A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.

Background: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. Methods and results: 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (p = 0.9) and between ablated and non-ablated EA individuals (p = 0.89). Conclusions: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.

A distinct septal pattern of late gadolinium enhancement specific for COVID-induced myocarditis: A multicenter cardiovascular magnetic resonance study.

BACKGROUND: COVID-19 is a great medical challenge as it provokes acute respiratory distress and has pulmonary manifestations and cardiovascular (CV) consequences. AIMS: This study compared cardiac injury in COVID-19 myocarditis patients with non-COVID-19 myocarditis patients. METHODS: Patients who recovered from COVID-19 were scheduled for cardiovascular magnetic resonance (CMR) owing to clinical myocarditis suspicion. The retrospective non-COVID-19 myocarditis (2018-2019) group was enrolled (n = 221 patients). All patients underwent contrast-enhanced CMR, the conventional myocarditis protocol, and late gadolinium enhancement (LGE). The COVID study group included 552 patients at a mean (standard deviation [SD]) age of 45.9 (12.6) years. RESULTS: CMR assessment confirmed myocarditis-like LGE in 46% of the cases (68.5% of the segments with LGE <25% transmural extent), left ventricular (LV) dilatation in 10%, and systolic dysfunction in 16% of cases. The COVID-19 myocarditis group showed a smaller median (interquartile range [IQR]) LV LGE (4.4% [2.9%-8.1%] vs. 5.9% [4.4%-11.8%]; P <0.001), lower LV end-diastolic volume (144.6 [125.5-178] ml vs. 162.8 [136.6-194] ml; P <0.001), limited functional consequence (left ventricular ejection fraction, 59% [54.1%-65%] vs. 58% [52%-63%]; P = 0.01), and a higher rate of pericarditis (13.6% vs. 6%; P = 0.03) compared to non-COVID-19 myocarditis. The COVID-19-induced injury was more frequent in septal segments (2, 3, 14), and non-COVID-19 myocarditis showed higher affinity to lateral wall segments (P <0.01). Neither obesity nor age was associated with LV injury or remodeling in subjects with COVID-19 myocarditis. CONCLUSIONS: COVID-19-induced myocarditis is associated with minor LV injury with a significantly more frequent septal pattern and a higher pericarditis rate than non-COVID-19 myocarditis.

Myocardial Injury on CMR in Patients With COVID-19 and Suspected Cardiac Involvement.

BACKGROUND: Myocardial injury in patients with COVID-19 and suspected cardiac involvement is not well understood. OBJECTIVES: The purpose of this study was to characterize myocardial injury in a multicenter cohort of patients with COVID-19 and suspected cardiac involvement referred for cardiac magnetic resonance (CMR). METHODS: This retrospective study consisted of 1,047 patients from 18 international sites with polymerase chain reaction-confirmed COVID-19 infection who underwent CMR. Myocardial injury was characterized as acute myocarditis, nonacute/nonischemic, acute ischemic, and nonacute/ischemic patterns on CMR. RESULTS: In this cohort, 20.9% of patients had nonischemic injury patterns (acute myocarditis: 7.9%; nonacute/nonischemic: 13.0%), and 6.7% of patients had ischemic injury patterns (acute ischemic: 1.9%; nonacute/ischemic: 4.8%). In a univariate analysis, variables associated with acute myocarditis patterns included chest discomfort (OR: 2.00; 95% CI: 1.17-3.40, P = 0.01), abnormal electrocardiogram (ECG) (OR: 1.90; 95% CI: 1.12-3.23; P = 0.02), natriuretic peptide elevation (OR: 2.99; 95% CI: 1.60-5.58; P = 0.0006), and troponin elevation (OR: 4.21; 95% CI: 2.41-7.36; P < 0.0001). Variables associated with acute ischemic patterns included chest discomfort (OR: 3.14; 95% CI: 1.04-9.49; P = 0.04), abnormal ECG (OR: 4.06; 95% CI: 1.10-14.92; P = 0.04), known coronary disease (OR: 33.30; 95% CI: 4.04-274.53; P = 0.001), hospitalization (OR: 4.98; 95% CI: 1.55-16.05; P = 0.007), natriuretic peptide elevation (OR: 4.19; 95% CI: 1.30-13.51; P = 0.02), and troponin elevation (OR: 25.27; 95% CI: 5.55-115.03; P < 0.0001). In a multivariate analysis, troponin elevation was strongly associated with acute myocarditis patterns (OR: 4.98; 95% CI: 1.76-14.05; P = 0.003). CONCLUSIONS: In this multicenter study of patients with COVID-19 with clinical suspicion for cardiac involvement referred for CMR, nonischemic and ischemic patterns were frequent when cardiac symptoms, ECG abnormalities, and cardiac biomarker elevations were present.

