbott, Chicago, IL, USA) in the United Kingdom.
UNASSIGNED: The clinical effectiveness review included 134 studies (240 articles). There were no studies directly comparing HeartMate 3 and medical management (a randomised trial is ongoing). The currently available left ventricular assist device improves patient survival and reduces stroke rates and complications compared to earlier devices and relative to medical management. For example, survival at 24 months is 77% with the HeartMate 3 device compared to 59% with the HeartMate II (MOMENTUM 3 trial). An indirect comparison demonstrated a reduction in mortality compared to medical management [relative risk of death 0.25 (95% confidence interval 0.13 to 0.47); 24 months; this study]. The cost-effectiveness review included 5 cost analyses and 14 economic evaluations covering different generations of devices and with different perspectives. The reported incremental costs per quality-adjusted life-year gained compared to medical management were lower for later generations of devices [as low as £46,207 (2019 prices; United Kingdom perspective; time horizon at least 5 years)]. The economic evaluation used different approaches to obtain the relative effects of current left ventricular assist devices compared to medical management from the United Kingdom National Health Service/personal social service perspective. All gave similar incremental cost-effectiveness ratios of £53,496-58,244 per quality-adjusted life-year gained - lifetime horizon. Model outputs were sensitive to parameter estimates relating to medical management. The findings did not materially differ on exploratory subgroup analyses based on the severity of heart failure.
UNASSIGNED: There was no direct evidence comparing the clinical effectiveness of HeartMate 3 to medical management. Indirect comparisons made were based on limited data from heterogeneous studies regarding the severity of heart failure (Interagency Registry for Mechanically Assisted Circulatory Support score distribution) and possible for survival only. Furthermore, the cost of medical management of advanced heart failure in the United Kingdom is not clear.
UNASSIGNED: Using cost-effectiveness criteria applied in the United Kingdom, left ventricular assist devices compared to medical management for patients with advanced heart failure ineligible for heart transplant may not be cost-effective. When available, data from the ongoing evaluation of HeartMate 3 compared to medical management can be used to update cost-effectiveness estimates. An audit of the costs of medical management in the United Kingdom is required to further decrease uncertainty in the economic evaluation.
UNASSIGNED: This study is registered as PROSPERO CRD42020158987.
UNASSIGNED: This award was funded by the National Institute for Health and Care Research (NIHR) Health Technology Assessment programme (NIHR award ref: NIHR128996) and is published in full in Health Technology Assessment; Vol. 28, No. 38. See the NIHR Funding and Awards website for further award information.
The majority of patients with advanced heart failure would be unsuitable for heart transplantation due to their age and comorbidities but selected patients could benefit from a left ventricular assist device. Left ventricular assist device therapy for such patients is known as ‘destination therapy’. This is a long-term therapy that involves implanting a battery-powered pump to support the patient’s heart. The purpose of this project was to collect and assess the research evidence on the effectiveness of left ventricular assist devices when used for destination therapy, and to estimate value for money compared to medical management from the United Kingdom National Health Service/personal social service perspective. This research identified that the currently available left ventricular assist device improves patient survival as well as reducing stroke rates and complications compared to earlier devices and relative to medical management. However, there is uncertainty in the evidence due to the absence of studies directly comparing the current device to medical therapy alone. An ongoing clinical trial is currently assessing this. It also means there is uncertainty about whether left ventricular assist devices could provide value for money as determined currently for the United Kingdom National Health Service.

