Left Atrial Myopathy is Associated With Exercise Incapacity and Ventilatory Inefficiency in Hypertrophic Cardiomyopathy.

BACKGROUND: Left atrial (LA) myopathy is an established component of hypertrophic cardiomyopathy (HCM); however, the data about its association with exercise incapacity or ventilatory inefficiency that may be seen in HCM patients are limited. This study aimed to explore the association between LA myopathy, evaluated by echocardiography LA strain, and exercise capacity and ventilatory efficiency, evaluated by cardiopulmonary exercise testing (CPET), in HCM patients. METHODS: This study included 241 consecutive HCM patients (aged 51.2±15.7 years 67.2% male) in sinus rhythm who underwent CPET and transthoracic echocardiography at the same visit. Exercise incapacity (maximal/predicted oxygen consumption [%peakVO2] <80%) and ventilatory inefficiency (ventilation/carbon dioxide output [VE/VCO2] slope >34) were assessed by CPET. Left atrial myopathy was examined by speckle-tracking myocardial deformation parameters: LA reservoir, conduit and booster strain. RESULTS: All three LA strain values were univariate predictors of exercise capacity and ventilatory efficiency. Among them, LA reservoir strain had the higher r correlation coefficient for predicting both %peakVO2 and VE/VCO2 slope. Left atrial reservoir strain, presence of angina and family history of HCM were independent predictors of exercise capacity. Left atrial reservoir strain, male gender and non-sustained ventricular tachycardia were independent predictors of ventilatory efficiency. Left atrial reservoir strain was a significant predictor of %peakVO2<80% with an optimal cut-off value of 27% (sensitivity 87% and specificity 31%) and VE/VCO2>34 with an optimal cut-off value of 18% (sensitivity 71% and specificity 83%). CONCLUSION: Left atrial myopathy, as reflected by the LA strain values, was associated with exercise incapacity and ventilatory inefficiency in HCM individuals. Left atrial reservoir strain was the only common independent predictor of %peakVO2 and VE/VCO2 slope.

Mitral regurgitation impact on left atrial myopathy in hypertrophic cardiomyopathy.

BACKGROUND: Recent studies have shown that mitral regurgitation (MR) represents a major determinant of left atrial (LA) function in patients with heart failure with preserved ejection fraction. The role of MR in determining LA myopathy in hypertrophic cardiomyopathy (HCM) is unknown. The aim of this study was to examine the association of MR with LA myopathy, assessed by LA strain values in HCM patients. METHODS: In total 250 consecutive patients (mean age 51 ± 16 years, 67.2% male) with an established diagnosis of HCM and with sinus rhythm at index echocardiography evaluation were included. LA reservoir, conduit and booster strain were analyzed, besides LA size, left ventricular (LV) systolic and diastolic function. The predictors of LA strain values were identified with linear regression analysis. RESULTS: Significant (more than mild) MR was a significant univariate predictor of all the three LA strain values. In multivariate linear regression analysis, independent predictors of LA reservoir strain were more than mild MR (r = -.23), LV global longitudinal strain (r = -.49), LA volume index (r = -.27) and patient age (r = -.23). Significant MR was also an independent determinant of LA conduit (r = -.17) and booster strain (r = -.12). In patients with LA volume index < 34 ml/m2 more than mild MR was an independent predictor of LA reservoir (r = -.32) and conduit strain (r = -.27), but not LA booster strain. CONCLUSION: Significant MR is associated with LA myopathy independently of the LV diastolic and systolic function and LA size.

Contemporary Data on the Status and Medical Management of Acute Heart Failure.

PURPOSE OF REVIEW: Acute heart failure (AHF) is among the leading causes for unplanned hospital admission. Despite advancements in the management of chronic heart failure, the prognosis of AHF remains poor with high in-hospital mortality and increased rates of unfavorable post-discharge outcomes. With this review, we aim to summarize current data on AHF epidemiology, focus on the different patient profiles and classifications, and discuss management, including novel therapeutic options in this area. RECENT FINDINGS: There is significant heterogeneity among patients admitted for AHF in their baseline characteristics, heart failure (HF) aetiology and precipitating factors leading to decompensation. A novel classification scheme based on four distinct clinical scenarios has been included in the most recent ESC guidelines, in an effort to better risk stratify patients and guide treatment. Intravenous diuretics, vasodilators, and inotropes remain the cornerstone of management in the acute phase, and expansion of use of mechanical circulatory support has been noted in recent years. Meanwhile, many treatments that have proved their value in chronic heart failure demonstrate promising results in the setting of AHF and research in this field is currently ongoing. Acute heart failure remains a major health challenge with high in-hospital mortality and unfavorable post-discharge outcomes. Admission for acute HF represents a window of opportunity for patients to initiate appropriate treatment as soon as possible after stabilization. Future studies are needed to elucidate which patients will benefit the most by available therapies and define the optimal timing for treatment implementation.