Protective role of N-acetylcysteine and Sulodexide on endothelial cells exposed on patients' serum after SARS-CoV-2 infection.

Severe acute respiratory syndrome coronavirus-2 causes hyperinflammation and activation of coagulation cascade and, as a result, aggravates endothelial cell dysfunction. N-acetylcysteine and Sulodexide have been found to mitigate endothelial damage. The influence on coronary artery endothelial cells of serum collected after 4 ± 1 months from coronavirus infection was studied. The concentrations of serum samples of interleukin 6, von Willebrand Factor, tissue Plasminogen Activator, and Plasminogen Activator Inhibitor-1 were studied. The cultures with serum of patients after coronavirus infection were incubated with N-acetylcysteine and Sulodexide to estimate their potential protective role. The blood inflammatory parameters were increased in the group of cultures incubated with serum from patients after coronavirus infection. Supplementation of the serum from patients after coronavirus infection with N-acetylcysteine or Sulodexide reduced the synthesis of interleukin 6 and von Willebrand Factor. No changes in the synthesis of tissue Plasminogen Activator were observed. N-acetylcysteine reduced the synthesis of Plasminogen Activator Inhibitor-1. N-acetylcysteine and Sulodexide increased the tPA/PAI-1 ratio. N-acetylcysteine may have a role in reducing the myocardial injury occurring in the post-COVID-19 syndrome. Sulodexide can also play a protective role in post-COVID-19 patients.

Left, but not right, ventricular status determines heart failure in adults with Ebstein anomaly - A case-control study based on magnetic resonance.

BACKGROUND: Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. We evaluate the size and function of both ventricles in patients with EA in cardiac magnetic resonance (CMR); to assess their association with the clinical markers of HF. METHODS: Study group: 37 unoperated adults with EA (mean age 43.0 ± 14.4y, 21[56.8%] males). CONTROLS: 25 volunteers (mean age 39.9 ± 10.9y, 15[60%] males). Study protocol included: CMR [ejection fraction (EF), end-diastolic (EDVind) and stroke volumes (SVind) indexed by body surface area]; cardiopulmonary test (peak VO2, %peak VO2, VE/VCO2 slope). RESULTS: Size and systolic function of LV were reduced comparing to the controls [LVEDVind (ml/m2): 63.7(range 38.7-94.2) vs. 79.3(48.7-105.1), p < 0.001; LV SVind (ml/m2): 35.8(22.9-55.1) vs. 49.2(37.8-71.7), p < 0.0001; LVEF(%): 58.3(34-70.5) vs. 62.0(52.0-77.0), p = 0.009]. RV was enlarged comparing to the controls [RVEDVind (ml/m2): 124.3(52.8-378.9) vs. 83.0(64.0-102.0), p < 0.0001) with impaired systolic function (RV SVind (ml/m2): 22.7(11.1-74.1) vs. 48.0(37.8-71.7), p < 0.0001; RVEF(%): 38.0(21.0-66.1) vs. 59.0(49.0-69.0), p < 0.0001). A significant correlation was found between LVEDVind vs. peakVO2 (r = 0.52, p = 0.001); LV SVind vs. peakVO2 (r = 0.47,p = 0.005). There was no correlation between the right ventricular status and exercise capacity. CONCLUSIONS: In adults with Ebstein anomaly the size of left ventricle is reduced, right ventricle is enlarged; the function of both is impaired. Abnormal exercise capacity is associated with left ventricular status. Ventricular interdependence probably plays a role in heart failure pathomechanism.