UNASSIGNED: LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON-related myopathy (SELENON-RM) are two rare neuromuscular diseases characterized by proximal and axial muscle weakness, scoliosis, spinal rigidity, low bone quality and respiratory impairment. Cardiac involvement has previously been described in retrospective studies and case reports, but large case series and prospective studies in unselected cohorts are lacking.
UNASSIGNED: The objective of this study is to conduct prevalence estimations, perform cardiac phenotyping, and provide recommendations for clinical care.
UNASSIGNED: In this case series including two time points, we conducted comprehensive assessments with electrocardiography (ECG) and transthoracic echocardiography (TTE). ECGs were systematically assessed for a large subset of variables. TTE included left and right ventricular ejection fraction (LVEF/RVEF) and left ventricular global longitudinal strain (GLS), the latter being a more early and sensitive marker of left ventricular dysfunction.
UNASSIGNED: 21 LAMA2-MD (M = 5; 20±14 years) and 10 SELENON-RM patients (M = 7; 18±12 years) were included. In most patients, QRS fragmentation and Q waves, markers of heterogeneous ventricular activation, were present both at baseline and at follow-up. GLS was abnormal (age specific in children, > -18% in adults) in 33% of LAMA2-MD and 43% of SELENON-RM patients at baseline. Reduced LVEF (<52% in males, <54% in females and <55% in pediatric population) was observed in three LAMA2-MD patients at baseline and in none of the SELENON-RM patients. GLS and LVEF did not change between baseline and follow-up. RVEF was normal in all patients.
UNASSIGNED: ECG abnormalities and abnormal GLS are prevalent in LAMA2-MD and SELENON-RM, yet abnormal LVEF was only seen in LAMA2-MD patients. One LAMA2-MD patient had a clinically relevant deterioration in LVEF during 1.5-year follow-up. We advise routine screening of all patients with LAMA2-MD or SELENON-RM with ECG and echocardiography at diagnosis, minimally every two years from second decade of life and if new cardiac signs arise.

BACKGROUND: Right ventricular dysfunction is associated with mortality in patients with acute respiratory distress syndrome (ARDS) but information in veno-venous extracorporeal membrane oxygenation (ECMO) settings is limited. Study objectives were to examine factors associated with right ventricular (RV) systolic dysfunction (RVSD) and RV dilation in ECMO patients with ARDS, to compare outcomes in those with and without RVSD and RV dilation defined by qualitative and quantitative parameters, and to describe RVSD evolution during ECMO.
METHODS: Retrospective observational study of adult ARDS patients supported with ECMO at a tertiary care hospital.
RESULTS: Of a total of 62 patients, 56% had RVSD and 61% had RV dilation by qualitative assessment. Male gender, COVID-19, hypercarbia, and pneumothorax were associated with RVSD and RV dilation. In-hospital mortality was significantly higher in patients with RV dilation vs. no dilation (42% vs. 17%, p = .05) but comparisons for patients with and without RVSD (37% vs. 26%, respectively) did not reach statistical significance. Findings were similar when RV size and function were quantified by right to left ventricle end-diastolic area ratio and fractional area change (39% vs. 21% and 36% vs. 20% respectively; p = NS). Of 39 patients with multiple echocardiograms, 9 of 18 with initially normal RV function developed RVSD while RV function normalized in 10 of 21 patients who began ECMO with RVSD.
CONCLUSIONS: Study results suggest an association of RV dilation and RVSD with worse outcomes and a dynamic nature of RV function necessitating close monitoring during the ECMO course.

BACKGROUND: Hypertension is the most common reversible cause of cardiovascular disease worldwide and more than one billion individuals suffer from the disease. Constant heart exposure to increased afterload progresses to maladaptive remodeling, leading to cardiac dysfunction. In this study, we aimed to evaluate cardiac function in response to hypertension treatment.
METHODS: One hundred patients diagnosed with hypertension were evaluated two times, with 3 to 6 months intervals, before and after antihypertensive therapy. Patients underwent clinical and echocardiographic evaluation in both visits and the interest effect of antihypertensive therapy on cardiac function was studied.
RESULTS: 58 men and 42 women with a mean age of 60.81 ± 11.8 years were studied. Mean systolic and diastolic pressure in the first visit was 163.05 ± 20.6 and 95.40 ± 10.4, respectively. On the second visit, mean systolic and diastolic pressure was 129.95 ± 10.4 and 82.35 ± 7.2 respectively (P value for both < 0.001). The mean value of Global Longitudinal Strain as the main parameter for evaluating left ventricular systolic function was -15.54% on the first visit and changed to -16.95% on the second visit (P value 0.025).
CONCLUSIONS: According to the results of this study, changes in parameters, indicator of systolic and diastolic function, after 3-6 months of antihypertensive therapy are significant. The most important point is that maladaptive remodeling of the heart is reversible if hypertension is diagnosed timely. To follow-up patients under antihypertensive therapy, GLS and parameters indicator of diastolic dysfunction, have the best diagnostic value in terms of detecting early stages of cardiac injury.

