Heart failure risk assessment in patients with hypertrophic cardiomyopathy based on the H2FPEF score.

AIMS: The aim of this study was to investigate whether the H2FPEF score, which was developed to improve the diagnosis of heart failure (HF) with preserved ejection fraction, is associated with HF outcomes in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: Patients with HCM and preserved left ventricular ejection fraction (LVEF ≥50%) were included from a multicentre registry and the H2FPEF score was calculated. Patients were divided into three groups: low (0-1), intermediate (2-5) and high (6-9) H2FPEF score. The primary combined endpoint was a composite of all-cause death and HF admissions, while the secondary endpoints were all-cause death and HF admissions separately. A total of 955 patients were included (age 51 ± 17 years, 310 [32.5%] female). Patients with a high H2FPEF score (n = 105) were more often female, and presented with more symptoms and comorbidities. On echocardiography, patients with a high H2FPEF score had lower LVEF, more impaired diastolic function and more frequently left ventricular outflow tract obstruction. During follow-up (median 90 months [interquartile range 49-176]), 103 (11%) patients died and 57 (6%) patients had a first HF hospitalization. Event-free survival rate for the primary combined and secondary endpoints was lower for patients with an intermediate and high H2FPEF score. On multivariate Cox regression analysis, female sex (hazard ratio [HR] 1.670, 95% confidence interval [CI] 1.157-2.410; p = 0.006), Asian ethnicity (HR 6.711, 95% CI 4.076-11.048; p < 0.001), ischaemic heart disease (HR 1.732, 95% CI 1.133-2.650; p = 0.011), left atrial diameter (HR 1.028, 95% CI 1.005-1.051; p = 0.016) and intermediate (HR 2.757, 95% CI 1.612-4.713; p < 0.001) or high H2FPEF score (HR 3.689, 95% CI 1.908-7.134; p < 0.001) were independently associated with the primary combined endpoint. CONCLUSION: The H2FPEF score is independently associated with HF outcome in patients with HCM and may be considered for risk stratification.

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.

Prognostic implications of genotype findings in non-ischaemic dilated cardiomyopathy: A network meta-analysis.

AIMS: Evidence on the relative impact of diverse genetic backgrounds associated with non-ischaemic dilated cardiomyopathy (DCM) remains contradictory. This study sought to synthesize the available data regarding long-term outcomes of different gene groups in DCM. METHODS AND RESULTS: Electronic databases were systematically screened to identify studies reporting prognostic data on pre-specified gene groups. Those included pathogenic/likely pathogenic (P/LP) variants, truncating titin variants (TTNtv), lamin A/C variants (LMNA), and desmosomal proteins. Outcomes were divided into composite adverse events (CAEs), malignant ventricular arrhythmic events (MVAEs) and heart failure events (HFEs). A total of 26 studies (n = 7255) were included in the meta-analysis and 6791 patients with genotyped DCM were analysed. Patients with P/LP variants had a higher risk for CAEs (odds ratio [OR] 2.10, 95% confidence interval [CI] 1.67-2.65), MVAEs (OR 1.86, 95% CI 1.52-2.26), and HFEs (OR 2.01, 95% CI 1.08-3.73) than genotype-negative patients. The presence of TTNtv was linked to a higher risk for CAEs (OR 1.78, 95% CI 1.20-2.63), but not MVAEs or HFEs. LMNA and desmosomal groups suffered a higher risk for CAEs, MVAEs, and HFEs compared to non-LMNA and non-desmosomal groups, respectively. When genes were indirectly compared, the presence of LMNA resulted in a more detrimental effect that TTNtv, with respect to all composite outcomes but no significant difference was found between LMNA and desmosomal genes. Desmosomal genes harboured a higher risk for MVAEs compared to TTNtv. CONCLUSIONS: Different genetic substrates associated with DCM result in divergent natural histories. Routine utilization of genetic testing should be employed to refine risk stratification and inform therapeutic strategies in DCM.

Incidence and Prognostic Factors of Radial Artery Occlusion in Transradial Coronary Catheterization.