Evaluation of mitral regurgitation by cardiac magnetic resonance and transthoracic echocardiography: a systematic review and meta-analysis.

Transthoracic echocardiography (TTE) and Cardiac Magnetic Resonance (CMR) have complementary roles in the severity grading of mitral regurgitation (MR). Our objective was to systematically review the correlation of MR severity as assessed by TTE and CMR. We searched MEDLINE and Cochrane Library for original series published between January 1st, 2000 and March 23rd, 2020. We used Cohen's kappa coefficient to measure agreement between modalities. We plotted a hierarchical summary receiver operator characteristic (HSROC) curve and estimated the area under the curve (AUC) to assess the concordance between the two imaging modalities for the detection of severe MR. We identified 858 studies, of which 65 underwent full-text assessment and 8 were included in the meta-analysis. A total of 718 patients were included (425 males, 59%) in the final analysis. There was significant heterogeneity in the methods used and considerable variation in kappa coefficient, ranging from 0.10 to 0.48. Seven out of eight studies provided the necessary data to plot HSROC curves and calculate the AUC. The AUC for detecting severe MR was 0.83 (95% CI 0.80 to 0.86), whereas the AUC for detecting moderate to severe MR was 0.83 (95% CI 0.79 to 0.86). The agreement between TTE and CMR in MR severity evaluation is modest across the entire spectrum of severity grading. However, when focusing on patients with at least moderate MR the concordance between TTE and CMR is very good. Further prospective studies comparing hard clinical endpoints based on the CMR and TTE assessment of MR severity are needed.

Atrial Fibrillation Is Associated with Cognitive Impairment, All-Cause Dementia, Vascular Dementia, and Alzheimer's Disease: a Systematic Review and Meta-Analysis.

BACKGROUND: Atrial fibrillation (AF) is a risk factor for cognitive impairment and dementia in patients with stroke history. However, the association between AF and cognitive impairment in broader populations is less clear. OBJECTIVE: To systematically review and quantitatively synthesize the existing evidence regarding the association of AF with cognitive impairment of any severity and etiology and dementia. METHODS: Medline, Scopus, and Cochrane Central were searched in order to identify studies investigating the association between AF and cognitive impairment (or dementia) cross-sectionally and longitudinally. Studies encompassing and analyzing exclusively patients with stroke history were excluded. A random-effects model meta-analysis was conducted. Potential sources of between-study heterogeneity were investigated via subgroup and meta-regression analyses. Sensitivity analyses including only studies reporting data on stroke-free patients, vascular dementia, and Alzheimer's disease were performed. RESULTS: In total, 43 studies were included. In the pooled analysis, AF was significantly associated with dementia (adjusted OR, 1.6; 95% CI, 1.3 to 2.1; I2, 31%) and the combined endpoint of cognitive impairment or dementia (pooled adjusted OR, 1.5; 95% CI, 1.4 to 1.8; I2, 34%). The results were significant, even when studies including only stroke-free patients were pooled together (unadjusted OR, 2.2; 95% CI, 1.4 to 3.5; I2, 96%), but the heterogeneity rates were high. AF was significantly associated with increased risk of both vascular (adjusted OR, 1.7; 95% CI, 1.2 to 2.3; I2, 43%) and Alzheimer's dementia (adjusted HR, 1.4; 95% CI, 1.2 to 1.6; I2, 42%). CONCLUSION: AF increases the risk of cognitive impairment, all-cause dementia, vascular dementia, and Alzheimer's disease. Future studies should employ interventions that may delay or even prevent cognitive decline in AF patients.

