Search Results (264)

Background and Objectives: Risk stratification in acute heart failure (AHF) remains challenging, particularly in settings where biomarker availability is limited. Echocardiography offers valuable hemodynamic insights, but no single parameter fully captures the complexity of biventricular dysfunction and pressure overload. This study aimed to evaluate a novel echocardiographic index (ViRTUE Index–VTI-RVRA-TAPSE Unified Evaluation) integrating a peak systolic gradient between the right ventricle and right atrium (RV-RA gradient), tricuspid annular plane systolic excursion (TAPSE), the velocity–time integral in the left ventricular outflow tract (VTI LVOT), NT-proBNP (N-terminal pro–B-type Natriuretic Peptide) levels, and in-hospital mortality among patients with AHF. Materials and Methods: We retrospectively analyzed 123 patients admitted with AHF. Echocardiographic evaluation at admission included TAPSE, VTI LVOT, and the RV-RA gradient. An index was calculated as ${\displaystyle \frac{RV-RA gradient }{TAPSE x VTI LVOT}}$. NT-proBNP levels and in-hospital outcomes were recorded. Statistical analysis included correlation, logistic regression, and ROC curve evaluation. Results: The proposed index showed a significant positive correlation with NT-proBNP values (r = 0.543, p < 0.0001) and good discriminative ability for elevated NT-proBNP (AUC = 0.79). It also correlated with in-hospital mortality (r = 0.193, p = 0.032) and showed moderate prognostic performance (AUC = 0.68). Higher index values were associated with greater mortality risk. Conclusions: This novel index, based on standard echocardiographic measurements, reflects both systolic dysfunction and pressure overload in AHF. Its correlation with NT-proBNP and in-hospital mortality highlights its potential as a practical, accessible bedside tool for early risk stratification, particularly when biomarker testing is unavailable or delayed. Full article

Hypertrophic cardiomyopathy (HCM) is a prevalent and often underdiagnosed genetic cardiac disorder characterized by left ventricular hypertrophy and, in many cases, dynamic left ventricular outflow tract obstruction (LVOTO). The development of cardiac myosin inhibitors (CMIs) represents an emerging therapeutic approach in the pharmacological management of obstructive HCM (oHCM). This review offers an integrated and up-to-date synthesis of the cardiac myosin inhibitor class, with a focus on mavacamten, aficamten, and the broader landscape of emerging agents. It also highlights recent clinical trial outcomes, pharmacokinetic and pharmacogenetic considerations, and potential future directions in therapy. Furthermore, we incorporate the most recent data up to May 2025, including late-breaking trial results and real-world safety findings, aiming to provide clinicians with a practical and comprehensive perspective on this evolving drug class. A narrative review was conducted by systematically searching PubMed, Scopus, Google Scholar, and ClinicalTrials.gov for English-language articles and trials published between January 2016 and May 2025. Keywords included “cardiac myosin inhibitor”, mavacamten”, “aficamten”, “MYK-224”, and “hypertrophic cardiomyopathy.” Inclusion criteria encompassed clinical trials and comprehensive reviews specifically addressing CMIs in cardiac applications. CMIs such as mavacamten and aficamten have demonstrated significant clinical benefits in reducing LVOT gradients, improving exercise capacity, and alleviating symptoms in patients with oHCM. Mavacamten is currently approved for clinical use, while aficamten is in advanced regulatory review. Comparative data suggest potential advantages of aficamten in the onset of action, pharmacokinetic profile, and tolerability. Emerging evidence supports the exploration of CMIs in pediatric populations, heart failure with preserved ejection fraction (HFpEF), and non-obstructive HCM (nHCM), although results are still preliminary. Cardiac myosin inhibitors offer a novel, pathophysiology-targeted approach to managing oHCM. While mavacamten has established efficacy, next-generation agents like aficamten may offer improved safety and versatility. Further long-term studies are needed to clarify their role across broader patient populations. Full article

