Types Of Myocarditis Research Articles

Long-Term Prognosis of Patients With Myocarditis Attributed to COVID-19 mRNA Vaccination, SARS-CoV-2 Infection, or Conventional Etiologies

Although patients with myocarditis after COVID-19 mRNA vaccination appear to have a good prognosis near hospital discharge, their longer-term prognosis and management remain unknown. To study the cardiovascular complications of post-COVID-19 mRNA vaccination myocarditis and other types of myocarditis during an 18-month follow-up, as well as disease management based on a study of the frequency of medical procedures and drug prescriptions. In this cohort study based on the French National Health Data System, all individuals aged 12 to 49 years hospitalized for myocarditis in France between December 27, 2020, and June 30, 2022, were identified. Individuals were categorized as having postvaccine myocarditis (within 7 days after COVID-19 mRNA vaccine), post-COVID-19 myocarditis (within 30 days of SARS-CoV-2 infection), or conventional myocarditis. The occurrence of clinical outcomes (hospital readmission for myopericarditis, other cardiovascular events, all-cause death, and a composite outcome of these events) over the 18 months following hospital admission were analyzed using weighted Cox models to standardize the comparisons with the conventional myocarditis group. Also, medical management after hospital discharge was longitudinally assessed using generalized estimated equation models. In total, 4635 individuals were hospitalized for myocarditis: 558 with postvaccine myocarditis, 298 with post-COVID-19 myocarditis, and 3779 with conventional myocarditis. Patients with postvaccine myocarditis were younger than those with post-COVID-19 and conventional myocarditis (mean [SD] age of 25.9 [8.6], 31.0 [10.9], and 28.3 [9.4] years, respectively) and were more frequently men (84%, 67%, and 79%). Patients with postvaccine myocarditis had a lower standardized incidence of the composite clinical outcome than those with conventional myocarditis (32/558 vs 497/3779 events; weighted hazard ratio, 0.55 [95% CI, 0.36-0.86]), whereas individuals with post-COVID-19 myocarditis had similar results (36/298 events; weighted hazard ratio, 1.04 [95% CI, 0.70-1.52]). The standardized frequency of medical procedures and drugs prescribed in patients with postvaccine myocarditis or post-COVID-19 myocarditis followed a similar trend in the 18 months following hospital discharge to that of patients with conventional myocarditis. Patients with post-COVID-19 mRNA vaccination myocarditis, contrary to those with post-COVID-19 myocarditis, show a lower frequency of cardiovascular complications than those with conventional myocarditis at 18 months. However, affected patients, mainly healthy young men, may require medical management up to several months after hospital discharge.

Myocarditis in children.Etiology. Pathogenesis. Epidemiology. Clinical presentation

Readers will be presented with a series of articles on myocarditis in children, which are based on clinical guidelines developed by a group of authors on this topic. Myocarditis is an inflammatory myocardial disease characterized by a wide range of clinical manifestations — from minor to sudden cardiac death. Given the absence of pathognomonic symptoms, as well as the similarity of manifestations in the onset with common childhood diseases such as respiratory distress, gastrointestinal diseases, infectious pathology, specialists often overlook the likelihood of a diagnosis of “myocarditis”, which causes a late start of therapy and an adverse outcome. Considering that myocarditis accounts for 12% of cases of sudden cardiac death in adolescents and young adults, suspicion of this disease in differential diagnosis in children with nonspecific symptoms and disease progression may be the key to the formation of a proper treatment strategy. Despite significant advances in the study of myocarditis in both adults and children, there are still many unresolved issues. The purpose of this article is to highlight the current view of the etiology, pathogenesis, epidemiology and clinical features in children with various types of myocarditis in order to increase the knowledge of practitioners for better clinical management of patients with myocarditis.

Cardiac Magnetic Resonance Imaging Findings and Clinical Features of COVID-19 Vaccine-Associated Myocarditis, Compared With Those of Other Types of Myocarditis.