Association Between Atrial Fibrillation and Cognitive Impairment in Individuals With Prior Stroke: A Meta-Analysis and Meta-Regression Analysis.

Background and Purpose- Atrial fibrillation (AF) is the most common chronic arrhythmia. Dementia and cognitive impairment (CI) are major burdens to public health. The prevalence of all 3 entities is projected to increase due to population aging. Previous reports have linked AF with a higher risk of CI and dementia in patients without prior stroke. Stroke is known to increase the risk for dementia and CI. It is unclear if AF in patients with history of stroke can further increase the risk for dementia or CI. Our purpose was to evaluate the impact of AF on risk for dementia or CI among patients with history of stroke. Methods- Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines were followed. Pubmed, Scopus, and Cochrane central were searched. The outcomes of interest were dementia, CI, and the composite end point of dementia or CI. A random-effect model meta-analysis was performed. Meta-regression analysis was also performed. Publication bias was assessed with the Egger test and with funnel plots. Results- Fourteen studies and 14 360 patients (1363 with AF) were included in the meta-analysis. In the meta-analysis of adjusted odds ratio, AF was associated with increased risk of CI (odds ratio, 1.60 [95% CI, 1.20-2.14]), dementia (odds ratio, 3.11 [95% CI, 2.05-4.73]), and the composite end point of CI or dementia (odds ratio, 2.26 [95% CI, 1.61-3.19]). The heterogeneity for the composite end point of dementia or CI was moderate (adjusted analysis). The heterogeneity for the analysis of the end point of CI only was substantial in the unadjusted analysis and moderate in the adjusted analysis. The heterogeneity for the end point of dementia only was moderate in the unadjusted analysis and zero in the adjusted analysis. Conclusions- Our results indicate that an association between AF and CI or dementia is patients with prior strokes is possible given the persistent positive associations we noticed in the unadjusted and adjusted analyses. The heterogeneity levels limit the certainty of our findings.

Predictors of vertigo in the emergency department: The preved study.

BACKGROUND AND PURPOSE: Acute vertigo (sense of motion) can be the sole manifestation of a posterior circulation stroke, and often gets missed in the emergency department (ED). The studies for evaluation of central vertigo have focused on physical exam findings, which require expertise and may not be suitable for rapid triage by a nurse in ED or by paramedics. METHODS: This cross sectional study included retrospective chart review of patients 18 years of age and older who presented to the Adult ED with acute dizziness or vertigo during the calendar year 2017. All the patients with a diagnosis of central or peripheral vertigo were included in the final analysis. Sensitivity, specificity, Likelihood Ratio of positive result (LR (+)) and Likelihood Ratio of negative result (LR (-)) for central and peripheral vertigo were calculated for risk factors, symptoms and physical examination features. Chi-squared test and univariate logistic regression were used to evaluate statistical correlation and to calculate the prevalence odds ratio (POR). RESULTS: Two hundred and forty nine out of 505 (49.3%) patients presenting with dizziness had vertigo. Of these, 14 had central vertigo and 163 had peripheral vertigo. Statistically significant variables were: constant symptoms of vertigo (p 0.000- POR 8.7, 95% confidence interval (CI) 2.3-33.1), no change in symptoms with head movement (p 0.000- POR 10.2, 95% CI 3.0-35.4), dysmetria (p 0.000- POR 56.8, 95% CI 5.8-557.1), and unsteady gait (p 0.000- POR 13.3, 95% CI 3.3-54.3). The sensitivity and specificity to detect central vertigo were 100% and 66.4% respectively if the patient had either unsteady gait, constant symptoms, or no change in symptoms with head movement, [VAIN triad (Vertigo- Ataxia, Incessant, or Non-positional)]. CONCLUSIONS: We suggest that triage with VAIN triad can be used to design prospective studies to develop a triage algorithm for the detection of central vertigo in the ED.

Development and Validation of a Clinical Score for Cardiovascular Risk Stratification of Long-Term Childhood Cancer Survivors.