Background: Iron deficiency (ID) is a prevalent comorbidity of heart failure (HF), affecting up to 59% of patients, regardless of the presence of anaemia. Although its negative impact on left ventricular (LV) function is well documented, its effect on right ventricular (RV) function remains unclear. This study assessed the effects of ID on RV global longitudinal strain (RV-GLS) in patients diagnosed with acute decompensated HF (ADHF). Methods: This study included data from 100 patients hospitalised with ADHF irrespective of LV ejection fraction (LVEF) value. ID was defined according to the European Society of Cardiology HF guidelines as serum ferritin <100 ng/mL or ferritin 100–299 ng/mL, with transferrin saturation <20%. Anaemia was defined according to World Health Organization criteria as haemoglobin level <12 g/dL in women and <13 g/dL in men. RV systolic function was assessed using parameters including RV ejection fraction (RVEF), tricuspid annular plane systolic excursion (TAPSE), RV fractional area change (FAC), peak systolic tissue Doppler velocity of the RV annulus (RV TDI S′), acceleration time of the RV outflow tract, and RV free wall GLS. Results: The mean (±SD) age of the study population (64% male) was 70 ± 10 years. The median LVEF was 35%, with 66% of patients classified with HF with reduced ejection fraction, 6% with HF with mid-range ejection fraction, and 28% with HF with preserved ejection fraction. Fifty-eight percent of patients had ID. There were no significant differences between patients with and without ID regarding demographics, LVEF, RV FAC, RV TDI S′, or systolic pulmonary artery pressure. However, TAPSE (15.6 versus [vs.] 17.2 mm; p = 0.05) and RV free wall GLS (−14.7% vs. −18.2%; p = 0.005) were significantly lower in patients with ID, indicating subclinical RV systolic dysfunction. Conclusions: ID was associated with subclinical impairment of RV systolic function in patients diagnosed with ADHF, as evidenced by reductions in TAPSE and RV-GLS, despite the preservation of conventional RV systolic function parameters. Further research validating these findings and exploring the underlying mechanisms is warranted. Full article

This technical report presents a compelling case for the use of very-high-power, very-short-duration (VHPSD) radiofrequency ablation as a promising and efficient strategy for treating symptomatic premature ventricular contractions (PVCs) originating from the right ventricular outflow tract (RVOT). The patient with frequent, symptomatic PVCs and a 24% burden underwent successful ablation using a 90 W/4 s recipe via the QDOT MICRO™ catheter. The procedure resulted in immediate and sustained elimination of PVCs, with only 4 s of ablation time, near-zero fluoroscopy, no complications, and no PVC recurrence at 6 months. VHPSD ablation, though originally developed for atrial fibrillation, demonstrated remarkable procedural efficiency, precision, and lesion efficacy in this case. Compared to standard power, long-duration (SPLD) ablation, VHPSD offers the potential to significantly reduce procedural time, minimize tissue edema, and lower complication risk, particularly advantageous in anatomically challenging areas or in situations where maintaining stable catheter contact for extended periods is difficult or unfeasible. This technical report suggests the transformative potential of VHPSD as a first-line ablation strategy for RVOT-PVCs, provided careful mapping and appropriate technique are used. It underscores the need for further prospective studies to validate its broader safety, efficacy, and role in PVC management, particularly in cases involving intramural origins. Full article