To compare the clinical and cardiac magnetic resonance (CMR) imaging findings of coronavirus disease 2019 (COVID-19) vaccine-associated myocarditis (VAM) with those of other types of myocarditis. From January 2020 to March 2022, a total of 39 patients diagnosed with myocarditis via CMR according to the Modified Lake Louise criteria were included in the present study. The patients were classified into two groups based on their vaccination status: COVID-19 VAM and other types of myocarditis not associated with COVID-19 vaccination. Clinical outcomes, including the development of clinically significant arrhythmias, sudden cardiac arrest, and death, and CMR imaging features were compared between COVID-19 VAM and other types of myocarditis. Of the 39 included patients (mean age, 39 years ± 16.4 [standard deviation]; 23 men), 23 (59%) had COVID-19 VAM and 16 (41%) had other types of myocarditis. The occurrence of clinical adverse events did not differ significantly between the two groups. As per the CMR imaging findings, the presence and dominant pattern of late gadolinium enhancement did not differ significantly between the two groups. The presence of high native T1 or T2 values was not significantly different between the two groups. Although the native T1 and T2 values tended to be lower in COVID-19 VAM than in other types of myocarditis, there were no statistically significant differences between the native T1 and T2 values in the two groups. The present study demonstrated that the CMR imaging findings and clinical outcomes of COVID-19 VAM did not differ significantly from those of other types of myocarditis during hospitalization.

COVID-19-related cardiac lesion: The questions of pathogenesis and diagnostics

Coronavirus infection is still a topic of interest in the medical community today. Among the heterogeneous clinical manifestations of this disease, lesions of cardiac structures often occur. They are mainly inflammatory in nature and can be acute or delayed. Aside from myocarditis, coronavirus infection can induce cardiac injuries, including acute coronary syndrome, thromboembolic events, heart failure, and heart rhythm disturbances. It is well known that the prognosis for patients with cardiac lesions significantly worsens; timely diagnosis and treatment initiation play an important role in preventing severe complications.
 This review presents the most recent literature data on the pathogenesis of cardiac lesions in COVID-19 patients and discusses the rational diagnosis of this pathology using modern techniques, such as laboratory, functional imaging (cardiac magnetic resonance is the most important of these), and invasive ones. It is now established that diagnosing myocarditis caused by coronavirus infection differs fundamentally from diagnosing other types of myocarditis. Furthermore, the main aspects of inflammatory heart lesions associated with COVID-19 vaccination are discussed, as this complication occurs more frequently than is commonly believed. It is often used as a rationale for refusing vaccination; however, this decision may severely affect the individual and the population.