Concomitance of COVID-19 and legionnaires' disease - a case series.

Coronavirus disease-2019 (COVID-19) and legionnaires disease (LD) caused by Gram-negative water-born bacteria Legionella pneumophila show certain similarities, including a predisposition to pulmonary involvement and extrapulmonary manifestations in some of the patients infected. One disease can mimic the other, both can rarely coexist. CASE SERIES REPORT: The authors describe 7 such cases (5 females), aged 51-90 years (mean 69.7 years) detected while screening 133 subjects with moderate to severe pneumonia and confirmed COVID- 19, which constituted 5.3% of the patients in whom urinary antigen test (UAT) for L. pneumophila was performed. The patients had multiple concomitant disorders: hypertension (6), heart failure (4), diabetes (4), obesity (4), coronary heart disease (3), chronic kidney disease (3), chronic obstructive pulmonary disease (3), anemia (3). Positive UAT was obtained at admission in 4 patients, and on 3rd, 11th and 14th days of hospitalization in the remaining 3 patients. One patient also had positive UAT for Streptococcus pneumoniae. We analyzed: radiological imaging, laboratory data (CRP, interleukin-6, procalcitonin, troponin I, BNP), ECG, echocardiography, treatment and outcome. Three patients required a modification of initial antibiotic therapy, two developed Clostridioides difficile infection. The duration of hospitalization ranged from 13 to 59 days (mean 24.3 days); two patients died. CONCLUSIONS: The authors suggest that the coexistence of COVID- 19 and LD may result in prolonged hospitalization, in increased mortality risk and in subsequent cardiovascular complications, including takotsubo syndrome (TTS) which was found in 2 cases, both presented as focal TTS (fTTS).

Marked self-limiting sinus bradycardia in COVID-19 patients not requiring therapy in the intensive care unit - case series report.

Cardiovascular complications of the COVID-19 comprise cardiac arrhythmias, including sinus bradycardia (SB). CASE REPORTS: The authors present clinical data of 19 hospitalized patients (12 males), aged 20-73 years, with marked (less than 45/min during daily hours) self-limiting SB. None of them had SB at admission or earlier, none had used cardiovascular medications potentially decreasing the heart rate. Pulmonary involvement was severe in 4, moderate in 13 and mild in 2 patients; 14 needed oxygen therapy (4 using high flow oxygen equipment), none required treatment in the intensive care unit. All patients were given low molecular weight heparin in a prophylactic dose, 13 intravenous ceftriaxone, 12 dexamethasone, 8 convalescent plasma. Before SB appearance, 12 patients were treated with remdesivir (3 patients did not receive a full planned dose) and 2 with tocilizumab. SB appeared suddenly on day 5-14 from the onset of the disease, with a minimal heart rate of 32-44/min and in 3 cases it was mildly symptomatic; 2 of those received ad-hoc atropine, one orciprenaline. Interleukin-6 (Il-6) and C-reactive protein (CRP) concentrations at SB onset were significantly lower than at admission (9.3 vs 70.0 pg/ml and 16.8 vs 98.5 mg/l, respectively). Cardiac troponin I was slightly elevated in 2 patients. ECG morphology abnormalities (transient negative T waves or ST depression) were found in 4 males. All subjects had normal left ventricular ejection fraction; in 5 echocardiography revealed small pericardial effusion; in 10 patients, longitudinal strain was also studied: reginal abnormalities were found in all of them, particularly in basal segments. SB lasted 3-11 days and was reversible in all patients; none required temporary stimulation. The COVID-19 course was favorable in all patients; they were stable at discharge. During 4-12 months of posthospital observation, including clinical features, control ECG and 24-hour Holter monitoring, none of the patients was qualified for pacemaker implantation.

Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic vs Magnetic Resonance Assessments of the Celermajer Index.

