Neuromuscular diseases and their cardiac manifestations under the spectrum of cardiovascular imaging.

Neuromuscular diseases (NMDs) include a broad spectrum of disorders that affect motor unit in every possible site, extending from the cell body of peripheral nerves to the muscle. The different lesion sites make this group of inherited disorders difficult to diagnose. Many NMDs, especially those involving skeletal muscles, can present significant cardiovascular complications, ranging from rhythm disturbances to the development of dilated or hypertrophic cardiomyopathy. Heart disease represents a major cause of morbidity and mortality among NMD patients, underlining the vital need for further familiarization with the pathogenesis and assessment of cardiac involvement. Cardiovascular imaging is the cornerstone for the evaluation of heart disorders in NMDs, with conventional echocardiography still offering a portable, affordable, and easily accessible solution. Meanwhile, newer echocardiographic techniques such as speckle tracking imaging in combination with cardiac magnetic resonance add new insights into further substrate characterization. The purpose of this review is to offer a brief presentation of the main NMDs and their cardiovascular complications, as well as the presentation of data that highlight the importance of cardiovascular imaging in early diagnosis, monitoring, and prognosis of these patients. Lastly, the authors provide a simple guide about which clinical features, imaging findings, and follow-up plan to adopt in each myopathic disorder.

Angiographic predictors of aberrant circumflex artery during cardiac catheterization.

OBJECTIVES: To identify angiographic predictors of aberrant left circumflex artery (LCx) by comparing left main (LM) length and bifurcation angle between patients with aberrant LCx and normal anatomy. BACKGROUND: Failure to recognize aberrant LCx during a cardiac catheterization may hamper correct diagnosis, delay intervention in acute coronary syndromes, and result in increased contrast volume, radiation exposure, and infarct size. METHODS: We retrospectively analyzed angiograms of aberrant LCx patients and normal anatomy matched controls, in three-participating centers. LM-length, bifurcation angle between the left anterior descending (LAD) and the first non-LAD branch of the LM, and procedural data were compared. RESULTS: Between 2003 and 2020, 136 patients with aberrant LCx and 135 controls were identified. More catheters (2.4 ± 0.6 vs. 2.2 ± 0.9, p = 0.009), larger contrast volumes (169 ± 94 ml vs. 129 ± 68 ml, p < 0.0005), and prolonged fluoroscopy time (652.9 ± 623.7 s vs. 393.1 ± 332.1 s, p < 0.0005), were required in the aberrant LCx-group compared with controls. Patients with aberrant LCx had a longer LM-length and a more acute bifurcation angle, both in caudal and cranial views, compared with controls (24.7 ± 8.1 vs. 10.8 ± 4.5 mm, p < 0.0005 and 26.7 ± 7.4 vs. 12 ± 5.5 mm, p < 0.0005, respectively, and 45.2° ± 12° vs. 88.8° ± 23°, p < 0.0005 and 51.9° ± 21° vs. 68.2° ± 28.3°, p < 0.0005, respectively). In ROC analysis, LM-length showed the best diagnostic accuracy for detecting aberrant LCx. In multiple logistic regression analysis, a cranially measured LM-length > 17.7 mm was associated with a 5.3 times greater probability of predicting aberrant LCx [95% CI (3.4-8.1), p < 0.0001]. CONCLUSIONS: Our study suggests that a long LM-length and an acute bifurcation angle can indicate the presence of aberrant LCx. We present a practical algorithm for its rapid identification.

Fibrosis in hypertrophic cardiomyopathy: role of novel echo techniques and multi-modality imaging assessment.