Background/Objectives: Radial artery occlusion (RAO) is the most common complication of transradial coronary catheterization. In this study, we aimed to evaluate the incidence of RAO and identify the risk factors that predispose patients to it. Methods: We conducted an investigator-initiated, prospective, multicenter, open-label study involving 1357 patients who underwent cardiac catheterization via the transradial route for angiography and/or a percutaneous coronary intervention (PCI). Univariate and multivariate logistic regression analyses were performed to identify potential predictors of RAO occurrence. Additionally, a subgroup analysis only for patients undergoing PCIs was performed. Results: The incidence of RAO was 9.5% overall, 10.6% in the angiography-only group and 6.2% in the PCI group. Independent predictors of RAO were as follows: (i) the female gender (aOR = 1.72 (1.05-2.83)), (ii) access site cross-over (aOR = 4.33 (1.02-18.39)), (iii) increased total time of the sheath in the artery (aOR = 1.01 (1.00-1.02)), (iv) radial artery spasms (aOR = 2.47 (1.40-4.36)), (v) the presence of a hematoma (aOR = 2.28 (1.28-4.06)), (vi) post-catheterization dabigatran use (aOR = 5.15 (1.29-20.55)), (vii) manual hemostasis (aOR = 1.94 (1.01-3.72)) and (viii) numbness at radial artery ultrasound (aOR = 8.25 (1.70-40)). Contrariwise, two variables were independently associated with increased odds for radial artery patency (RAP): (i) PCI performance (aOR = 0.19 (0.06-0.63)), and (ii) a higher dosage of intravenous heparin per patient weight (aOR = 0.98 (0.96-0.99)), particularly, a dosage of >50 IU/kg (aOR = 0.56 (0.31-1.00)). In the PCI subgroup, independent predictors of RAO were as follows: (i) radial artery spasms (aOR = 4.48 (1.42-14.16)), (ii) the use of intra-arterial nitroglycerin as a vasodilator (aOR = 7.40 (1.67-32.79)) and (iii) the presence of symptoms at echo (aOR = 3.80 (1.46-9.87)), either pain (aOR = 2.93 (1.05-8.15)) or numbness (aOR = 4.66 (1.17-18.57)). On the other hand, the use of intra-arterial verapamil as a vasodilator (aOR = 0.17 (0.04-0.76)) was independently associated with a greater frequency of RAP. Conclusions: The incidence of RAO in an unselected, all-comers European population after transradial coronary catheterization for angiography and/or PCIs is similar to that reported in the international literature. Several RAO prognostic factors have been confirmed, and new ones are described. The female gender, radial artery trauma and manual hemostasis are the strongest predictors of RAO. Our results could help in the future identification of patients at higher risk of RAO, for whom less invasive diagnostic procedures maybe preferred, if possible.

Moving from left ventricular ejection fraction to deformation imaging in mitral valve regurgitation.

The increasing prevalence of valvular heart diseases, specifically mitral regurgitation (MR), underscores the need for a careful and timely approach to intervention. Severe MR, whether primary or secondary, when left untreated leads to adverse outcomes, emphasizing the critical role of a timely surgical or transcatheter intervention. While left ventricular ejection fraction (LVEF) remains the guideline-recommended measure for assessing left ventricle damage, emerging evidence raises concerns regarding its reliability in MR due to its volume-dependent nature. This review summarizes the existing literature on the role of LVEF and deformation imaging techniques, emphasizing the latter's potential in providing a more accurate evaluation of intrinsic myocardial function. Moreover, it advocates the need for an integrated approach that combines traditional with emerging measures, aiming to optimize the management of patients with MR. It attempts to highlight the need for future research to validate the clinical application of deformation imaging techniques through large-scale studies.

The prognostic impact of diastolic dysfunction after transcatheter aortic valve replacement: A systematic review and meta-analysis.

BACKGROUND: Diastolic dysfunction (DD) is a long-established marker of disease progression in patients with aortic valve stenosis (AS), indicating valvular myocardial damage. Recently, substantial observational data have emerged demonstrating that worse pre-operative DD assessed using echocardiography is associated with adverse long-term clinical outcomes after transcatheter aortic valve replacement (TAVR). AIM: To systematically appraise and quantitatively synthesize current evidence on the prognostic impact of echocardiographic severe DD derived by echocardiography before TAVR. METHODS: A systemic literature review was undertaken in electronic databases to identify studies reporting the predictive value of severe DD in AS subjects undergoing TAVR. A random-effects meta-analysis was conducted to quantify the adjusted and unadjusted hazard ratios (HRs) for all-cause mortality and major adverse cardiovascular events (MACEs) for the presence of severe DD. RESULTS: Ten studies were deemed eligible for inclusion. Of those, 9 provided appropriate quantitative data for the meta-analysis, encompassing a total of 4,619 patients. The presence of severe DD was associated with increased risk for all-cause mortality (pooled unadjusted HR=2.56 [1.46-4.48]; p<0.01; I2=76 %) and MACEs (pooled unadjusted HR=1.82 [1.29-2.58]; p<0.01; I2=86 %). When adjusted for clinically-relevant parameters, the presence of severe DD retained independent association with all-cause mortality (pooled adjusted HR=2.35 [1.26-4.37]; p<0.01; I2=79 %) and MACEs (pooled adjusted HR= 2.52 [1.72-3.65]; p<0.01; I2=0 %). In subgroup analysis there was no difference on post-TAVR risk between the use of different diastolic function grading scores. CONCLUSION: Presence of severe DD assessed by echocardiography pre-TAVR is a major determinant of long-term adverse outcomes after the procedure.