In this Issue

HomeCirculation ResearchVol. 131, No. 8In this Issue Free AccessIn BriefPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessIn BriefPDF/EPUBIn this Issue Ruth Williams Ruth WilliamsRuth Williams Search for more papers by this author Originally published29 Sep 2022https://doi.org/10.1161/RES.0000000000000573Circulation Research. 2022;131:651is related toExtracellular Vesicles Regulate Sympatho-Excitation by Nrf2 in Heart FailureImpaired Dynamic Sarcoplasmic Reticulum Ca Buffering in Autosomal Dominant CPVT2is related toTranscriptional and Immune Landscape of Cardiac SarcoidosisCardiac Sarcoidosis Immune Landscape (p 654)Download figureDownload PowerPointLiu et al examine the transcriptional and cellular composition of cardiac sarcoidosis.Sarcoidosis is a rare inflammatory disease in which granulomas—small, localized sites of inflammation—afflict various parts of the body, most commonly the lungs, skin and lymph nodes. While sarcoidosis can be managed, there is no cure for the condition and, if it manifests in the heart, it can be deadly. Because little is known about the specific pathology of cardiac sarcoidosis (CS), and because it can resemble other forms of heart inflammation, Liu and colleagues set out to map the specific transcriptional and cellular landscape of CS lesions. Using a combination of spatial transcriptomics, single-nucleus RNA sequencing and immunofluorescence, the team compared tissue samples from CS patients with those from patients with other types of myocarditis. CS granulomas had a particular cellular arrangement, the team found, with HLA-DR+ macrophages immediately adjacent to and surrounding multinucleated giant cells. The giant cells expressed transmembrane glycoprotein NMB—a novel marker of these cells—while activation of mTOR genes was a feature of the surrounding macrophages. These analyses were carried out on a small number of patient samples but, if further studies validate the findings, they could form the basis of future diagnostic tests for CS, say the team.Impaired Dynamic SR Ca Buffering Causes AD-CPVT2 (p 673)Download figureDownload PowerPointWleklinski et al reveal the molecular effects of a dominant CPVT-causing mutation.Exercise or emotional stress can prompt the release of catecholamine hormones, which induce a fast heart rate, increased blood pressure and other features of the fight-or-flight response. For people with catecholaminergic polymorphic ventricular tachycardia (CPVT), however, physical activity or stress can cause potentially lethal arrythmias. Mutation of calsequestrin-2 (CASQ2)—a sarcoplasmic reticulum (SR) calcium binding protein—is a major cause of CPVT and can be recessive or dominant in nature. For many recessive mutations, disease occurs due to loss of CASQ2 protein. Wleklenski and colleagues have investigated a dominant lysine-to-arginine mutation (K180R) and find, by contrast, protein levels remain normal. In mice carrying the mutation, not only was the level of CASQ2 comparable to that in control animals, but so too was the protein’s subcellular localization. The mutation instead interfered with CASQ2’s calcium binding, or buffering, capability within the SR. The result was that, upon catecholamine injection or exercise, the unbound calcium released prematurely from the SR triggering spontaneous cell contractions. In uncovering this novel molecular etiology of CPVT, the work provides a basis for studying the consequences of other dominant CASQ2 mutations, say the team.EV-Mediated Heart Brain Communication in CHF (p 687)Download figureDownload PowerPointTian et al discover signals from the failing heart to the brain that may further exacerbate cardiac dysfunction.After a myocardial infarction, increased oxidative stress in the heart can contribute to adverse cardiac remodeling and ultimately heart failure. Nrf2 is a master activator of antioxidant genes, suggesting a protective role, but studies in rats have shown its expression to be suppressed after MI—likely due to upregulation of Nrf2-targeting microRNAs. These miRNAs can also be packaged into vesicles and released from stressed heart cells, and now Tian and colleagues show that rats and humans with chronic heart failure (CHF) have an abundance of these miRNA-containing EVs in their blood. In the rats with CHF, these EVs were found to be taken up by neurons of the rostral ventrolateral medulla (RVLM) wherein the miRNAs suppressed Nrf2 expression. The RVLM is a brain region that controls the sympathetic nervous system and, in the presence of the EVs, it ramped up sympathetic excitation. Because such elevated sympathetic activity can induce the fight-or-flight response—including increased heart rate and blood pressure—this would likely worsen heart failure progression. The team found, however, that inhibiting the miRNAs in the EVs prevented Nrf2 suppression in the RVLM and sympathetic activation, suggesting the pathway could be targeted therapeutically. Previous Back to top Next FiguresReferencesRelatedDetailsRelated articlesExtracellular Vesicles Regulate Sympatho-Excitation by Nrf2 in Heart FailureChanghai Tian, et al. Circulation Research. 2022;131:687-700Impaired Dynamic Sarcoplasmic Reticulum Ca Buffering in Autosomal Dominant CPVT2Matthew J. Wleklinski, et al. Circulation Research. 2022;131:673-686Transcriptional and Immune Landscape of Cardiac SarcoidosisJing Liu, et al. Circulation Research. 2022;131:654-669 September 30, 2022Vol 131, Issue 8 Advertisement Article InformationMetrics © 2022 American Heart Association, Inc.https://doi.org/10.1161/RES.0000000000000573 Originally publishedSeptember 29, 2022 PDF download Advertisement

Prevalence of myocarditis, genetic cardiomyopathies and their combinations among patients of the Cardiology Hospital of the V.N. Vinogradov Faculty Therapeutic Clinic of the Sechenov University

