Cardiac Sarcoidosis: A Comprehensive Clinical Review.

Sarcoidosis is an inflammatory multisystemic disease of unknown etiology characterized by the formation of non-caseating granulomas. Sarcoidosis can affect any organ, predominantly the lungs, lymphatic system, skin and eyes. While > 90% of patients with sarcoidosis have lung involvement, an estimated 5% of patients with sarcoidosis have clinically manifest cardiac sarcoidosis (CS), whereas approximately 25% have asymptomatic, clinically silent cardiac involvement verified by autopsy or imaging studies. CS can present with conduction disturbances, ventricular arrhythmias, heart failure or sudden cardiac death. Approximately 30% of < 60-year-old patients presenting with unexplained high degree atrioventricular (AV) block or ventricular tachycardia are diagnosed with CS, therefore CS should be strongly considered in such patients. CS is the second leading cause of death among patients affected by sarcoidosis after pulmonary sarcoidosis, therefore its early recognition is important, because early treatment may prevent death from cardiovascular involvement. The establishment of isolated CS diagnosis sometimes can be quite difficult, when extracardiac disease cannot be verified. The other reason for the difficulty to diagnose CS is that CS is a chameleon of cardiology and it can mimic (completely or almost completely) different cardiac diseases, such as arrhythmogenic cardiomyopathy, giant cell myocarditis, dilated, restrictive and hypertrophic cardiomyopathies. In this review article we will discuss the current diagnosis and management of CS and delineate the potential difficulties and pitfalls of establishing the diagnosis in atypical cases of isolated CS.

Trajectory of Diastolic Function after Heart Transplantation as Assessed by Left Atrial Deformation Analysis.

Diastolic dysfunction (DD) is a prevalent and clinically significant complication after heart transplantation (HTX). We aimed to characterize the diastolic function of HTX recipients with both short-term and long-term follow-ups by applying left atrial (LA) deformation analysis. We consecutively enrolled and followed up with 33 HTX patients. Three assessments were performed one month, 3-5 months, and 3-5 years after surgery. Beyond conventional echocardiographic measurements, apical four-chamber views optimized for speckle tracking analysis were acquired and post-processed by dedicated software solutions (TomTec AutoStrain LA and LV). Left atrial phasic functions were characterized by reservoir, conduit, and contraction strains. We categorized diastolic function according to current guidelines (normal diastolic function, indeterminate, DD). At the first assessment, nine (27%) patients were in the DD category, and eleven (33%) were indeterminate. At the second assessment, only one patient (3%) remained in the DD category and six (18%) were indeterminate. At the third assessment, 100% of patients were categorized as having normal diastolic function. LA reservoir strain gradually increased over time. LA contraction strain significantly improved from the second to the third assessment. We found a correlation between the LA reservoir strain and NT-proBNP (r = 0.40, p < 0.05). DD is prevalent immediately after HTX but rare until the end of the first postoperative quarter. Speckle tracking analysis enables the characterization of LA phasic functions that may reflect both short- and long-term changes in diastolic function and correlate with NT-proBNP.

Iron deficiency in young basketball players: Is a 100 µg/L ferritin cut-off appropriate for iron supplementation?: Results of a randomized placebo-controlled study.

BACKGROUND: Iron deficiency (ID) is one of the most common factors that may reduce sports performance, supplementation forms and doses are still not standardized in athletes. Our aim was to assess the iron status of young male basketball players and to study the effect of iron supplementation in a randomized placebo-controlled study. HYPOTHESIS: We hypothesized that due to the higher iron demand of athletes, the 100 µg/L ferritin cut-off may be appropriate to determine the non-anemic ID. METHODS: During a sports cardiology screening, questionnaires, laboratory tests, electrocardiograms, echocardiography exams, and cardiopulmonary exercise tests were performed. Athletes with ID (ferritin <100 µg/L) were randomized into iron and placebo groups. Ferrous sulfate (containing 100 mg elemental iron [II] and 60 mg ascorbic acid) or placebo (50 mg vitamin C) was administered for 3 months. All exams were repeated after the supplementation period. RESULTS: We included 65 (age 15.8 ± 1.7 years) basketball players divided into four age groups. Non-anemic ID was observed in 60 (92%) athletes. After supplementation, ferritin levels were higher in the iron group (75.5 ± 25.9 vs. 54.9 ± 10.4 µg/L, p < .01). Ferritin >100 µg/L level was achieved only in 15% of the athletes. There were no differences in performance between the groups (VO2 max: 53.6 ± 4.3 vs. 54.4 ± 5.7 mL/kg/min, p = .46; peak lactate: 9.1 ± 2.2 vs. 9.1 ± 2.6 mmol/L, p = .90). CONCLUSIONS: As a result of the 3-month iron supplementation, the ferritin levels increased; however, only a small portion of the athletes achieved the target ferritin level, while performance improvement was not detectable.