Contemporary evaluation and surgical approaches in congenital aortic valve disease have yielded limited success. The ability to evaluate and understand detailed flow characteristics surrounding surgical repair may be beneficial. This study explores the feasibility and utility of echocardiographic-based blood speckle imaging (BSI) in assessing pre- and post-operative flow characteristics in those with non-stenotic congenital aortic root disease undergoing aortic valve repair or valve-sparing root replacement (VSRR) surgery. Transesophageal echocardiogram was performed during the pre-operative and post-operative assessment surrounding aortic surgery for ten patients with non-stenotic congenital aortic root disease. BSI, utilizing block-matching algorithms, enabled detailed visualization and quantification of flow parameters from the echocardiographic data. Post-operative BSI unveiled enhanced hemodynamic patterns, characterized by quantified changes suggestive of the absence of stenosis and no more than trivial regurgitation. Rectification of an asymmetric jet and the reversal of flow on the posterior aspect of the ascending aorta resulted in a reduced oscillatory shear index ($OSI$) of $0.0543\pm 0.0207$ (pre-op) vs. $0.0275\pm 0.0159$ (post-op) and $p=0.0044$, increased peak wall shear stress of $1.9423\pm 0.6974$ (pre-op) vs. $3.6956\pm 1.4934$ (post-op) and $p=0.0035$, and increased time-averaged wall shear stress of $0.6885\pm 0.8004$ (pre-op) vs. $0.8312\pm 0.303$ (post-op) and $p=0.23$. This correction potentially attenuates cellular alterations within the endothelium. This study demonstrates that children and young adults with non-stenotic congenital aortic root disease undergoing valve-preserving operations experience significant improvements in flow dynamics within the left ventricular outflow tract and aortic root, accompanied by a reduction in $OSI$. These hemodynamic enhancements extend beyond the conventional echocardiographic assessments, offering immediate and valuable insights into the efficacy of surgical interventions. Full article

Background/Objectives: Left atrial function can be a tool for risk stratification for hypertrophic cardiomyopathy (HCM). Over the past decade, there has been growing interest in the application of strain analysis for earlier and more accurate prediction of cardiovascular disease prognosis. This study aimed to investigate the performance of left atrial strain analysis compared to conventional left atrial measures in predicting clinical outcomes in Asian patients with HCM. Methods and Results: This was a retrospective study involving 291 patients diagnosed with HCM between 2010 and 2017. Left atrial volumes were assessed using the method of discs in orthogonal plans at both end diastole and end systole. Left atrial (LA) strain was obtained using a post-hoc analysis with TOMTEC software. We tested the various left atrial parameters against outcomes of (1) heart failure hospitalization and (2) event-free survival from a composite of adverse events, including all-cause mortality, ventricular tachycardia (VT)/ventricular fibrillation (VF) events, appropriate device therapy if an implantable cardioverter defibrillator (ICD) was implanted, stroke, and heart failure hospitalization. The patients had a mean age of 59.0 ± 16.7 years with a male preponderance (71.2%). The cumulative event-free survival over a follow-up of 3.9 ± 2.7 years was 55.2% for patients with an abnormal LA strain versus 82.4% for patients without one (p < 0.001). Multivariable Cox regression analyses were performed separately for each LA parameter, adjusting for age, sex, LV mass index, LV ejection fraction (EF), E/e’, the presence of LV outflow tract (LVOT) obstruction at rest, and atrial fibrillation. An analysis showed that all parameters except for LAEF demonstrated an independent association with heart failure hospitalization. Left atrial strain outperformed the rest of the parameters by demonstrating an association with a composite of adverse events. Conclusions: In Asian patients with HCM, measures of left atrial strain were independently associated with heart failure hospitalization and a composite of adverse outcomes. Left atrial strain may be used as a tool to predict adverse outcomes in patients with HCM. Full article

Hypertrophic cardiomyopathy (HCM) is the most common monogenic heart disease, with an estimated prevalence of 1:600 in the general population, and is associated with significant morbidity. HCM is characterized by left ventricular hypertrophy and interventricular septal thickening due to sarcomere protein gene mutations. The recent emergence of cardiac myosin inhibitors (CMIs), specifically mavacamten and aficamten, has introduced a paradigm shift in HCM management by directly targeting the hypercontractile state of the disease. This review comprehensively discusses the molecular mechanisms of mavacamten and aficamten, highlighting their biochemical similarities and differences from available data. It evaluates their reported efficacy in completed clinical trials, such as reducing left ventricular outflow tract (LVOT) obstruction, improving functional capacity, and enhancing quality of life in HCM. It further provides insight and updates to ongoing trials of both CMIs. Finally, it compares and elaborates on the safety profiles of mavacamten and aficamten, discussing their favorable safety profiles shown in completed studies. In current clinical practice, only mavacamten is approved for use, and clinical insights concerning both CMIs are limited, but encouraging. In summary, cardiac myosin inhibitors are a promising class of disease-modifying drugs for HCM with proven short-term safety and efficacy, but limited data are available to fully determine their long-term effects and efficacy in diverse patient populations. Ongoing research is necessary to further explore and define their role in HCM management. Full article