Aim. To establish the prevalence of myocarditis and primary (genetic) cardiomyopathies (CMP) among patients in a cardiology hospital.Material and methods. Medical records of 671 patients of the cardiology department were analyzed. The diagnosis at admission and at discharge was recorded. The diagnoses were divided into 7 following categories: hypertension, coronary artery disease, heart disease, idiopathic arrhythmias, cardiomyopathy, myocarditis and others. Types of myocarditis and cardiomyopathy, the presence of arrhythmias and heart failure were also recorded.Results. Myocarditis was diagnosed in 194 (28,9%) patients, cardiomyopathy — in 76 (11,3%) patients, combination of cardiomyopathy and myocarditis — in 26 (3,9%) patients. Myocarditis with the development of arrhythmia and heart dilatation prevailed as follows: 47,4 and 41,2%, respectively. The most numerous CMPs were left ventricular noncompaction (n=30), non-inflammatory dilated CMP (n=13), hypertrophic CMP (n=10) and arrhythmogenic CMP of the right ventricle (n=9). In the group with idiopathic arrhythmias, 64,3% of patients were diagnosed with myocarditis, and 19,4% — with cardiomyopathy.Conclusion. The prevalence of non-coronary myocardial diseases among patients in a cardiology hospital is high and amounts to 40,2%. The presence of arrhythmias, heart failure or dilated cardiomyopathy may be a manifestation of non-coronary myocardial diseases, and requires a comprehensive examination aimed, in particular, at ruling out or verifying the diagnosis of myocarditis.

Clinical Identification and Characteristic Analysis of Giant Cell Myocarditis in 12 Cases.

Aims: Giant cell myocarditis (GCM) is a rare, rapidly progressing cardiomyopathy with high mortality, if not diagnosed and treated in time. We analyzed the progression and clinical manifestations of patients with definitive diagnosis of GCM.Methods and Result: We enrolled 12 patients diagnosed with GCM in the explanted heart during heart transplantation (HTx) or by endomyocardial biopsy (EMB) and collected information on demographic data, cardiac structure and function, arrhythmias, preliminary diagnosis, and delay of the diagnosis. Seven cases were diagnosed from biopsy samples during HTx, and five cases were diagnosed through EMB. Before the diagnosis of GCM based on pathological analysis, these patients had been incorrectly diagnosed with arrhythmogenic right ventricular cardiomyopathy (n = 5), dilated cardiomyopathy (n = 2), ventricular tachycardia (n = 2), viral myocarditis (n = 1), cardiac amyloidosis (n = 1), and ischemic cardiomyopathy (n = 1) based on clues such as symptoms, arrhythmia, and cardiac imaging. Patients diagnosed with GCM through EMB had a shorter symptom-onset-to-diagnosis time (6.6 ± 2.7 months) and milder heart damage (left ventricular ejection fraction, 47.2 ± 8.8%) than those diagnosed during HTx (11.0 ± 3.3 months, P = 0.034; 31.4 ± 10.9%, P = 0.024).Conclusion: GCM is easily misdiagnosed as other types of myocarditis and cardiomyopathy. Pathological examination of the myocardium is the most reliable diagnostic method for GCM. Endocardial biopsy can identify patients with GCM at an earlier stage.

Cardiac Sequelae of COVID-19 in Children and Young Adults

Coronavirus disease 2019 (COVID-19) has a predilection to cardiac involvement. The early clinical phase, during viremia, may manifest as pericarditis, acute myocarditis, and sepsis-related cardiomyopathy. Delayed presentations, such as multisystem inflammatory syndrome in children, coronary artery dilation/aneurysms, and late myocarditis, may occur in the weeks after the acute infection. These delayed presentations commonly test negative for severe acute respiratory syndrome coronavirus 2 via polymerase chain reaction testing and are thought to be primarily postviral hyperinflammatory sequelae. The long-term consequences of cardiac involvement in COVID-19 are unknown. Most recommendations for cardiac management are based on known conditions that are similar. For example, coronary aneurysms can be managed under Kawasaki disease guidelines. Similarly, for patients with COVID-19 myocarditis, they can be cleared for sports under protocols for other types of myocarditis. There is concern for cardiac involvement as a subclinical entity even in more minor presentations. Several expert algorithms have been developed for clearing competition athletes to return to exercise. Sports clearance should be individualized considering the severity of disease, age of patient, and performance level of the sport. [Pediatr Ann. 2021;50(3):e128-e135.].

Virome Sequencing in Patients With Myocarditis.