Evolving spectrum of arrhythmogenic cardiomyopathy: Implications for Sports Cardiology.

Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease, structurally characterized by progressive fibro-fatty replacement of the normal myocardium and clinically by ventricular arrhythmias (VAs). Predominantly thanks to the use of cardiac magnetic resonance, we have learnt that the spectrum of the disease encompasses not only the classical right ventricular phenotype, but also biventricular and left dominant variants. Sport activity contributes to the phenotypic expression and progression of ACM and may trigger life-threatening VAs and sudden cardiac death (SCD). We conducted a review of the literature about ACM and its implications in Sport Cardiology and summarized the main findings in this topic. Early identification of affected athletes through preparticipation screening (PPS) is fundamental but, while classical right-ventricular or biventricular phenotypes are usually suspected because of electrocardiogram (ECG) and echocardiographic abnormalities, variants with predominant left ventricular involvement are often characterized by normal ECG and unremarkable echocardiography. Usually the only manifestations of such variants are exercise-induced VAs and for this reason exercise testing may empower the diagnostic yield of the PPS. Patients with ACM are not eligible to competitive sports activity, but low-to-moderate intensity physical activity under medical supervision is possible in most cases.

Cardiopulmonary examinations of athletes returning to high-intensity sport activity following SARS-CoV-2 infection.

After SARS-CoV-2 infection, strict recommendations for return-to-sport were published. However, data are insufficient about the long-term effects on athletic performance. After suffering SARS-CoV-2 infection, and returning to maximal-intensity trainings, control examinations were performed with vita-maxima cardiopulmonary exercise testing (CPET). From various sports, 165 asymptomatic elite athletes (male: 122, age: 20y (IQR: 17-24y), training:16 h/w (IQR: 12-20 h/w), follow-up:93.5 days (IQR: 66.8-130.0 days) were examined. During CPET examinations, athletes achieved 94.7 ± 4.3% of maximal heart rate, 50.9 ± 6.0 mL/kg/min maximal oxygen uptake (V̇O2max), and 143.7 ± 30.4L/min maximal ventilation. Exercise induced arrhythmias (n = 7), significant horizontal/descending ST-depression (n = 3), ischemic heart disease (n = 1), hypertension (n = 7), slightly elevated pulmonary pressure (n = 2), and training-related hs-Troponin-T increase (n = 1) were revealed. Self-controlled CPET comparisons were performed in 62 athletes: due to intensive re-building training, exercise time, V̇O2max and ventilation increased compared to pre-COVID-19 results. However, exercise capacity decreased in 6 athletes. Further 18 athletes with ongoing minor long post-COVID symptoms, pathological ECG (ischemic ST-T changes, and arrhythmias) or laboratory findings (hsTroponin-T elevation) were controlled. Previous SARS-CoV-2-related myocarditis (n = 1), ischaemic heart disease (n = 1), anomalous coronary artery origin (n = 1), significant ventricular (n = 2) or atrial (n = 1) arrhythmias were diagnosed. Three months after SARS-CoV-2 infection, most of the athletes had satisfactory fitness levels. Some cases with SARS-CoV-2 related or not related pathologies requiring further examinations, treatment, or follow-up were revealed.

Factors influencing the SARS-CoV-2 infection and vaccination induced immune response in rheumatoid arthritis.