Periostin is overexpressed, correlated with fibrosis and differs among grades of cardiomyocyte hypertrophy in myectomy tissue of patients with hypertrophic cardiomyopathy.

Periostin, a secreted matricellular protein, has been implicated in cardiac extracellular matrix remodeling and fibrosis. Evidence suggest that periostin stimulates cardiomyocyte hypertrophy. The current study aims to investigate the extent of periostin expression in patients with advanced Hypertrophic Cardiomyopathy (HCM) and its correlation with fibrosis and hallmark histopathological features of the disease. Interventricular septal tissue from thirty-nine HCM patients who underwent myectomy and five controls who died from non-cardiac causes was obtained. Staining with Masson's Trichrome and immunohistochemistry were used to localize fibrosis and periostin respectively. The extent of fibrosis and the expression of periostin were defined as the stained percentage of total tissue area using digital pathology software. Periostin expression was higher in HCM patients compared to controls (p<0.0001), positively correlated with the extent of fibrosis (r = 0.82, p<0.001), positively correlated with maximal interventricular septal thickness (Rho = 0.33, p = 0.04) and negatively correlated with LVEF (r = -0.416, p = 0.009). Periostin was approximately co-localized with fibrosis. Mean periostin expression was lower in patients with mild grade cardiomyocyte hypertrophy compared to those with moderate grade (p = 0.049) and lower in patients with mild grade replacement fibrosis compared to moderate grade (p = 0.036). In conclusion, periostin is overexpressed in advanced HCM, correlated with fibrosis and possibly related to cardiomyocyte hypertrophy.

Unmet Needs in the Assessment of Right Ventricular Function for Severe Tricuspid Regurgitation.

Tricuspid regurgitation (TR) is a highly prevalent valvular heart disease that has been long overlooked, but lately its independent association with adverse cardiovascular outcomes was recognized. The time point to intervene and repair the tricuspid valve is defined by the right ventricular (RV) dilation and dysfunction that comes up at a later stage. While guidelines favor tricuspid valve repair before severe RV dysfunction ensues, the definition of RV dysfunction in a universal manner remains vague. As a result, the candidates for transcatheter or surgical TR procedures are often referred late, when advanced RV dysfunction is established, and any derived procedural survival benefit is attenuated. Thus, it is of paramount importance to establish a universal means of RV function assessment in patients with TR. Conventional echocardiographic indices of RV function routinely applied have fundamental flaws that limit the precise characterization of RV performance. More recently, novel echocardiographic indices such as strain via speckle-tracking have emerged, demonstrating promising results in the identification of early RV damage. Additionally, evidence of the role of alternative imaging modalities such as cardiac computed tomography and cardiac magnetic resonance, for RV functional assessment in TR, has recently arisen. This review provides a systematic appraisal of traditional and novel multimodality indices of RV function in severe TR and aims to refine RV function assessment, designate future directions, and ultimately, to improve the outcome of patients suffering from severe TR.

Prognostic Implications of Clinical, Laboratory and Echocardiographic Biomarkers in Patients with Acute Myocardial Infarction-Rationale and Design of the ''CLEAR-AMI Study''.

BACKGROUND: Acute myocardial infarction (AMI) remains a major cause of death worldwide. Survivors of AMI are particularly at high risk for additional cardiovascular events. Consequently, a comprehensive approach to secondary prevention is necessary to mitigate the occurrence of downstream complications. This may be achieved through a multiparametric tailored risk stratification by incorporating clinical, laboratory and echocardiographic parameters. METHODS: The ''CLEAR-AMI Study'' (ClinicalTrials.gov Identifier: NCT05791916) is a non-interventional, prospective study including consecutive patients with AMI without a known history of coronary artery disease. All patients satisfying these inclusion criteria are enrolled in the present study. The rationale of this study is to refine risk stratification by using clinical, laboratory and novel echocardiographic biomarkers. All the patients undergo a comprehensive transthoracic echocardiographic assessment, including strain and myocardial work analysis of the left and right heart chambers, within 48 h of admission after coronary angiography. Their laboratory profile focusing on systemic inflammation is captured during the first 24 h upon admission, and their demographic characteristics, past medical history, and therapeutic management are recorded. The angioplasty details are documented, the non-culprit coronary lesions are archived, and the SYNTAX score is employed to evaluate the complexity of coronary artery disease. A 24-month follow-up period will be recorded for all patients recruited. CONCLUSION: The ''CLEAR-AMI" study is an ongoing prospective registry endeavoring to refine risk assessment in patients with AMI without a known history of coronary artery disease, by incorporating echocardiographic parameters, biochemical indices, and clinical and coronary characteristics in the acute phase of AMI.