Polymerase chain reaction analyses of cardiac tissues have detected viral sequences in up to 67% of cases of myocarditis. However, viruses have not been implicated in giant cell myocarditis (GCM). Furthermore, efforts to detect viruses implicated in myocarditis have been unsuccessful in more accessible samples such as peripheral blood. We used Virome Capture Sequencing for Vertbrate Viruses (VirCapSeq-VERT), a method that simultaneously screens for all known vertebrate viruses, to investigate viruses in 33 patients with myocarditis. We investigated peripheral blood mononuclear cells (n=24), plasma (n=27), endomyocardial biopsies (n=2), and cardiac tissue samples from explanted hearts (n=13). Nine patients (27%) had GCM and 4 patients (13%) had fulminant myocarditis. We found the following viruses in the blood of patients with myocarditis: Epstein Barr virus (n=11, 41%), human pegivirus (n=1, 4%), human endogenous retrovirus K (n=27, 100%), and anellovirus (n=15, 56%). All tissue samples from fulminant myocarditis (n=2) and GCM (n=13) contained human endogenous retrovirus K. No nucleic acids from viruses previously implicated in myocarditis or other human illnesses were detected in relevant amounts in cardiac tissue samples from GCM or in blood samples from other types of myocarditis. These findings do not exclude a role for viral infection in GCM but do suggest that if viruses are implicated, the mechanism is likely to be indirect rather than due to cytotoxic infection of myocardium.

Treatment efficacy of arrhythmias and dilated cardiomyopathy syndrome of immune-inflammatory nature using plasmapheresis

Aim. To study the efficacy of plasmapheresis as the main type of pathogenic treatment or in combination with immunosuppressive therapy in patients with dilated cardiomyopathy (DCMP) and arrhythmias of immune-inflammatory nature. Material and methods. The main group included 20 patients with arrhythmic myocarditis (with premature supraventricular / ventricular contraction >3000/day, n=3/8, atrial fibrillation (AF) n=9) and 14 patients with DCMP syndrome (enddiastolic volume (EDV) left ventricle (LV) 6,3±0,6 cm, ejection fraction (EF) 33,5±8,1%). The inclusion criterion was an increase of at least 2 types of anti-cardiac antibodies titers ≥ twice. Myocarditis is diagnosed using myocardial biopsy, magnetic resonance imaging, multispiral computed tomography, scintigraphy, coronary angiography. We used a course of discrete plasmapheresis. The comparison group included 26 patients with an arrhythmic myocarditis and 19 with DCMP syndrome (EDV 6,6±0,8 cm, EF 32,6±7,3%), which plasmapheresis was not used. Dynamics was assessed at 6 and 12 months. Results. In groups of patients with arrhythmias and DCMP, a significant decrease in anti-cardiac antibodies titers was observed immediately after plasmapheresis and in control studies (p<0,05). In patients with arrhythmias, a health-promoting effect (a decrease in the number of premature contraction and a frequency of atrial fibrillation ≥75%) was observed in 65% of the main group and 58% of the comparison group. Predictor of plasmapheresis efficiency was a titer of specific antinuclear factor ≥1: 40 (sensitivity — 92,3%, specificity — 71,4%, AUC — 0,813, p<0,05). Methylprednisolone was prescribed to 45% of patients in the main group and 73% to patients in the comparison group (p>0,05) at a dose of 8 [4; 16] and 16 [10; 24] mg per day, respectively, p>0,05. In patients with DCMP in the main group, a significant increase in EF (p<0,05) (up to 41,4±8,2% and 46,3±12,7% vs 39,1±13,7% and 37,2±10,7% in the comparison group) and the distance of 6-minute walking test was obtained. A good effect (increase in EF by 10% or more) was noted in 50% of the main group and 32% of the comparison group. The predictor of plasmapheresis efficacy was systolic pressure in the pulmonary artery ≥28,5 mm Hg. (sensitivity — 100%, specificity — 71,4%, AUC — 0,893, p<0,05). In the main group, methylprednisolone was assigned to 43% of patients, in the comparison group — 89%, p<0,05. The average doses of methylprednisolone in the main group were significantly lower than in the comparison group (8 [8; 17,25] vs 16 [13; 28] mg per day, p<0,05). Conclusion. Positive clinical response to plasmapheresis was noted in 65% of patients with arrhythmias and in 50% of patients with DCMP of immune-inflammatory nature. In patients with different types of myocarditis, plasmapheresis increases the efficacy of antiarrhythmic and immunosuppressive therapy.