Mitral valve disease, particularly in the context of extensive mitral annular calcification (MAC), poses significant challenges for traditional surgical management. Transcatheter mitral valve replacement (TMVR) has emerged as a promising alternative for high-risk and inoperable patients, driven by rapid advancements in valve technology, imaging techniques, and procedural strategies. Nevertheless, complications such as paravalvular leak (PVL), left ventricular outflow tract (LVOT) obstruction, and hemolysis remain obstacles to optimal outcomes, particularly in patients with complex annular anatomy. We present the case of an 89-year-old female with severe mitral stenosis and MAC who developed acute hemolytic anemia following experimental TMVR using the Edwards SAPIEN S3 valve. This case serves as a platform to explore recent advances in TMVR, including novel device platforms, enhanced imaging modalities for pre-procedural planning, innovative deployment strategies, and emerging adjunctive techniques aimed at reducing complications. Through this case, we underscore persistent challenges and emphasize the importance of meticulous patient selection and vigilant follow-up. Despite substantial progress, TMVR in the setting of MAC remains high-risk, demanding continued innovation in valve design, refined patient stratification, and improved peri-procedural management to enhance outcomes and mitigate risks such as hemolysis. Full article

Background: In patients undergoing surgical aortic valve replacement due to severe aortic valve stenosis (AS), the presence of right ventricular damage markers (RVDMs) determined by echocardiography is a poor prognostic indicator. However, such data is not available in patient groups who have undergone transcatheter aortic valve replacement (TAVI). The aim of this study is to investigate the prognostic value of RVDM determined by computed tomography (CT) in a patient group undergoing TAVI. Material and Method: This multicentre, prospective study included 184 patients who underwent TAVI. In basal CT, the pulmonary artery (PA) diameter and right ventricular outflow tract myocardial thickness (RVOTMT) in mid-systole and maximal right and left ventricular diameter (RVD/LVD) ratios in mid-diastole were measured as indicators of RVDM before the TAVI procedure. The primary endpoint of the study was 1-year mortality. Results: The primary endpoint of the study was observed in 42 patients (22%). Among the CT parameters, the PA diameter, RVOTMT, and maximal RVD/LVD ratio were observed to be higher in the mortality group (p < 0.001). In the ROC analysis, a PA diameter of 30.5 and above had a 78% sensitivity and 82% specificity (AUC: 0.87 95% CI 0.82–0.93, p < 0.001), an RVOTMT of 4 mm and above had 90% sensitivity and 87% specificity (AUC: 0.93 95% CI 0.87–0.99, p < 0.001), and a maximal RVD/LVD ratio of 0.91 and above showed 90% sensitivity and 92% specificity (AUC: 0.94 95% CI 0.89–0.99, p < 0.001) to predict one-year mortality. In the Cox regression analysis, the RVOTMT and maximal RVD/ LVD ratios were found to be the best independent parameters showing 1-year mortality. Conclusion: This study showed that RVDMs determined by CT are strong predictors of 1-year mortality in severe AS patients treated with TAVI. Full article