Hypertrophic cardiomyopathy (HCM) represents one of the primary cardiomyopathies and may lead to heart failure and sudden cardiac death. Among various histologic features of the disease examined, assessment of myocardial fibrosis may offer valuable information, since it may be considered the common nominator for all HCM connected complications. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) has emerged as the reference noninvasive method for visualizing and quantifying myocardial fibrosis in patients with HCM. T1 mapping, a promising new CMR technique, may provide an advantage over conventional LGE-CMR, by permitting a more valid quantification of diffuse fibrosis. On the other hand, echocardiography offers a significantly more portable, affordable, and easily accessible solution for the study of fibrosis. Various echocardiographic techniques ranging from integrated backscatter and contrast-enhanced ultrasound to two- (2D) or three-dimensional (3D) deformation and shear wave imaging may offer new insights into substrate characterization in HCM. The aim of this review is to describe thoroughly all different modalities that may be used in everyday clinical practice for HCM fibrosis evaluation (with special focus on echocardiographic techniques), to concisely present available evidence and to argue in favor of multi-modality imaging application. It is essential to understand that the role of various imaging modalities is not competitive but complementary, since the information provided by each one is necessary to illuminate the complex pathophysiologic pathways of HCM, offering a personalized approach and treatment in every patient.

Impact of centre volume on atrial fibrillation ablation outcomes in Europe: a report from the ESC EHRA EORP Atrial Fibrillation Ablation Long-Term (AFA LT) Registry.

AIMS: The aim of the study was to investigate differences in clinical outcomes and complication rates among European atrial fibrillation (AF) ablation centres related to the volume of AF ablations performed. METHODS AND RESULTS: Data for this analysis were extracted from the ESC EHRA EORP European AF Ablation Long-Term Study Registry. Based on 33rd and 67th percentiles of number of AF ablations performed, the participating centres were classified into high volume (HV) (≥ 180 procedures/year), medium volume (MV) (<180 and ≥74/year), and low volume (LV) (<74/year). A total of 91 centres in 26 European countries enrolled in 3368 patients. There was a significantly higher reporting of cardiovascular complications and stroke incidence in LV centres compared with HV and MV (P = 0.039 and 0.008, respectively) and a lower success rate after AF ablation (55.3% in HV vs. 57.2% in LV vs. 67.4% in MV centres, P < 0.001), despite lower CHA2DS2-VASc score of patients, enrolled in LVs and less complex ablation techniques used. Adjustments of confounding factors (including type of AF ablation) led to elimination of these differences. CONCLUSION: Low-volume centres tended to present slightly higher cardiovascular complications' and stroke incidence and a lower unadjusted success rate after AF ablation, despite the fact that ablation procedures and patients were of lower risk compared with MV and HV centres. On the other hand, adjusted overall complication and recurrence rates were non-significantly different among different volume centres, a fact reflecting the heterogeneity of patient and procedural profiles, and a counterbalance between expertise and risk level among participating centres.

Arterial Function after a 246 km Ultra-marathon Running Race.

There is little research about the effects of ultra-endurance exercise on arterial morphological and functional properties. The aim was to assess the acute changes of the carotid-femoral pulse wave velocity and carotid doppler-derived parameters following an ultra-marathon race as well as the intima-media thickness of the carotid artery in ultra-marathon runners. Twenty athletes were examined at baseline and within 10 mins after a 246 km running race. Measurements included carotid-femoral pulse wave velocity, peak-systolic and end-diastolic velocities of carotid artery blood flow, pulsatility and resistivity indices and blood biochemical parameters. The intima-media thickness of the right and left carotid artery was measured before the race. Arterial stiffness and carotid artery intima media thickness at rest remained within known normal limits. The ultra-marathon race significantly increased carotid-femoral pulse wave velocity by 22.6% and pulsatility index by 10.2%. There was a decrease in body weight by 3.35% and an increase of all biochemical markers of muscle damage after the race. Additionally, C-reactive protein was correlated with both pulsatility and resistivity indices post-race. This study shows that immediately after a 246 km ultra-marathon running race, acute increase of arterial stiffness and vascular resistance were evident. The carotid artery thickness of ultra-marathon runners was within normal range.

Free Cortisol Is a More Accurate Marker for Adrenal Function and Does Not Correlate with Renal Function in Cirrhosis.