Background: To investigate the factors that have significant impact on the Severe Acute Respiratory Syndrome Corona Virus 2 (SARS-CoV-2) infection and vaccination induced immune response in rheumatoid arthritis (RA). Methods: Serological response was measured by quantifying anti-SARS-CoV-2 specific antibodies, while the cell-mediated response was measured by a whole-blood test quantifying the interferon (IFN)-γ response to different SARS-CoV-2-specific domains. Results: We prospectively enrolled 109 RA patients and 43 healthy controls. The median time (IQR) between the confirmed infection or the last vaccination dose and the day when samples were taken ("sampling interval") was 3.67 (2.03, 5.50) months in the RA group. Anti-Spike (anti-S) specific antibodies were detected in 94% of RA patients. Among the investigated patient related variables, age (p<0.004), sampling interval (p<0.001), the brand of the vaccine (p<0.001) and targeted RA therapy (TNF-inhibitor, IL-6 inhibitor, anti-CD20 therapy) had significant effect on the anti-S levels. After covariate adjustment TNF-inhibitor therapy decreased the anti-S antibody concentrations by 80% (p<0.001). The same figures for IL-6 inhibitor and anti-CD20 therapy were 74% (p=0.049) and 97% (p=0.002), respectively. Compared to subjects who were infected but were not vaccinated, the RNA COVID-19 vaccines increased the anti-S antibody levels to 71.1 (mRNA-1273) and 36.0 (BNT162b2) fold (p<0.001). The corresponding figure for the ChAdOx1s vaccine is 18.1(p=0.037). Anti-CCP (anti-cyclic citrullinated peptides) positive patients had 6.28 times (p= 0.00165) higher anti-S levels, than the anti-CCP negative patients. Positive T-cell response was observed in 87% of the healthy volunteer group and in 52% of the RA patient group. Following vaccination or infection it declined significantly (p= 0.044) but more slowly than that of anti-S titer (6%/month versus 25%). Specific T-cell responses were decreased by 65% in patients treated with anti-CD20 therapy (p=0.055). Conclusion: Our study showed that the SARS-CoV-2-specific antibody levels were substantially reduced in RA patients treated with TNF-α-inhibitors (N=51) and IL-6-inhibitor (N=15). In addition, anti-CD20 therapy (N=4) inhibited both SARS-CoV-2-induced humoral and cellular immune responses. Furthermore, the magnitude of humoral and cellular immune response was dependent on the age and decreased over time. The RNA vaccines and ChAdOx1s vaccine effectively increased the level of anti-S antibodies.

Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy.

Cardiac sarcoidosis (CS) is a chameleon of cardiology, and it can mimic different cardiac diseases; among them is arrhythmogenic cardiomyopathy (ACM). We admitted a 70-year-old female patient with heart failure symptoms in 2015, who fulfilled all major ECG and non-invasive imaging criteria of biventricular ACM. She was well with the recommended medications for 3 years, showing only isolated cardiac involvement, but in 2018, cervical and mediastinal lymphadenopathy appeared and cervical lymph node core biopsy histology, bronchoalveolar lavage flow cytometry strongly suggested extracardiac sarcoidosis. Therefore, our suspicion was that sarcoidosis is responsible for the cardiac involvement, which was not confirmed by PET-CT and gallium scintigraphy examinations. At the end of 2018, she died in septicaemia with multiorgan failure, and only autopsy verified her CS. A new ECG algorithm published in 2021 for the differential diagnosis of CS and biventricular ACM, when applied on her ECGs recorded in 2015, suggested the diagnosis of CS.

Cardiac Amyloidosis with Normal Wall Thickness: Prevalence, Clinical Characteristics and Outcome in a Retrospective Analysis.