The anatomical arrangement of the atrioventricular node has been likened to a riddle wrapped up in an enigma. There are several reasons for this alleged mystery, not least the marked variability in structure between different species. Lack of detailed knowledge of the location of the node relative to the atrial and ventricular septal structures has also contributed to previous misunderstandings. Recent studies comparing the findings of gross dissection with virtual dissection of living datasets, combined with access to a large number of serially sectioned human and animal hearts, have served to provide the evidence to solve the riddle. We summarise these findings in this review. We explain how the node is located within the atrial walls of the inferior pyramidal space. It becomes the non-branching component of the atrioventricular conduction axis as the axis extends through the plane of atrioventricular insulation to enter the infero-septal recess of the left ventricular outflow tract. The node itself is formed by contributions from the tricuspid and mitral vestibules, with extensive additional inputs from the base of the atrial septum. We show how knowledge of development enhances the appreciation of the arrangements and offers an explanation as to why, on occasion, there can be persisting nodoventricular connections. We discuss the findings relative to the circuits producing atrioventricular re-entry tachycardia. We conclude by emphasising the significance of the variation of the anatomical arrangements within different mammalian species. Full article

Background/Objectives: Bovine pericardial valve conduits (PVCs) are commonly used for right ventricular outflow tract reconstruction in both pediatric and adult patients. Calcification, particularly prevalent in children and young adults, is a leading cause of conduit failure and is affected by the chemical composition of the treated biomaterials. In this study, we aimed to compare the structural changes in diepoxy-treated (DE-PVCs) and glutaraldehyde-treated PVCs (GA-PVCs) and to identify factors contributing to tissue mineralization in a large animal model. Methods: Pulmonary artery replacement was performed in minipigs (33–88 kg) using twelve DE-PVCs and four GA-PVCs. After six months, the animals were euthanized, and the explanted PVCs underwent macroscopic and microscopic examination. Results: Large calcium deposits formed along conduit joining suture (CJS) lines in all PVCs, regardless of the cross-linking agent. Mineral clusters surrounded the multifilament braided thread, and its fibers were encrusted with hydroxyapatite crystals. In DE-PVCs, no mineralization occurred outside the suture lines, and they showed successful integration and graft vitalization with a uniform neointima and well-developed endothelial monolayer. GA-PVCs developed a rigid external capsule, foci of collagen fiber calcification within the walls, and neointimal hyperplasia with limited endothelial coverage. Conclusions: In PVCs, calcification predominantly occurs along the CJS lines, where the multifilament suture acts as a nucleation site for hydroxyapatite crystals. DE treatment prevents collagen mineralization, unlike GA, and offers better integration, reduced neointimal hyperplasia, and a well-developed endothelial layer. These findings suggest that DE-PVCs may be a superior option for pediatric cardiac surgery by reducing calcification and improving conduit durability. Overall, the results will help optimize PVC manufacturing strategies to lower the risk of conduit failure. Full article

Tetralogy of Fallot (ToF) with pulmonary atresia (PA) and complete right ventricular outflow tract obstruction (RVOTO) represents one of the most critical forms of congenital heart disease in neonates. These cases require complex and timely interventions to ensure survival and optimize long-term outcomes. While surgical correction offers a favorable prognosis, the period from birth to surgery is often marked by significant hemodynamic, respiratory and nutritional challenges, particularly in neonatal intensive care units (NICUs). This study aims to outline a structured, physiology-guided approach to the preoperative management of neonates with ToF and complete RVOTO, emphasizing stabilization strategies, hemodynamic support, ventilatory management and nutritional optimization. We performed a focused literature review of practices in neonatal ToF management and illustrated our experience through two case reports highlighting divergent outcomes in infants with the same anatomical diagnosis. The management strategies covered include delivery room stabilization, the use of prostaglandins, mechanical ventilation techniques, nutritional interventions and the timing of surgical intervention. A phased, physiology-guided management strategy is the key to the successful preoperative treatment of ToF with pulmonary atresia. Optimizing hemodynamics, ensuring adequate pulmonary blood flow and supporting nutritional needs are the main drivers for growth and may reduce the time from diagnosis to surgical correction. Full article