BACKGROUND: The accuracy of diagnosis and clinical implications of the hepatoadrenal syndrome, as currently diagnosed using total cortisol, remain to be validated. AIM: The aim of this study was to assess adrenal function using free cortisol in stable cirrhosis and study the potential implications of any abnormalities for renal and/or cardiac function. METHODS: Sixty-one stable consecutively enrolled patients with cirrhosis underwent assessment of adrenal function using the low-dose short Synacthen test, renal function by 51Cr-EDTA glomerular filtration rate (GFR), and cardiac function by two-dimensional echocardiography. RESULTS: Eleven patients (18%) had total peak cortisol (PC) < 500 nmol/L, but no patient had free PC < 33 nmol/L indicating that diagnosis of AI using total cortisol is not confirmed using free cortisol. Free cortisol did not correlate with GFR or parameters of cardiac function. Patients with higher Child-Pugh class had progressively lower free cortisol. Patients with low GFR < 60 mL/min (N = 22) had more frequently grade II-III diastolic dysfunction (66.7% vs. 17.6%; p = 0.005) and had higher Child-Pugh and MELD score compared to those with normal GFR. CONCLUSIONS: Diagnosis of AI using total cortisol is not confirmed using free cortisol and is thus considered unreliable in cirrhosis. Free cortisol is not associated with renal or cardiac dysfunction. Lower free cortisol in more advanced stages of liver disease might be secondary to decreased synthesis due to lower cholesterol levels. Irrespective of free cortisol, parameters of cardiac dysfunction are associated with renal impairment supporting the cardio-renal hypothesis.

Prognostic value of electrocardiographic time intervals and QT rate dependence in hypertrophic cardiomyopathy.

INTRODUCTION: Preventing sudden cardiac death (SCD) is one of the main goals in hypertrophic cardiomyopathy (HCM). Many variables have been proposed, however the European and American guidelines do not incorporate any ECG or Holter monitoring derived variables other than the presence of ventricular arrhythmia in their risk stratification models. In the present study we evaluated electrocardiographic parameters in risk stratification of HCM. METHODS AND RESULTS: Novel electrocardiographic parameters including the index of cardio-electrophysiological balance (iCEB), individualized QT correction (QTi) and QT rate dependence were evaluated along with established risk factors. A composite endpoint of SCD was defined as out of hospital cardiac arrest, appropriate ICD shock and sustained ventricular tachycardia. Cox regression analysis was used to evaluate predictors of SCD. Out of the 466 HCM patients, 31 reached the composite endpoint during a follow up of 75 ±â€¯86 months. In a multivariate model, nor iCEB, QTi or QT rate dependence were predictors of SCD. Only male gender (p < 0.01; OR 13.1; CI 1.74-98.83), negative T waves in the inferior leads (p = 0.04; OR 2.51; CI 1.03-6.13) and familial sudden death (p < 0.01; OR 3.03; CI 1.39-6.59) were significant predictors. On top of either the ESC risk score or the 3 traditional 'American risk factors', only male gender was a significant predictor of SCD. CONCLUSION: No ECG or Holter monitoring parameters added in risk stratification for SCD in HCM. However, male gender and negative T waves in the inferior leads are promising novel markers to evaluate in larger cohorts.

Right ventricular remodelling after transcatheter pulmonary valve implantation.

OBJECTIVES: To define the optimal timing for percutaneous pulmonary valve implantation (PPVI) in patients with severe pulmonary regurgitation (PR) after Fallot's Tetralogy (ToF) correction. BACKGROUND: PPVI among the aforementioned patients is mainly driven by symptoms or by severe right ventricular (RV) dilatation/dysfunction. The optimal timing for PPVI is still disputed. METHODS: Twenty patients [age 13.9 ± 9.2 years, (range 4.3-44.9), male 70%] with severe PR (≥3 grade) secondary to previous correction of ToF, underwent Melody valve (Medtronic, Minneapolis, MN) implantation, after a pre-stent placement. Full echocardiographic assessment (traditional and deformation analysis) and cardiovascular magnetic resonance evaluation were performed before and at 3 months after the intervention. 'Favorable remodelling' was considered the upper quartile of RV size decrease (>20% in 3 months). RESULTS: After PPVI, indexed RV effective stroke volume increased from 38.4 ± 9.5 to 51.4 ± 10.7 mL/m2 , (P = 0.005), while RV end-diastolic volume and strain indices decreased (123.1 ± 24.1-101.5 ± 18.3 mL/m2 , P = 0.005 and -23.5 ± 2.5 to -21 ± 2.5%, P = 0.002, respectively). After inserting pre-PPVI clinical, RV volumetric and deformation parameters in a multiple regression model, only time after last surgical correction causing PR remained as significant regressor of RV remodelling [R2 = 0.60, beta = 0.387, 95%CI(0.07-0.7), P = 0.019]. Volume reduction and functional improvement were more pronounced in patients treated with PPVI earlier than 7 years after last RV outflow tract (RVOT) correction, reaching close-to-normal values. CONCLUSIONS: Early PPVI (<7 years after last RVOT operation) is associated with a more favorable RV reverse remodelling toward normal range and should be considered, before symptoms or RV damage become apparent. © 2017 Wiley Periodicals, Inc.