Background: Cardiac amyloidosis (CA) is a rare, progressive, infiltrative cardiac disease. Light chain (AL) and transthyretin (ATTR) amyloidosis are in the background in almost all cases. New, easily available diagnostic tools and recently introduced novel therapies for both types of CA put this disease into the field of interest. Increased left ventricular wall thickness (IWT) detected by echocardiography is generally thought to be a necessary part of the diagnosis. We aimed to determine the proportion of CA patients without IWT, and to define the clinical characteristics of this cohort. Methods: In an academic tertiary center for CA, we identified patients diagnosed and treated for CA between January 2009 and February 2022. In a retrospective analysis we defined the proportion of patients with (≥12 mm) and without (<12 mm) IWT, and described their clinical features. Results: We identified 98 patients suitable for the analysis. In total, 70 had AL and 27 ATTR CA; 89 patients had CA with IWT and 9 patients (9%) had CA without IWT. All non-IWT patients had AL type CA. Both group of patients had clinically significant disease, which is supported by the relevant elevation in cardiac biomarker levels. There was no difference between the outcome of the two groups. Conclusion: Patients without IWT form a relevant subgroup among those with CA. Our results suggest that diagnostic algorithms and criteria should take these individuals into consideration, and, therefore, give them access to effective treatments.

Potential clinical relevance of cardiac magnetic resonance to diagnose cardiac light chain amyloidosis.

BACKGROUND: While patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging. Cardiac magnetic resonance (CMR) analyses play an essential role in the differential diagnosis of cardiomyopathies; however, limited data are available from cardiac AL-Amyloidosis. Hence, the purpose of the present study was to analyze the potential role of CMR in the detection of cardiac AL-amyloidosis. METHODS: We included 35 patients with proved cardiac AL-amyloidosis and two control groups constituted by 330 patients with hypertrophic cardiomyopathy (HCM) and 70 patients with arterial hypertension (HT), who underwent CMR examination. The phenotype and degree of left ventricular (LV) hypertrophy and the amount and pattern of late gadolinium enhancement (LGE) were evaluated. In addition, global and regional LV strain parameters were also analyzed using feature-tracking techniques. Sensitivity and specificity of several CMR parameters were analyzed in diagnosing cardiac AL-amyloidosis. RESULTS: The sensitivity and specificity of diffuse septal subendocardial LGE in diagnosing cardiac AL-amyloidosis was 88% and 100%, respectively. Likewise, the sensitivity and specificity of septal myocardial nulling prior to blood pool was 71% and 100%, respectively. In addition, a LV end-diastolic septal wall thickness ≥ 15 mm had an optimal diagnostic performance to differentiate cardiac AL-amyloidosis from HT (sensitivity 91%, specificity 89%). On the other hand, a reduced global LV longitudinal strain (< 15%) plus apical sparing (apex-to-base longitudinal strain > 2) had a very low sensitivity (6%) in detecting AL-Amyloidosis, but with very high specificity (100%). CONCLUSIONS: The findings from this study suggest that CMR could have an optimal diagnostic performance in the diagnosis of cardiac AL-amyloidosis. Hence, further larger studies are warranted to validate the findings from this study.

Prognostic significance of cardiac magnetic resonance-based markers in patients with hypertrophic cardiomyopathy.

The prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.

Prognosis and clinical characteristics of patients with early ventricular fibrillation in the 6-week guideline-offered time period: is it safe to wait 6 weeks with the assessment? (results from the VMAJOR-MI Registry).

BACKGROUND: The most common, potentially fatal complication following an acute myocardial infarction (AMI) is early ventricular fibrillation (EVF). According to the guidelines, the assessment of implanting an implantable cardioverter defibrillator (ICD) is sufficient 6 weeks after the event, in patients with reduced left ventricular ejection fraction (LVEF), regardless of VF. The present study aimed to evaluate the 6-week prognosis of patients surviving an EVF. We divided the patients in two group based on their general condition at the time they left the hospital. We investigated the clinical characteristics of patients discharged in good general health but still dying within 6 weeks. METHODS: The present study comprised 12,270 patients with AMI following their primary revascularization in the first 12 h of symptom onset. Five hundred and forty-seven of them suffered EVF due to the AMI. Clinical and 6-week mortality data were examined. RESULTS: Poor general condition correlates with multiple comorbidities, higher troponin levels, more severe complications after the event. Patients leaving in good condition thought to be low risk, from dying. But low LVEF, high blood sugar, high cardiac biomarker level, poor renal function elevates the risk of dying within 6 weeks. However, there is no difference in clinical characteristics between EVF- cases and EVF+ cases in good condition who dies within 6 weeks. CONCLUSIONS: According to our study we can select patients who are safe in the critical 6-week period and those who need closer follow-up despite leaving in good general condition.