Should we fear infarct-like myocarditis?

IntroductionInfarct-like myocarditis is the common presentation of acute myocarditis with a good prognosis, mentioned in the literature. The restriction of physical activity for up to 6 months is usually recommended. The aim of our study was to show the characteristic features and specificity of this particular presentation of myocarditis and to evaluate its prognosis, relative to physical activity, after hospital discharge. MethodsFrom 2012 to 2016, 93 patients were hospitalized with acute myocarditis at the University Hospital of Pilsen, 73 (78%) of them had infarct-like myocarditis and were evaluated by basic diagnostic parameters.Thirty-two patients with infarct-like myocarditis were involved in our prospective observational study where echocardiography (ECHO), magnetic resonance imaging (MRI) including late gadolinium enhancement (LGE) volume, bicycle ergometry (BE) as well as troponin screening tests were regularly performed. None of the patients were professional athletes.One month after hospital discharge, all patients underwent bicycle ergometry without any load limitation. After the 1-month follow-up, patients were allowed to increase physical exertion gradually to included job performance. ResultsInitially, mild left ventricular systolic dysfunction was found in 43.8%, that was normalized before the first outpatient follow-up. Other patients had normal systolic function. Initially, all of the patients had significantly elevated troponin level. After 1 and 6 months, hypersensitive troponin values were under the 99th percentile of the healthy population. All of the patients had positive LGE in subepicardial localization. After 1 month, the LGE volume median had decreased by up to 53% and after 6 months it had decreased by up to 40% of the original value. The achieved exertion during BE, at the 6-month follow-up (9.7±2.2 METs) was statistically significantly better than after the 1-month follow-up (8.9±2.1 METs, p=0.0023). On the last BE follow-ups (9.3±2.1 METs), no significant change was seen (p=0.2331). The average follow-up time was 2 years. ConclusionInfarct-like myocarditis was the most common presentation of acute myocarditis during the observation time. No patient had an early recurrence of the disease (i.e., within 1 year) and no left ventricular (LV) systolic dysfunction was developed. Infarct-like myocarditis has a specific feature that differs from other types of myocarditis. It has good prognosis, which is independent of physical exertion starting 1 month after hospital discharge.

Multislice spiral computed tomography of the heart in dilated cardiomyopathy: possibilities in the verification of myocarditis (in comparison with myocardial biopsy) and in the evaluation of prognosis