An Overview of Pharmacotherapy in Hypertrophic Cardiomyopathy: Current Speculations and Clinical Perspectives.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. Its clinical course is variable, ranging from a benign asymptomatic or mildly symptomatic course throughout life, to severe symptoms (dyspnea, angina, palpitations) or cardiovascular events (syncope and thromboembolism). Sudden cardiac death (SCD) remains the most striking manifestation of the disease, affecting a minority of patients. This review focuses on the medical treatments applied according to the symptomatology in obstructive and nonobstructive HCM; a special reference is made to atrial fibrillation and arterial hypertension, which often coexist with the disease. Current literature about the pharmaceutical prevention of SCD is also analyzed and novel pharmacologic agents and approaches that may represent the future management of HCM are critically reviewed. The analysis of interventional techniques that are used in cases of medical treatment failure is avoided. Rather than enumerating clinical studies and guidelines, this review provides a concise and contemporary analysis of HCM pharmacotherapy, developing applicable algorithms for clinicians and highlighting promising future drug regimens.

Diastolic dysfunction is associated with low urinary sodium excretion in patients with decompensated cirrhosis.

BACKGROUND/AIM: The pathogenesis and the clinical impact of diastolic dysfunction (DD) in cirrhosis remain unclear. Our aim was to investigate the factors significantly associated with the presence of DD in patients with decompensated cirrhosis on the waiting list for liver transplantation. MATERIAL AND METHODS: consecutive patients with decompensated cirrhosis, who admitted for transplant assessment, were prospectively evaluated. We assessed the independent factors associated with the presence of DD, while their discriminative ability was evaluated by AUC curve. The diagnosis of DD was based on Doppler echocardiography and classified into three categories according to the current guidelines. RESULTS: we evaluated 115 consecutive patients. Sixty six patients (57.3%-group 1) had DD and 49 (42.7%-group 2) had not DD. The 2 groups had similar Child-Pugh/MELD scores and survival. In multivariable logistic regression analysis, pulse rate (OR: 1.082, 95% CI: 1.03-1.15, p = 0.004), and UNa24h (OR: 0.98, 95% CI: 0.97- 0.99, p = 0.004) were the only variables independently associated with the presence of DD. In the subgroup of consecutive patients (n = 31) with evaluation of cytokines, those (n = 22) with DD, compared to those (n = 9) without DD, had significantly higher levels of inteleukin-6 [145 (45-2000) vs. 56 (10-149)pg/mL, p = 0.043]. CONCLUSIONS: We found that DD was independently associated with lower 24-hour urine sodium. Although no correlation was found between DD and severity of liver disease or survival, further studies are needed for final conclusions.

Left Ventricular Myocardial Mechanics in Cirrhosis: A Speckle Tracking Echocardiographic Study.