Relationship between Cardiac Remodeling and Exercise Capacity in Elite Athletes: Incremental Value of Left Atrial Morphology and Function Assessed by Three-Dimensional Echocardiography.

BACKGROUND: Data are scarce regarding left atrial (LA) adaptation to regular physical exercise. The aim of this study was to examine left ventricular (LV) and also LA morphologic and functional remodeling in elite athletes using three-dimensional (3D) echocardiography. METHODS: In this retrospective analysis, the study group consisted of 138 elite athletes (mean age, 20 ± 4 years; 62% men) and 50 sedentary control subjects. Electrocardiographically gated full-volume 3D data sets were obtained for offline analysis using dedicated software for 3D LA and LV measurements. Body surface area-indexed LA maximal volume (LAVmax) and LV end-diastolic volume were determined. LA total emptying fraction, LA passive and LA active emptying fraction, and LV global longitudinal strain were also calculated. Athletes also underwent cardiopulmonary exercise testing to determine peak oxygen uptake. RESULTS: Athletes demonstrated higher 3D LAVmax (32 ± 6 vs 26 ± 8 mL/m2) and indexed LV end-diastolic volume (85 ± 12 vs 62 ± 10 mL/m2) compared with control subjects (P < .001 for both). Functional measures of the left ventricle and left atrium, such as the absolute value of 3D LV global longitudinal strain (19 ± 2% vs 22 ± 2%), LA total emptying fraction (58 ± 6% vs 64 ± 6%), and active emptying fraction (24 ± 10% vs 32 ± 10%) were lower in athletes (P < .001 for all). Male athletes had higher indexed LV end-diastolic volume compared with female athletes (89 ± 13 vs 80 ± 8 mL/m2, P < .001), but LAVmax did not differ between genders (32 ± 6 vs 33 ± 5 mL/m2, P = .18). Besides heart rate, gender, and body surface area, 3D LAVmax, LV global longitudinal strain, and LA passive emptying fraction were independent predictors of peak oxygen uptake. CONCLUSIONS: Regular physical exercise results in marked LA and LV remodeling with considerable gender differences as explored by 3D echocardiography. In contrast with various cardiovascular diseases, more pronounced LA dilation and lower resting functional measures are associated with better exercise performance.

Coexistence of aortic valve stenosis and cardiac amyloidosis: echocardiographic and clinical significance.

BACKGROUND: Left ventricular hypertrophy and diastolic dysfunction are common echocardiographic features of both aortic valve stenosis (AS) and cardiac amyloidosis (CA). These two different entities therefore may mask each other. From recent years, there is a growing body of evidence about the relatively high incidence of wild-type transthyretin (wtTTR) amyloidosis in AS, but there are scarce data on the prevalence of AS in CA, particularly in AL-type amyloidosis. The echocardiographic approach to these patients is not obvious, and not evidence based. We aimed to study the prevalence, severity, and type of AS in patients with CA and also to evaluate the potential of echocardiography in the diagnostic process. METHODS: Between January 2009 and January 2019, we retrospectively analyzed the clinical and echocardiographic data, and the echocardiographic work up of 55 consecutive CA patients. RESULTS: 80% of our CA patients had AL amyloidosis. We identified 5 patients (9%) with moderate to severe AS: two with moderate AS and three with low-flow, low-grade AS (LFLG AS). Further analysis of the latter three patients with dobutamine stress echocardiography revealed pseudo-severe LFLG AS in two, and true-severe AS in one patient. CONCLUSION: The prevalence of moderate to severe AS is 9% in our population of CA patients, the majority of whom have AL amyloidosis. Dobutamine echocardiography seems to be appropriate for the further characterization of patients with LFLG AS, even with normal ejection fraction.

The effect of contrast agents on left ventricular parameters calculated by a threshold-based software module: does it truly matter?