UNASSIGNED: To determine the factors associated with left ventricular diastolic dysfunction (LVDD) in adults residing in a region of the Andes in Peru.
UNASSIGNED: A case-control study was conducted on adults living at an altitude of more than 3000 meters in Peru. Cases consisted of patients diagnosed with LVDD through echocardiography, whereas controls were adults without LVDD, as confirmed by echocardiography.
UNASSIGNED: A total of 50 cases and 100 controls were included in the study. Among them, 38.7% had high blood pressure, and 41.3% were overweight. Upon adjusted analysis, age 60 or older (aOR: 4.06; 95%CI: 1.29-12.8), female sex (aOR: 2.24; 95%CI: 1.01-4.96) and left ventricular hypertrophy (aOR: 3.17; 95%CI: 1.41-7.17) were identified as statistically significant factors associated with LVDD.
UNASSIGNED: The risk of LVDD is associated with older adults, female gender, and left ventricular hypertrophy among individuals residing above 3000 meters altitude in a region of the Andes, in Peru.
UNASSIGNED: Determinar los factores asociados con la disfunción diastólica del ventrículo izquierdo (DDVI) en adultos de una región de los Andes, en Perú.
UNASSIGNED: Estudio de casos y controles en adultos residentes a más de 3000 metros de altitud en Perú. Los casos fueron pacientes adultos diagnosticados con DDVI por ecocardiografía, y los controles fueron adultos sin DDVI por ecocardiografía.
UNASSIGNED: Se incluyeron 50 casos y 100 controles. El 38.7% tuvieron hipertensión arterial y el 41.3% sobrepeso. En el análisis ajustado, la edad de 60 o más años (ORa: 4.06; IC95%: 1.29-12.8), el sexo femenino (ORa: 2.24; IC95%: 1.01-4.96) y la hipertrofia ventricular izquierda (ORa: 3.17; IC95%: 1.41-7.17) fueron factores estadísticamente significativos.
UNASSIGNED: El riesgo de DDVI estuvo asociado a los adultos mayores, las mujeres y los pacientes con hipertrofia ventricular izquierda que viven por encima de los 3000 metros de altitud en una región de los Andes, en Perú.

UNASSIGNED: Systemic right ventricle (RV) dysfunction is associated with lower transplant-free survival (TFS) in hypoplastic left heart syndrome (HLHS), but the likelihood of functional improvement and utility of heart failure (HF) medications is not understood.
UNASSIGNED: The authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function.
UNASSIGNED: The SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days. We compared TFS, HF medications, and surgical interventions in HLHS patients with and without functional normalization.
UNASSIGNED: Of 99 patients with HLHS and RV dysfunction, 52% had normalized function for ≥30 days. TFS at 2 years after dysfunction onset was lower in those without normalization (14% vs 78%, P < 0.001). Patients without normalization were less likely to reach target dosing (TD) of HF medications (27% vs 47% on 1 medication at TD, P < 0.001) and undergo Fontan completion (7% vs 53%, P < 0.001). Clinical factors associated with improved TFS were normalization of function for ≥30 days, onset of dysfunction after bidirectional Glenn, and exposure to ACE inhibition.
UNASSIGNED: Our cohort of HLHS patients with systemic RV dysfunction demonstrated a novel finding of improved TFS in those with functional normalization for ≥30 days. Achieving TD of HF medications was associated with improved outcomes. This may reflect patient stability and tolerance for HF medication more than its therapeutic effect, but it can help inform decisions to proceed with surgical palliation or list for transplant.