Cirrhosis is commonly associated with impaired left ventricular (LV) myocardial contractile reserve to stress and diastolic dysfunction. The aim of this study was to assess LV systolic performance at rest, using both "standard" echocardiographic indices and novel deformation-rotational parameters, in order to elucidate the pathophysiologic basis of cardiac dysfunction in cirrhosis. Seventy-seven men with cirrhosis (mean age 54.4 ± 9.7) of variable Child-Pugh class (A, B, C) and 20 healthy control subjects were prospectively evaluated by standard as well as speckle tracking echocardiography. Left ventricular ejection fraction (LVEF) was significantly higher in patients with cirrhosis compared to controls (64.6 ± 5.7% in controls vs. 71 ± 9.5%, 71.2 ± 7.1%, and 73 ± 7% in Child-Pugh classes A, B, and C, respectively, P = 0.002). Interestingly, LV systolic function augmentation was not associated with changes in LV longitudinal deformation (LV strain -19 ± 1.9% in controls vs. -20.1 ± 5.3% in class A vs. -21.3 ± 2.6% in class B vs. -21 ± 3.4% in class C, P = NS), but a statistically significant increase in LV apical systolic rotation and accordingly in LV twist was observed (LV twist 13.0 ± 3° in controls vs. 14.9 ± 5° in class A vs. 16.5 ± 2.8° in class B vs. 18.2 ± 2.9° in class C, P < 0.0005). Despite the increase in LV rotation, time to both basal and apical peak systolic rotation was significantly delayed in patients compared to healthy controls (P = 0.015 and P = 0.017 accordingly). Increased EF in cirrhosis could be attributed to increased LV torsion. Despite the "improved" rotation values at rest, there is a significant time delay in succeeding peak systolic rotation, hampering also the consequent untwisting-diastolic period.

Recent advancements and applications of deep learning in heart failure: Α systematic review.

BACKGROUND: Heart failure (HF), a global health challenge, requires innovative diagnostic and management approaches. The rapid evolution of deep learning (DL) in healthcare necessitates a comprehensive review to evaluate these developments and their potential to enhance HF evaluation, aligning clinical practices with technological advancements. OBJECTIVE: This review aims to systematically explore the contributions of DL technologies in the assessment of HF, focusing on their potential to improve diagnostic accuracy, personalize treatment strategies, and address the impact of comorbidities. METHODS: A thorough literature search was conducted across four major electronic databases: PubMed, Scopus, Web of Science and IEEE Xplore, yielding 137 articles that were subsequently categorized into five primary application areas: cardiovascular disease (CVD) classification, HF detection, image analysis, risk assessment, and other clinical analyses. The selection criteria focused on studies utilizing DL algorithms for HF assessment, not limited to HF detection but extending to any attempt in analyzing and interpreting HF-related data. RESULTS: The analysis revealed a notable emphasis on CVD classification and HF detection, with DL algorithms showing significant promise in distinguishing between affected individuals and healthy subjects. Furthermore, the review highlights DL's capacity to identify underlying cardiomyopathies and other comorbidities, underscoring its utility in refining diagnostic processes and tailoring treatment plans to individual patient needs. CONCLUSIONS: This review establishes DL as a key innovation in HF management, highlighting its role in advancing diagnostic accuracy and personalized care. The insights provided advocate for the integration of DL in clinical settings and suggest directions for future research to enhance patient outcomes in HF care.

Clinical characteristics and natural history of hypertrophic cardiomyopathy with midventricular obstruction.

BACKGROUND: The prevalence, clinical characteristics and natural history of patients with hypertrophic cardiomyopathy (HCM) and midventricular obstruction (MVO) have not been adequately studied. METHODS AND RESULTS: A single-center cohort consisting of 423 patients (mean age, 49.3±17.2 years; 66.2% male) was thoroughly followed up for a median of 84 months (7 years; range, 6-480 months). MVO, characterized by the echocardiographic appearance of midventricular muscular apposition with a simultaneous mid-cavitary gradient ≥30mmHg, was identified in 34 patients (8%). Patients with MVO tended to be more symptomatic during their initial evaluation (>90% presented with NYHA class ≥II) compared to the rest of the HCM cohort. Apical aneurysm formation was identified in more than one-fourth of patients with MVO (26.5%), being a characteristic of the group. On multivariate Cox regression hazard analysis, presence of MVO strongly predicted progression to end-stage (burnt out) HCM and related heart failure (HF) deaths (hazard ratio, [HR], 2.62; 95% confidence interval [CI]: 1.2-8.8; P=0.047), as well as sudden death and associated lethal arrhythmic events (HR, 3.3; 95% CI: 1.26-8.85; P=0.016). CONCLUSIONS: MVO is a distinct phenotype of HCM associated with unfavorable prognosis in terms of end-stage HCM, sudden death and lethal arrhythmic events. The high adverse outcome rate necessitates early recognition of MVO and appropriate therapeutic interventions.