The acquisition of short-axis (SA) cine magnetic resonance (MR) images after the administration of contrast agent (CA) is a common, time-saving technique, but a decreased difference in the blood-myocardium contrast on these steady-state free precession (SSFP) cine scans could change the calculated parameters when using threshold-based papillary and trabecular muscle (PTM) quantification. We studied the effect of CA on the parameters calculated from pre- and post-CA SA cine images in noncompaction cardiomyopathy (NC-CMP) and healthy (H) participants using a threshold-based module. A total of 39 individuals (20 patients and 19 healthy) were included prospectively in this study. After the pre-CA SA images were acquired, i.v. gadobutrol (GA) or gadobenate dimeglumine (GD) (GA vs. GD: NC-CMP = 12 vs. 8; C = 12 vs. 7) was administered, and SA scans were repeated after two minutes. A threshold-based PTM software was used for postprocessing. Pre-CA and post-CA SA images were analyzed, and the parameters were compared in both the NC-CMP and H groups. The left ventricular volumes were significantly larger, while the left ventricular myocardial (LVmass) and trabecular mass (LVtrab) values were significantly smaller on the post-CA scans (NC-CMP: pre-CA vs. post-CA, EDV: 74.0 ± 13.6 vs. 81.1 ± 16.3 ml/m2, ESV: 25.3 ± 7.3 vs. 30.1 ± 11.2 ml/m2, LVmass-ED: 82.5 ± 17.5 vs. 75.7 ± 15.9 g/m2, LVtrab-ED: 25.0 ± 6.6 vs. 18.9 ± 4.7 g/m2; Healthy: preCA vs. post-CA, EDV: 69.7 ± 11.9 vs. 72.2 ± 10.7 ml/m2, ESV: 22.6 ± 5.7 vs. 23.9 ± 6.3 ml/m2, LVmass-ED: 71.3 ± 13.6 vs. 68.7 ± 13.9 g/m2, LVtrab-ED: 19.4 ± 2.6 vs. 16.2 ± 3.0 g/m2; p < 0.05). The decreased blood-myocardium contrast difference on post-CA SSFP SA cine images leads to altered cardiac parameters when using threshold-based software for evaluation.

Cardiac magnetic resonance based deformation imaging: role of feature tracking in athletes with suspected arrhythmogenic right ventricular cardiomyopathy.

Both, arrhythmogenic right ventricular cardiomyopathy (ARVC) and regular training are associated with right ventricular (RV) remodelling. Cardiac magnetic resonance (CMR) is given an important role in the diagnosis of ARVC in current task force criteria (TFC), however, they contain no cut-off values for athletes. We aimed to confirm the added value of feature tracking and to provide new cut-off values to differentiate between ARVC and athlete's heart. Healthy athletes with training of minimal 15 h/week (n = 34), patients with definite ARVC (n = 34) and highly trained athletes with ARVC (n = 8) were examined by CMR. Left and right ventricular volumes and masses were determined. Global right and left ventricular, and regional strain analysis for the RV free wall was performed using feature tracking on balanced steady-state free precession cine images. 94% of healthy athletes showed RV dilatation of the proposed TFC, 14.7% showed RV ejection fraction (RVEF) between 45-50%, none of them had RVEF < 45%. Although RVEF showed the highest accuracy in differentiating between athlete's heart and ARVC, only 37.5% of athletes with ARVC showed RVEF < 45%. The only parameters falling in the pathological range (based on our established cut-off values: > - 25.6 and > - 1.4, respectively) in all athletes with ARVC were the strain and strain rate of the midventricular RV free wall. Establishing RVEF and RV strain analysis provides an important tool to distinguish ARVC from athlete's heart. CMR based regional strain and strain rate values may help to identify ARVC even in highly trained athletes with preserved RVEF.

Uncommon presentation of a rare tumour - incidental finding in an asymptomatic patient: case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours.