BACKGROUND: Long-term childhood cancer survivors (CCS) are at increased risk of adverse cardiovascular events; however, there is a paucity of risk-stratification tools to identify those at higher-than-normal risk. SUBJECTS, MATERIALS, AND METHODS: This was a population-based study using data from the Surveillance, Epidemiology, and End Results Program (1973-2013). Long-term CCS (age at diagnosis ≤19 years, survival ≥5 years) were followed up over a median time period of 12.3 (5-40.9) years. Independent predictors of cardiovascular mortality (CVM) were combined into a risk score, which was developed in a derivation set (n = 22,374), and validated in separate patient registries (n = 6,437). RESULTS: In the derivation registries, older age at diagnosis (≥10 years vs. reference group of 1-5 years), male sex, non-white race, a history of lymphoma, and a history of radiation were independently associated with an increased risk of CVM among long-term CCS (p < .05). A risk score derived from this model (Childhood and Adolescence Cancer Survivor CardioVascular score [CHACS-CV], range: 0-8) showed good discrimination for CVM (Harrell's C-index [95% confidence interval (CI)]: 0.73 [0.68-0.78], p < .001) and identified a high-risk group (CHACS-CV ≥6), with cumulative CVM incidence over 30 years of 6.0% (95% CI: 4.3%-8.1%) versus 2.6% (95% CI: 1.8%-3.7%), and 0.7% (95% CI: 0.5%-1.0%) in the mid- (CHACS-CV = 4-5) and low-risk groups (CHACS-CV ≤3), respectively (plog-rank < .001). In the validation set, the respective cumulative incidence rates were 4.7%, 3.1%, and 0.8% (plog-rank < .001). CONCLUSION: We propose a simple risk score that can be applied in everyday clinical practice to identify long-term CCS at increased cardiovascular risk, who may benefit from early cardiovascular screening, and risk-reduction strategies. IMPLICATIONS FOR PRACTICE: Childhood cancer survivors (CCS) are known to be at increased cardiovascular risk. Currently available prognostic tools focus on treatment-related adverse events and late development of congestive heart failure, but there is no prognostic model to date to estimate the risk of cardiovascular mortality among long-term CCS. A simple clinical tool is proposed for cardiovascular risk stratification of long-term CCS based on easily obtainable information from their medical history. This scoring system may be used as a first-line screening tool to assist health care providers in identifying those who may benefit from closer follow-up and enable timely deployment of preventive strategies.

Pregnancy associated plasma protein-A as a prognostic biomarker of all-cause mortality and cardiovascular events in patients presenting with chest pain: a systematic review.

AIM: Novel biomarkers have been proposed for identification of patients at greater risk of future adverse events among those presenting with chest pain. In this review, we aim to elucidate the ability of pregnancy associated plasma protein-A (PAPP-A) to predict mortality and other cardiovascular events in this patient population. METHODS: A literature search of the electronic databases Medline, Scopus, Cochrane Library and ClinicalTrials.gov was performed in order to identify studies investigating the utility of PAPP-A to predict mortality and adverse cardiovascular events in patients with chest pain. RESULTS: Eight studies met our inclusion criteria. Five of these studies pertained to patients with confirmed ischemic chest pain, while the rest included patients presenting with chest pain possibly due to acute coronary syndrome, irrespectively of the underlying cause. Although the results for long-term events were inconclusive in both groups of patients, higher PAPP-A concentrations were found to be a significant predictor of short-term adverse events in patients with confirmed ischemic chest pain. CONCLUSIONS: PAPP-A appears to be a potentially useful biomarker for short-term risk stratification of patients presenting with chest pain of ischemic origin. However, there is an eminent need for more standardized clinical studies investigating the prognostic value of this biomarker.

Cardiac magnetic resonance for ventricular arrhythmias: a systematic review and meta-analysis.