BACKGROUND: The severity score of Ebstein anomaly (EA) that corresponds to clinical status is still under research, with the Celermajer index (Cel-ind) being one of those. The agreement between echocardiographic and cardiac magnetic resonance (CMR) assessment of Cel-ind is not known. We determined the agreement between echocardiography- and CMR-derived Cel-ind and its relationship with heart failure markers. METHODS: A total of 37 unoperated adults with EA (mean age, 43.0 ± 14.4 years) underwent echocardiography, CMR, and cardiopulmonary tests. For the Cel-ind, end-diastolic areas in echocardiography or end-diastolic volumes in CMR were used according to the following formula: Cel-ind = (right atrium + atrialized right ventricle)/(functional right ventricle + left atrium + left ventricle). On the basis of this assumption, patients were classified as follows: grade 1 = Cel-ind < 0.5, grade 2 = 0.5 to 0.99, grade 3 = 1.0 to 1.49, grade 4 > 1.5. The agreement between echocardiographic and CMR was determined with the intraclass correlation coefficient or Cohen's kappa (<0.2 poor agreement; 0.2-0.4 fair agreement; 0.4-0.6 moderate agreement; 0.6-0.8 good agreement; 0.8-1.0 very good agreement). RESULTS: The median echoCel-ind was 0.9 (range, 0.4-2.3), and the median cmrCel-ind was 0.7 (range, 0.3-5.3). Grade 1 or 2 was found in 19 patients (51.3%) by echocardiography and in 27 patients (72.9%) by CMR. The agreement between imaging methods was only fair (kappa = 0.39, P = 0.002) for the 4-grade classification and moderate (intra-class correlation coefficient = 0.43; 95% confidence interval, 0.13-0.66) for Cel-ind calculation. Significant correlations between Cel-ind in CMR and cardiopulmonary parameters were found (for peak oxygen uptake: R = -0.35, P = 0.034; for the ventilation/carbon dioxide slope: R = 0.46, P = 0.005). Neither of them correlated with echocardiographic severity score. CONCLUSIONS: The agreement between echocardiographic and CMR assessment of the Cel-ind is at most moderate; echocardiography usually overestimates, but rarely underestimates, EA severity. Cel-ind by CMR seems to be more valuable, because it is associated with heart failure markers.

The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome.

Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged 13.9 ± 2.2 years, were studied. CMR was performed in 39 patients and angio-MR in 36. Echocardiography was performed in all patients. The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the ascending aorta (AA) and aortic arch, present in 16 patients (45.7%), a bicuspid aortic valve (BAV), present in 16 patients (41.0%), and partial anomalous pulmonary venous return (PAPVR), present in six patients (17.1%). Aortic dilatation (Z-score > 2) was mostly seen at the sinotubular junction (STJ) (15 patients; 42.8%), the AA (15 patients; 42.8%), the thoracoabdominal aorta at the level of a diaphragm (15 patients; 42.8%), and the transverse segment (14 patients; 40.0%). An aortic size index (ASI) above 2.0 cm/m2 was present in six patients (17.1%) and above 2.5 cm/m2 in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (Z-score < -2) in 10 (25.6%), 9 (23.1%), and 8 patients (20.5%), respectively. A webbed neck was correlated with the presence of vascular anomalies (p = 0.006). The age and body mass index (BMI) were correlated with the diameter of the aorta. Patients with BAV had a greater aortic diameter at the ascending aorta (AA) segment (p = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio (p = 0.002; r = 0.49). There was a significant correlation between the right ventricular (p = 0.002, r = 0.46) and aortic diameters at the STJ segment (p = 0.0047, r = 0.48), as measured by echocardiography and CMR. Magnetic resonance can identify cardiovascular anomalies, dilatation of the aorta, pericardial fluid, and functional impairment of the ventricles not detected by echocardiography. BMI, age, BAV, and elongation of the AA influence aortic dilatation. The ASI and AD/DD ratio are important markers of aortic dilatation. The performed diagnostics did not indicate a negative influence of GH treatment on the cardiovascular system.