BACKGROUND: A solitary fibrous tumour is a rare, mainly benign spindle cell mesenchymal tumour most commonly originating from the pleura. An intrapericardial location of a solitary fibrous tumour is extremely unusual. We present a case of an asymptomatic patient with a slow-growing massive benign cardiac solitary fibrous tumour. CASE PRESENTATION: A 37-year-old asymptomatic female patient was referred to our hospital with an enlarged cardiac silhouette found on her screening chest X-ray. The echocardiographic examination revealed pericardial effusion and an inhomogeneous mobile mass located in the pericardial sac around the left ventricle. Cardiac magnetic resonance (MRI) examination showed an intrapericardial, semilunar-shaped mass attached to the pulmonary trunk with an intermediate signal intensity on proton density-weighted images and high signal intensity on T2-weighted spectral fat saturation inversion recovery images. First-pass perfusion and early and late gadolinium-enhanced images showed a vascularized mass with septated, patchy, inhomogeneous late enhancement. Coronary computed tomography angiography revealed no invasion of the coronaries. Based on the retrospectively analysed screening chest X-rays, the mass had started to form at least 7 years earlier. Complete resection of the tumour with partial resection of the pulmonary trunk was performed. Histological evaluation of the septated, cystic mass revealed tumour cells forming an irregular patternless pattern; immunohistochemically, the cells tested positive for vimentin, CD34, CD99 and STAT6 but negative for keratin (AE1-AE3), CD31 and S100. Thus, the diagnosis of an intrapericardial solitary fibrous tumour was established. There has been no recurrence for 3 years based on the regular MRI follow-up. CONCLUSION: Intrapericardial SFTs, showing slow growth dynamics, can present with massive extent even in completely asymptomatic patients. MRI is exceedingly useful for characterizing intrapericardial masses, allowing precise surgical planning, and is reliable for long-term follow up.

Severe mitral regurgitation and heart failure due to caseous calcification of the mitral annulus.

Caseous calcification is a rare form of mitral annular calcification. Echocardiography reveals an echodense mass in the inferior mitral annulus with smooth borders and an echolucent inner core. We present a case where caseous calcification of the mitral annulus caused severe mitral regurgitation, atrial fibrillation and heart failure. Transthoracic echocardiography, transesophageal echocardiography, cardiac magnetic resonance and computed tomography were performed and ensured the diagnosis. The mass was surgically removed and a prosthetic valve was implanted. We conclude that caseous calcification of the mitral annulus should be considered not only in the differential diagnosis of cardiac masses but also in the background of mitral regurgitation, atrial fibrillation and heart failure. This case also represents the usefulness of multimodal imaging in identifying cardiac masses.

[Arrhytmogenic right ventricle--prognostic significance of exercise test]. / Arrhythmogen jobb kamrai dysplasia--a terheléses vizsgálat prognosztikai jelentosége panaszmentes egyénben.

UNLABELLED: The authors summarize the present knowledge on arrhythmogenic right ventricular cardiomyopathy/dysplasia. Limited data are available about natural history of asymptomatic patients with arrhythmogenic right ventricle cardiomyopathy/dysplasia, who have a ventricular tachycardia during exercise test. A 25-year old female patient was treated with osteosynthesis because of ankle injury. Cardiology consultation was performed because of an abnormal ECG. Physical examination was normal. ECG showed a normal sinus rhythm, left axis deviation, negative T waves in leads II, III, aVF and V2-V6. Chest X-ray and laboratory findings were normal. Echocardiography showed normal left ventricular ejection fraction along with inferior akinesis and dilated right ventricle. Bicycle exercise test revealed a good exercise tolerability (9 MET), and after sporadic ventricular extra systoles ventricular tachycardia developed lasting for 3 minutes, which spontaneously stopped after aborting the test and performed abdominal strain. MRI was performed which has shown normal left ventricular size, wall motion and ejection fraction and depressed right ventricle function (ejection fraction 31.6%) enlarged right ventricular end-systolic and diastolic volumes, hypo-akinetic regions without aneurysm and bulging. No contrast enhancement was seen in the thin right ventricular wall. According to abnormal ECG and MRI findings arrhythmogenic right ventricle cardiomyopathy/dysplasia was diagnosed. No ICD implantation was indicated because the patient was asymptomatic, and no sudden cardiac death occurred in the family. Three month later the patient was found dead. At autopsy the right ventricular chamber was markedly enlarged, with multiple translucent areas of fatty accumulation accompanied with extended myocytes loss. There was a characteristic triangle dysplasia: the inflow, outflow tracts and apical areas. The coronaries were free of atherosclerosis. Mallory's phosphotungstic acid-hematoxilin stain demonstrated the presence of fibrosis within the scattered myocardium. CONCLUSION: malignant ventricular arrhythmia provoked by exercise test in an asymptomatic arrhythmogenic right ventricle cardiomyopathy/dysplasia patient with negative family history should be an indication for ICD implantation.