Efficacy of various "classic" echocardiographic and laboratory indices in distinguishing the "gray zone" between athlete's heart and hypertrophic cardiomyopathy: a pilot study.

Left ventricular hypertrophy (LVH) with intraventricular septum thickness (IVST) between 1.2 and 1.5 cm in athletes represents a "gray zone" between physiologic adaptation and mild hypertrophic cardiomyopathy (HCM). Various echo and laboratory parameters have been reported till now in the literature to discriminate the "gray zone" entities. Aim of this study was to assess the efficacy of these "classic" parameters in differentiating physiologic LVH in athletes from mild HCM in a highly selected population. Nine highly trained athletes with IVST (1.28 ± 0.07 cm), 9 patients with mild HCM (1.38 ± 0.11 cm), and 26 athletes without LVH (1.06 ± 0.09 cm; P < 0.0005) underwent echocardiographic study, cardiopulmonary treadmill exercise stress test, and brain natriuretic peptide (BNP) measurement before and after exercise. Among all parameters tested, 7 were found to significantly differ between "gray zone" groups. After bootstrapping analysis, it was found that athletes with left ventricular end-diastolic diameter <4.74 cm, mitral deceleration time >200 ms, isovolumic relaxation time >94 ms, tricuspid E/A < 1.63, septum Em < 9.5 cm/sec, relative wall thickness >0.445, and a BNP value at rest >9.84 pg/mL had a greater possibility for having underlying cardiomyopathy. A 10-point score based on these parameters showed accuracy (area under the curve = 0.958 [95%CI: 0.738-1.0; P = 0.00005, standard error = 0.0342]) for revealing HCM in a gray zone athletic population. Differentiation of adaptive LVH versus HCM in a gray zone population could be facilitated by recognition of certain features referring to LV dimensions, diastolic function, and BNP.

Clinical Validation of an Artificial Intelligence-Based Tool for Automatic Estimation of Left Ventricular Ejection Fraction and Strain in Echocardiography: Protocol for a Two-Phase Prospective Cohort Study.

BACKGROUND: Echocardiography (ECHO) is a type of ultrasonographic procedure for examining the cardiac function and morphology, with functional parameters of the left ventricle (LV), such as the ejection fraction (EF) and global longitudinal strain (GLS), being important indicators. Estimation of LV-EF and LV-GLS is performed either manually or semiautomatically by cardiologists and requires a nonnegligible amount of time, while estimation accuracy depends on scan quality and the clinician's experience in ECHO, leading to considerable measurement variability. OBJECTIVE: The aim of this study is to externally validate the clinical performance of a trained artificial intelligence (AI)-based tool that automatically estimates LV-EF and LV-GLS from transthoracic ECHO scans and to produce preliminary evidence regarding its utility. METHODS: This is a prospective cohort study conducted in 2 phases. ECHO scans will be collected from 120 participants referred for ECHO examination based on routine clinical practice in the Hippokration General Hospital, Thessaloniki, Greece. During the first phase, 60 scans will be processed by 15 cardiologists of different experience levels and the AI-based tool to determine whether the latter is noninferior in LV-EF and LV-GLS estimation accuracy (primary outcomes) compared to cardiologists. Secondary outcomes include the time required for estimation and Bland-Altman plots and intraclass correlation coefficients to assess measurement reliability for both the AI and cardiologists. In the second phase, the rest of the scans will be examined by the same cardiologists with and without the AI-based tool to primarily evaluate whether the combination of the cardiologist and the tool is superior in terms of correctness of LV function diagnosis (normal or abnormal) to the cardiologist's routine examination practice, accounting for the cardiologist's level of ECHO experience. Secondary outcomes include time to diagnosis and the system usability scale score. Reference LV-EF and LV-GLS measurements and LV function diagnoses will be provided by a panel of 3 expert cardiologists. RESULTS: Recruitment started in September 2022, and data collection is ongoing. The results of the first phase are expected to be available by summer 2023, while the study will conclude in May 2024, with the end of the second phase. CONCLUSIONS: This study will provide external evidence regarding the clinical performance and utility of the AI-based tool based on prospectively collected ECHO scans in the routine clinical setting, thus reflecting real-world clinical scenarios. The study protocol may be useful to investigators conducting similar research. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/44650.