BACKGROUND: Cardiac magnetic resonance (CMR) allows comprehensive myocardial tissue characterisation, revealing areas of myocardial inflammation or fibrosis that may predispose to ventricular arrhythmias (VAs). With this study, we aimed to estimate the prevalence of structural heart disease (SHD) and decipher the prognostic implications of CMR in selected patients presenting with significant VAs. METHODS: Electronic databases were searched for studies enrolling adult patients that underwent CMR for diagnostic or prognostic purposes in the setting of significant VAs. A random effects model meta-analysis of proportions was performed to estimate the prevalence of SHD. HRs were pooled together in order to evaluate the prognostic value of CMR. RESULTS: The prevalence of SHD was reported in 18 studies. In all-comers with significant VAs, the pooled rate of SHD post-CMR evaluation was 39% (24% in the subgroup of premature ventricular contractions and/or non-sustained ventricular tachycardia vs 63% in the subgroup of more complex VAs). A change in diagnosis after use of CMR ranged from 21% to 66% with a pooled average of 35% (29%-41%). A non-ischaemic cardiomyopathy was the most frequently identified SHD (56%), followed by ischaemic heart disease (21%) and hypertrophic cardiomyopathy (5%). After pooling together data from six studies, we found that the presence of late gadolinium enhancement was associated with increased risk of major adverse outcomes in patients with significant VAs (pooled HR: 1.79; 95% CI 1.33 to 2.42). CONCLUSION: CMR is a valuable tool in the diagnostic and prognostic evaluation of patients with VAs. CMR should be considered early after initial evaluation in the diagnostic algorithm for VAs of unclear aetiology as this strategy may also define prognosis and improve risk stratification.

Antiplatelet Versus Anticoagulation Treatment in Patients With Fontan Operation: A Systematic Review and Meta-Analysis.

Thromboembolism is a significant complication after the Fontan procedure because of endothelial dysfunction, abnormal blood flow, and hypercoagulability. This is the reason why it is recommended for these patients to receive thromboprophylaxis. The aim of our study was to compare the efficacy and safety of antiplatelets versus anticoagulants in patients with a history of a Fontan procedure. A systematic literature review was performed on the electronic databases PubMed, Cochrane, and Scopus, and the grey literature for retrieving studies comparing antiplatelets with anticoagulants and/or no medication on patients with Fontan circulation. We used the random effect model for synthesizing the data. A total of 26 and 20 studies were included in the qualitative and quantitative analysis, respectively. No difference was observed between antiplatelets and anticoagulants in the rate of thromboembolic events [odds ratio (OR), 1.47; 95% confidence interval (CI), 0.66-3.26]. Anticoagulants were more effective than no medication for thromboprophylaxis (OR, 0.17; 95% CI, 0.05-0.61), while comparison between antiplatelets and no medication showed no difference in thromboembolic episodes (OR, 0.25; 95% CI, 0.06-1.09). Antiplatelets were safer than anticoagulants with regards to any bleeding episodes (OR, 0.57; 95% CI, 0.34-0.95). In conclusion, no difference could be found between antiplatelets and anticoagulants in terms of efficacy. However, antiplatelets seem to be safer, as they are responsible for fewer bleeding events. Additional randomized controlled trials are needed to produce robust results.

Prevalence, characteristics, and natural history of apical phenotype in a large cohort of patients with hypertrophic cardiomyopathy.

BACKGROUND: Apical hypertrophic cardiomyopathy (ApHCM) is a variant of hypertrophic cardiomyopathy (HCM) with distinct imaging and clinical characteristics. Data on the prognosis of this HCM subgroup appear conflicting. Our study aims to clarify the natural history of ApHCM and identify predictors of outcomes. MATERIALS AND METHODS: A total of 856 patients with HCM were retrospectively examined. ApHCM was defined as asymmetric left ventricular hypertrophy confined predominantly at the apex, either isolated (pure ApHCM type) or with co-existent hypertrophy of the interventricular septum (mixed ApHCM). Echocardiographic, clinical, and survival data were compared between individuals with ApHCM and non-ApHCM. RESULTS: A total of 143 (16.7%) patients were diagnosed with ApHCM. Compared with non-ApHCM, subjects with apical HCM were diagnosed at an older age and had better echocardiographic indices and more comorbidities at baseline. Apical aneurysms were more prevalent among the ApHCM phenotype (6.3% vs. 1.7%, p = 0.003). During a mean follow-up of 6 ± 3 years, ApHCM was characterized by lower all-cause, cardiovascular, heart failure-related mortality, and ventricular arrhythmic events compared with non-ApHCM. Multivariate analysis identified atrial fibrillation and HCM risk-sudden cardiac death (SCD) as independent predictors of the composite outcome of overall mortality and hospitalizations for cardiovascular reasons (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.9-9.5 for atrial fibrillation and HR 1.2, 95% CI 1.02-1.3 for HCM risk-SCD) in ApHCM. CONCLUSIONS: ApHCM exhibited a lower rate of all-cause mortality and arrhythmic events in the middle-aged population of patients with HCM. Atrial fibrillation and HCM risk-sudden cardiac death were independent predictors of a composite of overall mortality and cardiovascular hospitalizations among those with ApHCM.