UNASSIGNED: Although severe acute respiratory failure is the primary cause of morbidity and mortality in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, this viral infection leads to cardiovascular disease in some individuals. Cardiac effects of the virus include myocarditis, pericarditis, arrhythmias, coronary aneurysms and cardiomyopathy, and can result in cardiogenic shock and multisystem organ failure.
UNASSIGNED: This review summarises cardiac manifesta-tions of SARS-CoV-2 in the paediatric population. We performed a scoping review of cardiovascular disease associated with acute coronavirus disease 2019 (COVID-19) infection, multisystem inflammatory syndrome in children (MIS-C), and mRNA COVID-19 vaccines. Also examined are special considerations for paediatric athletes and return to play following COVID-19 infection.
UNASSIGNED: Children presenting with acute COVID-19 should be screened for cardiac dysfunction and a thorough history should be obtained. Further cardiovascular evaluation should be considered following any signs/symptoms of arrhythmias, low cardiac output, and/or myopericarditis. Patients admitted with severe acute COVID-19 should be monitored with continuous cardiac monitoring. Laboratory testing, as clinically indicated, includes tests for troponin and B-type natriuretic peptide or N-terminal pro-brain natriuretic peptide. Echocardiography with strain evaluation and/or cardiac magnetic resonance imaging should be considered to evaluate diastolic and systolic dysfunction, coronary anatomy, the pericardium and the myocardium. For patients with MIS-C, combination therapy with intravenous immunoglobulin and glucocorticoid therapy is safe and potentially disease altering. Treatment of MIS-C targets the hyperimmune response. Supportive care, including mechanical support, is needed in some cases.
UNASSIGNED: Cardiovascular disease is a striking feature of SARS-CoV-2 infection. Most infants, children and adolescents with COVID-19 cardiac disease fully recover with no lasting cardiac dysfunction. However, long-term studies and further research are needed to assess cardiovascular risk with variants of SARS-CoV-2 and to understand the pathophysiology of cardiac dysfunction with COVID-19.

With continued medical and surgical advancements, most children and adolescents with congenital heart disease are expected to survive to adulthood. Chronic heart failure is increasingly being recognized as a major contributor to ongoing morbidity and mortality in this population as it ages, and treatment strategies to prevent and treat heart failure in the pediatric population are needed. In addition to primary myocardial dysfunction, anatomical and pathophysiological abnormalities specific to various congenital heart disease lesions contribute to the development of heart failure and affect potential strategies commonly used to treat adult patients with heart failure. This scientific statement highlights the significant knowledge gaps in understanding the epidemiology, pathophysiology, staging, and outcomes of chronic heart failure in children and adolescents with congenital heart disease not amenable to catheter-based or surgical interventions. Efforts to harmonize the definitions, staging, follow-up, and approach to heart failure in children with congenital heart disease are critical to enable the conduct of rigorous scientific studies to advance our understanding of the actual burden of heart failure in this population and to allow the development of evidence-based heart failure therapies that can improve outcomes for this high-risk cohort.

OBJECTIVE: Twins resulting from a complicated monochorionic (MC) twin pregnancy are at risk for postnatal evolution of pulmonary hypertension (PH) and cardiac dysfunction (CD). Both pathologies are important contributors to short- and long-term morbidity in these infants. The aim of the present retrospective single-center cohort study was to evaluate the need for vasoactive treatment for PH and CD in these neonates.
METHODS: In-born neonates following a complicated MC twin pregnancy admitted to the department of neonatology of the University Children\'s Hospital Bonn (UKB) between October 2019 and December 2023 were screened for study inclusion. Finally, 70 neonates were included in the final analysis, with 37 neonates subclassified as recipient twins (group A) and 33 neonates as donor twins (group B).
RESULTS: The overall PH incidence at day of life (DOL) 1 was 17% and decreased to 6% at DOL 7 (p = 0.013), with no PH findings at DOL 28. The overall incidence of CD was 56% at DOL 1 and decreased strongly until DOL 7 (10%, p = 0.015), with no diagnosis of CD at DOL 28. The use of dobutamine, norepinephrine, and vasopressin at DOL 1 until DOL 7 did not differ between the subgroups, whereas the dosing of milrinone was significantly higher in Group B at DOL 1 (p = 0.043). Inhaled nitric oxide (iNO) was used in 16% of the cohort, and a levosimendan therapy was administered in 34% of the neonates. One-third of the cohort was treated with oral beta blockers, and in 10%, an intravenous beta blockade (landiolol) was administered. The maximum levosimendan vasoactive-inotropic score (LVISmax) increased from DOL 1 (12.4 [3/27]) to DOL 2 (14.6 [1/68], p = 0.777), with a significant decrease thereafter as measured at DOL 7 (9.5 [2/30], p = 0.011).
CONCLUSIONS: Early PH and CD are frequent diagnoses in neonates following a complicated MC twin pregnancy, and an individualized vasoactive treatment strategy is required in the management of these infants.