An echocardiographic study of acute, progressive cardiac changes following a 246 km running race.

We describe the case of an ultra-marathon runner who finished first the "Spartathlon", a 246 km running race. The finishing time was the second fastest time ever in "Spartathlon". After finishing the race, the athlete suffered non-cardiac syncope and was administered intravenously 3 L of fluids for 5 hours. He underwent two echocardiographic assessments, one immediately after the finish of the race and the second 5 h later. Post-exercise fluid administration led to an increase in dimensions of all cardiac cavities, accompanied by a decrease in left ventricular (LV) end-diastolic interventricular septum thickness and posterior wall thickness of 0.1 cm. Dimensions and the respiratory profile of inferior vena cava improved after the race, reflecting alleviation of exercise-related hypovolaemia. Additionaly, LV global longitudinal strain improved, but right ventricular (RV) systolic function continued to deteriorate, mainly due to impairment of basal and medial RV free wall longitudinal strain. Study of this case offers a unique model for understanding the successive changes of cardiac structure and function following an ultra-marathon running race.

Iron therapy and severe arrhythmias in HFrEF: rationale, study design, and baseline results of the RESAFE-HF trial.

AIMS: The Iron Intravenous Therapy in Reducing the burden of Severe Arrhythmias in HFrEF (RESAFE-HF) registry study aims to provide real-word evidence on the impact of intravenous ferric carboxymaltose (FCM) on the arrhythmic burden of patients with heart failure with reduced ejection fraction (HFrEF), iron deficiency (ID), and implanted cardiac implantable electronic devices (CIEDs). METHODS AND RESULTS: The RESAFE-HF (NCT04974021) study was designed as a prospective, single-centre, and open-label registry study with baseline, 3, 6, and 12 month visits. Adult patients with HFrEF and CIEDs scheduled to receive IV FCM as treatment for ID as part of clinical practice were eligible to participate. The primary endpoint is the composite iron-related endpoint of haemoglobin ≥ 12 g/dL, ferritin ≥ 50 ng/L, and transferrin saturation > 20%. Secondary endpoints include unplanned HF-related hospitalizations, ventricular tachyarrhythmias detected by CIEDs and Holter monitors, echocardiographic markers, functional status (VO2 max and 6 min walk test), blood biomarkers, and quality of life. In total, 106 patients with a median age of 72 years (14.4) were included. The majority were male (84.9%), whereas 92.5% of patients were categorized to New York Heart Association II/III. Patients' arrhythmic burden prior to FCM administration was significant-19 patients (17.9%) received appropriate CIED therapy for termination of ventricular tachyarrhythmia in the preceding 12 months, and 75.5% of patients have frequent, repetitive multiform premature ventricular contractions. CONCLUSIONS: The RESAFE-HF trial is expected to provide evidence on the effect of treating ID with FCM in HFrEF based on real-world data. Special focus will be given on the arrhythmic burden post-FCM administration.

Brugada syndrome masked by ibutilide treatment in a patient with atrial flutter.

We present a case of Brugada syndrome in a young patient whose typical ECG pattern was 'masked' after ibutilide was administered for atrial flutter cardioversion. Ibutilide, a class III antiarrhythmic agent used for the treatment of atrial fibrillation and flutter, prolongs the action potential duration plateau phase by augmenting the slow component of the inward Na(+) current and by blocking the rapid component of the delayed rectifier potassium current. Insights into the pathophysiology of Brugada syndrome and this first-reported action of ibutilide are supplied, providing a plausible scientific basis for the masking effect of ibutilide. Furthermore, issues concerning the safety of ibutilide administration in patients with Brugada syndrome along with the importance of programmed ventricular stimulation and especially short-long-short sequence protocol in inducing ventricular fibrillation in these patients are also discussed.