Differences in Demographics, in-Hospital Management and Short-Term Prognosis in Admissions for Acutely Decompensated Heart Failure to Cardiology vs. Internal Medicine Departments: A Prospective Study.

Heart failure (HF) is among the leading causes of unplanned hospital admissions worldwide. Patients with HF carry a high burden of comorbidities; hence, they are frequently admitted for non-cardiac conditions and managed in Internal Medicine Departments (IMD). The aim of our study was to investigate differences in demographics, in-hospital management, and short-term outcomes of HF patients admitted to IMD vs. cardiology departments (CD). A prospective cohort study enrolling consecutive patients with acutely decompensated HF either as primary or as secondary diagnosis during the index hospitalization was conducted. Our primary endpoint was a combined endpoint of in-hospital mortality and 30-day rehospitalization for HF. A total of 302 patients participated in the study, with 45% of them admitted to IMD. Patients managed by internists were older with less pronounced HF symptoms on admission. In-hospital mortality was higher for patients admitted to IMD vs. CD (21% vs. 6%, p < 0.001). The composite endpoint of in-hospital death and heart failure hospitalizations at 30 days post-discharge was higher for patients admitted to IMD both in univariate [OR: 3.2, 95% CI (1.8-5.7); p < 0.001] and in multivariate analysis [OR 3.74, 95% CI (1.72-8.12); p = 0.001]. In addition, the HF rehospitalization rate at 6 months after discharge was higher in IMD patients [HR 1.65, 95% CI (1.1, 2.4), p = 0.01]. Overall, HF patients admitted to IMD have worse short-term outcomes compared to patients admitted to CD.

Cardiovascular Magnetic Resonance Parametric Mapping Techniques for the Assessment of Chronic Coronary Syndromes.

The term chronic coronary syndromes encompasses a variety of clinical presentations of coronary artery disease (CAD), ranging from stable angina due to epicardial coronary artery disease to microvascular coronary dysfunction. Cardiac magnetic resonance (CMR) imaging has an established role in the diagnosis, prognostication and treatment planning of patients with CAD. Recent advances in parametric mapping CMR techniques have added value in the assessment of patients with chronic coronary syndromes, even without the need for gadolinium contrast administration. Furthermore, quantitative perfusion CMR techniques have enabled the non-invasive assessment of myocardial blood flow and myocardial perfusion reserve and can reliably identify multivessel coronary artery disease and microvascular dysfunction. This review summarizes the clinical applications and the prognostic value of the novel CMR parametric mapping techniques in the setting of chronic coronary syndromes and discusses their strengths, pitfalls and future directions.

Magnesium Disorders and Prognosis in Heart Failure: A Systematic Review.

Magnesium is an essential mineral for the human body and plays an important role in cardiovascular health. Hypomagnesaemia has been linked with increased cardiovascular mortality in heart failure; however, previous studies have yielded conflicting results. Even fewer studies have addressed the association between hypermagnesemia and prognosis in heart failure. The aim of the present systematic review was to investigate the association of serum magnesium levels with cardiovascular and all-cause mortality in patients with heart failure and reduced ejection fraction (HFrEF). Cardiovascular morbidity, referring to heart failure rehospitalizations and ventricular arrhythmias, was also investigated. Eligible studies were identified by searching PubMed and Scopus. The Quality in Prognosis (QUIPS) tool was used to assess the quality of included studies. Eight studies (total of 13,539 patients with HFrEF) that assessed the effects of serum magnesium levels on cardiovascular mortality, all-cause mortality, and cardiovascular morbidity met inclusion criteria. In half of the studies, hypomagnesemia was found to be an independent risk factor for cardiovascular mortality, including sudden cardiac death. Only 1 study reported that hypermagnesemia (serum magnesium levels above 2.4 mg/dL) is a prognostic factor for noncardiac mortality suggesting that hypermagnesemia is more likely an indicator of comorbidities rather than a true independent prognostic marker. Finally, low serum magnesium levels were not associated with readmissions for heart failure or ventricular arrhythmias in patients with HFrEF.