To investigate whether intravenous contrast-enhanced multislice spiral computed tomography (computed tomography) (MSCT) versus myocardial morphological examination can diagnose myocarditis and the non-inflammatory causes of dilated cardiomyopathy (DCM) and evaluate prognosis in patients with the latter. A study group consisted of 130 patients, including 95 men (46.8±11.9 years), with DCM (mean left ventricular (LV) end-diastolic dimension (EDD), 6.6±0.8 cm; mean LV ejection fraction (EF), 29.8±9.3%; NYHA functional class (FC) III (II; III)). All the patients underwent intravenous contrast-enhanced 320-slice CT of the heart; myocardial morphological examination was made in 48 patients (endomyocardial biopsy in 29 patients, intraoperative biopsy in 7, and autopsy in 9, and study of the explanted heart in 3). In addition, cardiotropic viral DNA in the blood and myocardium and the level of anticardiolipin antibodies were determined; echocardiography (in all the patients), scintigraphy (n = 45), magnetic resonance imaging (MRI) (n = 21), and coronary angiography (CG) (n = 46), and a genetic consultation were performed. A comparison group comprised 20 patients, including 14 men (69.3±9.2 years), with coronary atherosclerosis (40% or more stenoses) according to MSCT findings in the absence of criteria for DCM (mean LV EDD, 4.8±0.5 cm; mean LV EF, 59.4±4.6%). Morphological/comprehensive examination showed that myocarditis as a cause of DCM was diagnosed in 76 (65%) patients; its concurrence with genetic cardiomyopathies was in 17 more patients (17%). MSCT of the heart revealed lower accumulation areas in 2 (1.5%) patients (type 1 based on the proposed rating scale), delayed myocardial contrast agent accumulation (DMCAA) in 81 (62.3%): subendocardial accumulation (type 2) in 8, intramyocardial accumulation in 4 (type 3), subepicardial accumulation in 52 (type 4), and transmural accumulation in 15 (type 5); DMCAA was not noted in 49 patients. DMCAA was not found in the comparison group. As compared with biopsy, the sensitivity, specificity, predictive value of positive and negative results of the tests in detecting active myocarditis for all the types of DMCAA were 77.4, 47.1, 72.7, and 53.3%, respectively; those for types 3-5 of DMCAA were 77.4, 52.9, 75.0, and 56.3%; those in detecting all the morphological types of myocarditis were 68.3, 28.6, 84.8, and 13.3%, and those for types 3-5 were 65.9, 28.6, 84.4, and 12.5%, respectively. Comparison of the data of MSCT and those of comprehensive examination in all the patients with DCM, the diagnostic significance in detecting myocarditis for all the types of DMCAA was 70.6, 67.9, 88.9 and 38.8%, respectively; that for DMCAA types 3-5 was 60.8, 67.9, 87.3, and 32.3%. In the study group, MSCT also identified the non-compacted myocardium (n = 31 (23.8%)), coronary atherosclerosis (n = 31 (23%)), which is confirmed by CG findings in 15 patients. The patients with DMCAA significantly more frequently showed a relationship with previous infection, acute onset, significantly higher NYHA FCs, end-diastolic and end-systolic LV volumes, and insignificantly lower LV EF. During a mean follow-up periods of 12 (6; 37.25) months, the overall mortality rate was 17.7% (23 deaths); the death + transplantation index was 20% (n = 26). All the types of DMCAA were found to be significantly related to prognosis: in the DMCAA group, the mortality rate was 21.5% versus 7.8% in the non-DMCAA group (odds ratio 3.22; 95% confidence interval, 1.02 to 10.21; p < 0.05). MSCT with the assessment of delayed contrast enhancement (and simultaneous CT coronary angiography) can be used for the non-invasive diagnosis of myocarditis in patients with DCM, including that in the presence of contraindications to MRI. DMCAA correlates with the presence of myocarditis, its activity, the degree of functional disorders, and prognosis.

Heart Transplantation in Giant Cell Myocarditis: Analysis of the United Network for Organ Sharing Registry

BackgroundGiant cell myocarditis (GCM) is a lethal, rapidly progressive disease, for which heart transplantation is the treatment of choice. We sought to describe the characteristics and outcomes of patients with GCM who undergo heart transplantation. Methods and ResultsWe used the United Network for Organ Sharing thoracic organ transplantation registry to identify adults with GCM as the primary diagnosis and compared their characteristics and outcomes with patients who underwent transplantation for other types of myocarditis and for idiopathic dilated cardiomyopathy (IDCMP). A total of 32 patients with GCM were compared with 219 patients with myocarditis and 14,221 patients with IDCMP. Median age at listing for GCM was 52 years (interquartile range 40–55 y), and the majority were white (94%), male (63%), and listed as 1A (44%). Biventricular assist devices were used more frequently in GCM compared with IDCMP (31% vs 2%; P < .001). After transplantation, there were no statistically significant differences among GCM, myocarditis, and IDCMP patients regarding pacemaker implantation, dialysis initiation, or stroke rate. GCM patients had increased risk of acute rejection compared with IDCMP patients (16% vs 5.0%; P = .021) but no difference in rehospitalization for rejection among the 3 etiologies (P = .88). The cumulative survivals for GCM patients at 1, 5, and 10 years were 94%, 82%, and 68%, respectively, which was similar to the other etiologies (P = .11). ConclusionsCompared with patients with IDCMP, those with GCM present more acutely and have significantly higher utilization of biventricular mechanical circulatory support. Despite higher rates of early rejection, post-transplantation survival of patients with GCM was similar to that of other myocarditides and IDCMP.