Background: The arrhythmogenic mechanism of idiopathic ventricular arrhythmias (VAs) from the outflow tracts (OTs) and adjacent anatomical structures has been described to be triggered activity. However, it is incompletely understood why this focal mechanism mainly originates from the OTs and what factors could precipitate it. The aim of this study was to further elucidate the arrhythmogenic mechanisms underlying focal ventricular arrhythmias originating from the outflow tracts. Methods: Six patients referred for catheter ablation of OT-related PVCs were included in this study. Programmed atrial stimulation at the interatrial septum or within the coronary sinus was performed. Pacing at the AV annuli was capable of evoking OT-PVCs with an ECG morphology identical to clinical PVCs by presumably capturing specific fibers within the network of nodal-type tissue of the AV junctional sleeves. Results: Based on the analysis of intracardiac electrograms, the observed PVCs could indeed be elicited as a result of prior atrial stimulation. Conclusions: Our findings suggest that unique pathways might exist between specific periannular atrial locations and the OTs, the activation of which could result in triggering PVCs from the presumed “exit site” of these pathways in the OTs. These findings might facilitate the development of a novel ablation strategy, which might also include the mapping of atrial locations, in order to identify and ablate the presumed “entry sites” of these special pathways. Full article

Neural crest cells (NCCs) play a significant role in the development of ventricular outflow tracts (OFTs), and cardiac neural crest cells (cNCCs) are involved in the development of the embryonic conus, suggesting that these cell lineages may be a teratogenic target for the development of cardiopathies in offspring conceived under a hyperglycemic environment. We evaluate the effect of the hyperglycemic intrauterine environment on the morphological and anatomical changes in the conal ridges along with the alterations in the spatiotemporal expression of AP2α in offspring hearts at 13, 15, and 17 DPC. The anatomical and histological analysis of the hearts in the experimental group presented smaller dimensions compared to the control group in the offspring at the three ages studied. Consequently, this resulted in a hyperglycemic environment that altered the immunostaining of AP2α in the hearts of the offspring at the three ages studied. Thus, the hyperglycemic intrauterine environment in offspring caused important morphological alterations in the development of conal ridges and promoted the generation of conotruncal heart defects in which the double outlet of the right ventricle, the atrioventricular (AV) canal, predominated. Therefore, knowing that exposing the offspring to more glucose potentially can lead to complications during organogenesis of the circulatory and central nervous systems. Full article

Background and Objectives: Aortic stenosis (AS) and hypertrophic obstructive cardiomyopathy (HOCM) are two disease entities that result in left ventricular outflow tract (LVOT) obstruction. We sought to evaluate the longitudinal outcomes of fixed obstruction in severe valvular AS versus dynamic flow obstruction in HOCM. Materials and Methods: Consecutive data with index echocardiographic diagnoses of severe AS and HOCM were collected in a tertiary academic centre between 2010 and 2017. Demographics, comorbidities and clinical outcomes were compared. Results: A total of 134 patients were studied. In the AS group, the mean MPG was 57.2 mmHg ± 13.9, the mean AVA was 0.7 cm² ± 0.2, and the mean Vmax was 4.7 m/s ± 0.5 (p < 0.001). In the HOCM group, the mean LVOT gradient was 60.1 mmHg ± 35.5, the mean IVSd was 17.5 mm ± 4.6, and the mean LVPWd was 12.9 mm ± 2.9 (p < 0.001). Kaplan–Meier curves showed lower cumulative survival with an early separation in heart failure outcomes in the AS arm compared with the HOCM arm (p = 0.023). Similarly, there were higher rates of all-cause mortality for AS compared with HOCM (p = 0.001). For the multivariable Cox regression analysis, AS was significantly associated with a higher incidence of heart failure compared with HOCM after adjusting for the baseline demographics, comorbidities and echocardiographic parameters. There were no significant differences in terms of stroke or cardiovascular (CV) hospitalisation outcomes between the two cohorts. Conclusions: Fixed LVOT obstruction in AS was associated with worse outcomes of heart failure and all-cause mortality compared with dynamic LVOT obstruction in HOCM. Severe AS was an independent predictor of heart failure outcomes after adjustments. Full article