Advances in treatment of chronic thromboembolic pulmonary hypertension.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a progressive pulmonary vascular disease which can lead to right heart failure and death, if left untreated. CTEPH is caused by persistent obstruction of large, middle-sized, or distal pulmonary arteries due to limited thromboembolic resolution in the pulmonary vascular arterial tree. Every patient with CTEPH should undergo evaluation for Pulmonary Endarterectomy (PEA) after referral to institutions with an experienced multidisciplinary CTEPH team. Although management of distal thromboembolic lesions with PEA remains a challenge due to their difficult accessibility, limited distal CTEPH is not considered an absolute contraindication for PEA, as more expertise surgical teams operate on them successfully. Furthermore, in up to 30-50% of patients who undergo PEA, curative treatment is not achieved due to incomplete thrombi removal or extensive pulmonary microvascular disease. Medical therapies that target the underlying pulmonary microvascular disease can offer symptomatic and hemodynamic benefits, although they do not deal with the core mechanism of the disease which is the removal of thromboembolic material from pulmonary vasculature. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) is a reasonable treatment option for inoperable CTEPH and recurrent/persistent pulmonary hypertension after PEA. Advancements in diagnostic modalities and refinements of BPA technique have decreased the complication rate and increased its beneficial effects in hemodynamics, symptoms, right ventricular function and long-term survival. Ongoing trials and future prospective cohorts will provide evidence regarding the optimal selection of patients and lesions prone to BPA treatment along with hybrid therapeutic strategies combining pharmacological therapy, PEA and BPA, which can potentially change the standard of care in CTEPH.

The prognostic value of right ventricular ejection fraction by cardiovascular magnetic resonance in heart failure: A systematic review and meta-analysis.

BACKGROUND: Cardiac magnetic resonance (CMR) is considered the gold standard for the assessment of right ventricular ejection fraction (RVEF). Previous studies have suggested that RVEF may be a predictor of adverse outcomes in heart failure (HF). In this study, we aimed to systematically review the prognostic value of RVEF, evaluated by CMR, across the spectrum of left ventricular systolic function in patients with HF. METHODS: Electronic databases were searched for studies investigating the prognostic value of RVEF in HF, irrespective of left ventricular ejection fraction (LVEF). A random-effects meta-analysis was conducted for mortality and HF hospitalization. Subgroup analyses were also performed based on the presence of reduced (<50%) or preserved LVEF (≥50%). RESULTS: In total, 46 studies enrolling 14,344 patients were included. In the pooled analyses, impaired RVEF was a powerful predictor of mortality (HR: 1.26, 95% CI: 1.18-1.33, I2: 13%, per 10% decrease in RVEF) and death or HF hospitalization (HR: 1.31, 95% Cl: 1.2-1.42, I2: 27%, per 10% decrease in RVEF). A decrease in RVEF was strongly associated with increased risk of mortality or hospitalization both in HF with reduced EF (HR: 1.24, 95% CI: 1.13-1.36, I2: 2%, per 10% decrease in RVEF) and in HF with preserved EF (HR: 1.24, 95% CI: 1.09-1.40, I2: 0%, per 10% decrease in RVEF). CONCLUSION: Impaired RVEF on CMR strongly predicts adverse outcomes in patients with HF regardless of LVEF. RV systolic function should be carefully evaluated in these patients. Prospero Registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021256967.

Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy.