Mesenteric vascular dysfunction after cardiopulmonary bypass with cardiac arrest is aggravated by coexistent heart failure.

Although patients suffering from heart failure (HF) have an increased incidence of nonocclusive mesenteric ischemia after opened heart surgery, the impact of cardiopulmonary bypass with cardiac arrest (CPB) on mesenteric vascular circulation in such situation remains unexplored. Therefore, the present study investigates the effects of CPB on mesenteric vascular reactivity, regional metabolism, and oxidative stress in an experimental model of HF. Volume-overload HF was induced in six dogs by bilateral femoral arteriovenous fistula. Six sham-operated dogs were used as controls. Eight weeks later, the short-term effects of 90 min of CPB were assessed in vivo during acute experiments. The significant increase in left ventricular end-diastolic volume in HF animals did not influence the vasodilator response of the superior mesenteric artery to acetylcholine (ACH) and nitroprusside (SNP) under baseline conditions. However, reduced mesenteric oxygen delivery, increased oxygen extraction, and lactate release were found during CPB in the HF group. In addition, an increased free radical production was assessed in the HF group during (89 +/- 23 x 10 relative light units [RLU]) and after CPB (93 +/- 15 x 10 RLU) compared with controls (45 +/- 15 and 49 +/- 7 x 10 RLU, respectively). Finally, 90 min of CPB led to a more pronounced decrease of ACH- (-22% +/- 5% vs. -42% +/- 9%, P < 0.05) and SNP- (-14% +/- 4% vs. -50% +/- 7%, P < 0.002) induced mesenteric vasodilations in the HF group compared with controls. We conclude that coexistent HF significantly enhances the pathological effects of CPB on the mesenteric vascular circulation by additionally altering endothelial and smooth muscle vascular function consequent to augmented oxidative stress.

Effect of incessant ventricular tachyarrhythmias on serum endothelin and big-endothelin levels.

Endothelin-1 (ET-1) is a potent endogenous arrhythmogenic substance. The aim of our study was to investigate the changes of serum ET-1 and big-endothelin levels in patients suffering from spontaneous, incessant ventricular tachyarrhythmias. The 11 consecutive patients' (mean age, 59 +/- 11 years) underlying diseases were ischemic heart disease, valvular heart disease, dilated cardiomyopathy, primary electrical disease, and arrhythmogenic right ventricular dysplasia in five cases, three cases, one case, one case, and one case, respectively. The mean ejection fraction was 39 +/- 14%, New York Heart Association functional status was I, II, and III in two cases, four cases, and five cases, respectively. Ventricular tachycardias (VT) were detected in five patients, ventricular fibrillation (VF) in three patients, and VT + VF in three patients. VTs terminated spontaneously in two cases. Six patients required multiple external cardioversion/defibrillation shocks, while implantable cardioverter defibrillators terminated all sustained arrhythmias successfully in four cases. Blood samples were collected during arrhythmias and 24 hours (control) following the last VT/VF episode. Serum ET-1 and big-endothelin levels were measured with western blot analysis after immunoprecipitation. Serum ET-1 and big-endothelin levels were significantly higher during the last VT/VF compared with the control period (ET-1, 65.8 +/- 26.8 fmol/mL versus 53.9 +/- 22.3 fmol/mL, P < 0.05; big-endothelin, 115.2 +/- 39.3 fmol/mL versus 89.2 +/- 25.1 fmol/mL, P < 0.05). There was a negative correlation between the age and big-endothelin level measured at both times (during VT/VF, r = 0.94, P < 0.05; control, r = 0.91, P < 0.05). In conclusion, serum big-endothelin and ET-1 levels were significantly higher during incessant VT/VF, which can be a cause of multiple arrhythmia recurrence.