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.

Transcatheter Aortic Valve Implantation with the Portico Valve: 2-Year Outcomes of a Multicenter, Real-World Registry.

INTRODUCTION: The self-expanding, resheathable, repositionable transcatheter aortic heart valve Portico is being used successfully for transcatheter aortic valve implantation procedures (TAVI) in patients with severe aortic stenosis. The aim of this study was to evaluate outcomes at 2 years after TAVI with the Portico valve. METHODS: Multicenter registry of clinical, echocardiographic and survival data from consecutive patients treated with the Portico TAVI system (Abbott, Chicago, IL, USA) in three cath labs in Northern Greece and Epirus during 2017-2020. The primary end point was all-cause mortality at 24 months. Secondary end points included procedural outcomes (efficacy and safety) and echocardiographic measurements. RESULTS: A total of 90 patients (81 ± 6 years, 50% females, mean age 81 ± 6 years) were included in the registry. The indication for implantation was severe, symptomatic aortic stenosis (NYHA III, IV) in eighty-two (91.1%) and degeneration of a prosthetic aortic valve in eight (8.9%) patients. All patients were categorized as high surgical risk (mean Logistic Euroscore 25.9 ± 10, Euroscore II 7.7 ± 4.4 and STS score 10.8 ± 8.9). The procedure was performed transfemorally in all patients, under general anesthesia in 95.6%, under TOE guidance in 21.1%, with native valve predilatation in 46.7%, and the "resheath" option was used in 31.1% of the cases. The implantation was successful in 97.8% and there was a need for a second valve in 2.2% of the cases. Complications included permanent pacemaker implantation (16.7%), access cite complications (15.6%), arrythmias (23.3%), paravalvular leak (moderate 7.8%, severe 1.1%), acute kidney injury (7.8%), no strokes and one death during the procedure. Aortic valve peak velocity, peak and mean pressure gradients, were significantly reduced after the procedure. All-cause mortality at 1, 12 and 24 months was 4.4%, 6.7% and 7.8%, respectively. CONCLUSIONS: TAVI with the Portico system comprises an effective and safe solution for the management of severe, symptomatic aortic stenosis in high-risk surgical patients.

Right Ventricular Global Longitudinal Strain and Short-Term Prognosis in Patients With First Acute Myocardial Infarction.

Right ventricular (RV) dysfunction after acute myocardial infarction (AMI) is a recognized predictor of dismal prognosis. However, the most reliable RV index to predict mortality early after revascularization remains undetermined. This study aimed to explore the ability of RV global longitudinal strain (GLS) to predict inhospital mortality in patients with first AMI. All consecutive patients with first AMI were prospectively enrolled from March 2022 until February 2023. An echocardiogram was performed 24 hours after successful revascularization and RV GLS alongside conventional echocardiographic indexes were measured. Inhospital mortality was recorded. A total of 300 patients (age 61.2 ± 11.8 years, 74% male) were included in the study. RV GLS was the only RV performance index that differed significantly between anterior and inferior ST-segment-elevation patients with AMI (14.5 ± 5.2% vs 17.4 ± 5.1% respectively, p <0.001). After revascularization, 23 patients (7.7%) died in hospital. The model of Global Registry of Acute Coronary Event risk score and left ventricular ejection fraction, built for predicting inhospital mortality, significantly improved its prognostic performance only by the addition of RV GLS (chi-square value increase by 7.485, p = 0.006) compared with the other RV function indexes. RV GLS was independently associated with inhospital mortality (odds ratio 0.83, 95% confidence interval 0.71 to 0.97, p = 0.017) after adjustment for Global Registry of Acute Coronary Event risk score and left ventricular ejection fraction. Echocardiographic RV GLS measured 24 hours after revascularization in patients with first AMI outperformed conventional RV function indexes in predicting inhospital mortality.

Apical Hypertrophic Cardiomyopathy: Diagnosis, Natural History, and Management.