BACKGROUND: The aim of our study was to assess the performance of the new American College of Cardiology (ACC)/American Heart Association (AHA) Guidelines, with respect to sudden cardiac death (SCD) prevention, in comparison with the established risk score of the European Society of Cardiology (hypertrophic cardiomyopathy [HCM] Risk-SCD), in a large Mediterranean cohort of HCM patients. METHODS: The clinical and imaging characteristics of 784 HCM patients (mean age at first evaluation 52 ± 16 years, 67.2% males) were analyzed retrospectively. The sensitivity, specificity, and negative predictive value for SCD events of the presence of ≥1 risk factor for SCD according to the ACC/AHA Guidelines 2020 and of the HCM Risk-SCD≥6% and HCM Risk-SCD≥4% were estimated during follow-up. RESULTS: During follow-up, 47 (6%) patients suffered an SCD event. The presence of ≥1 major risk factor for SCD according to the new ACC/AHA Guidelines had 96% sensitivity (95% CI 85.5-99.5%) with modest specificity of 59% (95% CI 55-62.2%) and negative predictive value of 99.5% (95% CI 98.2-99.9%). On the contrary, HCM- Risk-SCD≥6% had a relatively low sensitivity (32%, 95% CI 19.1-47.1%) and high specificity of 95% (95% CI 93.1-96.4%), whereas, HCM-Risk-SCD≥4% had sensitivity of 60% (95% CI 44-74%) and specificity of 83.9% (95% CI 80-85.6%). Both the HCM Risk-SCD cut-off values demonstrated lower negative predictive value but higher accuracy than the ACC/AHA algorithm for SCD prediction. CONCLUSION: The novel ACC/AHA proposed algorithm identifies most of the patients with an SCD event with the cost of numerous defibrillator implantations. HCM-Risk-SCD demonstrated higher specificity, whereas its sensitivity and negative predictive value are modest.

Cardiovascular magnetic resonance as a complementary method to transthoracic echocardiography for aortic valve area estimation in patients with aortic stenosis: A systematic review and meta-analysis.

BACKGROUND: Aortic stenosis (AS) is the most common valvular heart disease. While two-dimensional transthoracic echocardiography (2D-TTE) is the standard imaging modality for AS assessment, cardiac magnetic resonance (CMR) offers a reliable and reproducible alternative. The aim of this study was to compare AVA measurements as determined by TTE and CMR in patients with AS. METHODS: Electronic databases were searched to identify studies comparing TTE continuity equation to CMR planimetry for AVA assessment. A meta-analysis of mean difference was conducted by using the random effects model. Sensitivity analysis was performed after excluding studies reporting AVA indexed to body surface area (BSA). Heterogeneity was assessed with I2. RESULTS: A total of 12 studies, encompassing 621 patients, were included in our systematic review. In the pooled analysis, measurements of AVA by CMR planimetry were found to be significantly higher than those calculated by the continuity equation in TTE (pooled mean difference: 0.09, 95% confidence intervals (CI): 0.01, 0.17, and I2: 93%). The results remained significant, albeit with moderate heterogeneity this time, after excluding the analysis measurements of AVA indexed to BSA (pooled mean difference: 0.08, 95% CI: 0.03 to 0.13, and I2 = 61%). CONCLUSIONS: CMR planimetry slightly overestimates AVA compared to TTE continuity equation. Although, 2D-TTE should be the primary imaging modality for the estimation of AVA, CMR may be useful when there is discrepancy with the clinical assessment or when TTE results are discordant or difficult to obtain.

The predictive value of left ventricular and left atrial mechanics for atrial fibrillation and heart failure in hypertrophic cardiomyopathy: a prospective cohort study.

Atrial fibrillation (AF) and heart failure (HF) represent clinical turning points, altering the natural history of HCM and influencing long-term outcome of the disease. The aim of this study was to evaluate the ability of left ventricular (LV) and left atrial (LA) myocardial deformation parameters to predict new-onset AF and HF outcomes in patients with HCM. This was a prospective study that included HCM patients without severe valvular heart disease, prior myocardial infarction or history of AF. The study sample consisted of 250 patients (mean age 50.8 ± 15.8, 67.2% male). Two-dimensional (2D) speckle tracking deformation parameters including global longitudinal strain (GLS), radial strain, circumferential strain, LA reservoir strain (LAεres), LA conduit strain (LAεcon) and LA booster strain(LAεboost) were examined. During a mean follow-up of 2.5 ± 1.2 years, 44 patients developed new-onset AF. All the LV and LA deformation parameters were significant univariate predictors of AF. GLS and LAεres had the highest C statistic among the LV and LA functional indices. In multivariable analysis, only LAεres remained an independent predictor of the arrhythmia (HR 0.91, 95% CI 0.85-0.98, p: 0.008). Similarly, GLS and LAεres had the highest predictive value among the 2D speckle tracking parameters for HF outcomes. LAεres remained an independent predictor after adjusting for significant covariates. GLS and LAεres demonstrated high predictive value for the development of AF and HF in HCM. LAεres was the only independent predictor of both outcomes.Clinical trial registration: ClinicalTrials.gov identifier: NCT04112511.