Apical hypertrophic cardiomyopathy (ApHCM) represents a rare variant of hypertrophic cardiomyopathy (HCM) with distinct phenotypic characteristics. The prevalence of this variant varies according to each study's geographic region. The leading imaging modality for the diagnosis of ApHCM is echocardiography. Cardiac magnetic resonance, however, is the gold standard for ApHCM diagnosis in case of poor acoustic windows or equivocal echocardiographic findings but also in cases of suspected apical aneurysms. The prognosis of ApHCM was reported to be relatively benign, although more recent studies seem to contradict this, demonstrating similar incidence of adverse events compared with the general HCM population. The aim of this review is to summarize the available evidence for the diagnosis of ApHCM, highlight distinctions in comparison to more frequent forms of HCM with regards to its natural history, prognosis, and management strategies.

Shifting from Left Ventricular Ejection Fraction to Strain Imaging in Aortic Stenosis.

Adverse ventricular remodeling is an inflexion point of disease progression in aortic stenosis (AS) and a major determinant of prognosis. Intervention before irreversible myocardial damage is of paramount importance to sustain favorable post-operative outcomes. Current guidelines recommend a left ventricular ejection fraction (LVEF)-based strategy to determine the threshold for intervention in AS. However, LVEF has several pitfalls: it denotes the left ventricular cavity volumetric changes and it is not suited to detecting subtle signs of myocardial damage. Strain has emerged as a contemporary imaging biomarker that describes intramyocardial contractile force, providing information on subclinical myocardial dysfunction due to fibrosis. A large body of evidence advocates its use to determine the switch from adaptive to maladaptive myocardial changes in AS, and to refine thresholds for intervention. Although mainly studied in echocardiography, studies exploring the role of strain in multi-detector row computed tomography and cardiac magnetic resonance are emerging. This review, therefore, summarizes contemporary evidence on the role of LVEF and strain imaging in AS prognosis, aiming to move from an LVEF-based to a strain-based approach for risk stratification and therapeutic decision-making in AS.

Clinical Value of Novel Echocardiographic Biomarkers Assessing Myocardial Work in Acute Heart Failure-Rationale and Design of the "Beyond Myo-HF Study".

BACKGROUND: Despite ongoing treatment advancements in chronic heart failure (HF), mortality and readmission rates remain high for patients hospitalized for decompensated acute HF. These patients represent a distinct HF group, which requires emergent echocardiographic evaluation in an attempt to provide optimal and individualized acute care. The role of serial advanced echocardiographic assessment in acute HF for risk stratification and treatment guidance has not been thoroughly explored. METHODS: The "Beyond Myo-HF Study" is a prospective, non-interventional cohort trial designed to enroll acutely admitted patients with symptoms and/or signs of HF. The aim of this study is to investigate whether intrahospital changes of conventional and novel echocardiographic indices of myocardial function and congestion-related markers can predict early mortality, late mortality, and HF rehospitalization. As per the protocol, all patients undergo a pair of state-of-the-art echocardiographic assessments, with a rigorous protocol including speckle tracking analysis of all cardiac chambers and myocardial work analysis for the left and right ventricle, upon admission and pre-discharge. Their laboratory profile is captured at those two time-points, and their therapeutic management is recorded. Patients will be followed-up for a median period of 12 months after enrollment. CONCLUSIONS: The "Beyond Myo-HF" study is an ongoing, prospective trial aspiring to provide deep insight into the pathophysiology of acute HF, to enlighten the reverse cardiac functional and anatomical remodeling during hospitalization, and to recognize echocardiographic patterns capable of predicting adverse outcomes during and post decompensation of acute HF.

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.

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.

Wild-Type Transthyretin Amyloid Cardiomyopathy: The Gordian-Knot of Novel Therapeutic Regimens.

Wild-type TTR amyloidosis (wtATTR) represents a disease difficult to diagnose with poor prognosis. Increased clinical suspicion is key, allowing for timely diagnosis. Until recently, only off-label therapies were available but recent introduction of disease specific therapy has shown potential to alter the natural history of the disease. Tafamidis, the only currently approved drug for the therapy of wtATTR, provided significantly better survival and quality of life. However, not all subgroups of patients derived equal benefit. This, along with the increased cost of treatment raised question on whether treatment should be invariably administered through the wtATTR population. This review aims to summarize current evidence on the natural history and staging systems for wtATTR, as well as available treatment options. Special consideration is given to the selection process of patients who would be expected to gain maximum benefit from tafamidis treatment, based on an ethical and cost-effective point of view.