Interventional treatment in patients with myocarditis: Pro and Contra

Ventricular tachyarrhythmias (VTs) are often encountered in patients with inflammatory heart diseases. VT can become lifethreatening in patients with myocarditis, and the management may vary in different types of myocarditis. Purpose of the study is to describe VT characteristics in patients with verified myocarditis, and to evaluate the efficacy and safety of VT management, when tailored to the type of myocarditis. Study population comprised: 56 patients with morphologically verified myocarditis; 18 patients with primary cardiomyopathy (control group). All patients underwent full clinical evaluation, endomyocardial biopsy (including immunohistochemical analysis). Forty (54 %) patients underwent radiofrequency catheter ablation of VT. An implantable cardioverter-defibrillator (ICD) was inserted in 17 patients. There was no statistically significant difference between myocarditis and primary cardiomyopathy groups by demographic and echocardiographic data. In myocarditis group, nonsustained VT and/or frequent premature ventricular beats were seen in 59 % of patients; sustained VT in 12,5 % subjects, 1 patient had a history of ventricular fibrillation. VT ablation was associated with a significant decrease in VT recurrence (p=0,0009) during the follow-up period. Active myocarditis was associated with a higher VT recurrence rate (67 % in active vs. 19 % in borderline myocarditis). Among patients with ICD implantation, only one subject (with active myocarditis at admission) had life-threatening ventricular arrhythmia. In this selected group of patients with verified myocarditis and clinically significant VTs, catheter ablation seems at least partly effective. Patients with borderline myocarditis and symptomatic VTs may benefit from ablation. Therefore, morphological diagnostic of myocarditis can be a key point in choice of treatment.

Eosinophilic Myocarditis due to Toxocariasis: Not a Rare Cause.

Myocarditis is a clinically important disease because of the high mortality. From the perspective of treatment strategy, eosinophilic myocarditis should be distinguished from other types of myocarditis. Toxocariasis, caused by Toxocara canis or Toxocara cati, is known as a cause of eosinophilic myocarditis but is considered rare. As it is an unpopular disease, eosinophilic myocarditis due to toxocariasis may be underdiagnosed. We experienced two cases of eosinophilic myocarditis due to toxocariasis from different geographical areas in quick succession between 2013 and 2014. Case 1 is 32-year-old man. Case 2 is 66-year-old woman. In both cases, diagnosis was done by endomyocardial biopsy and IgG-ELISA against Toxocara excretory-secretory antigen. Only a corticosteroid was used in Case 1, whereas a corticosteroid and albendazole were used in Case 2 as induction therapy. Both patients recovered. Albendazole was also used in Case 1 to prevent recurrence after induction therapy. Eosinophilic myocarditis by toxocariasis may in actuality not be a rare disease, and corticosteroid is an effective drug as induction therapy even before use of albendazole.

Abstract 17506: A Young Man with a Great Fall: Putting the Pieces Together

A 35 year-old-male with hypertension and schizoaffective disorder presented with a first episode of non-prodromal syncope and traumatic fall resulting in a mandibular fracture. Physical examination revealed orthostatic tachycardia, normal cardiovascular examination, and no abnormalities with carotid sinus massage. Electrocardiogram showed NSR at 66 bpm, a single PVC, QTc interval 493 ms, and abnormal ST-T wave contour in V2-V3. Laboratory evaluation revealed normal electrolytes, mildly elevated troponin (0.763), and no leukocytosis. Postural orthostatic tachycardia syndrome was considered unlikely given that syncope did not occur after prolonged standing, and that his use of trazodone and risperidone can both cause orthostatic tachycardia. Transthoracic echocardiography (TTE) revealed decreased systolic function (EF 45%), paradoxical septal motion, normal LV wall thickness, and no valvular disease. Cardiac MRI showed moderate biventricular systolic dysfunction, LVEF 43%, RVEF 33%, mild 4-chamber dilatation, and a small pericardial effusion. Mild T2 hyperintensity in the mid-ventricular septum and anteroseptum, and scattered foci of patchy delayed gadolinium enhancement within the mid-anteroseptal, anterior and anterolateral LV segments were consistent with acute myocarditis. The reported prevalence of myocarditis among patients with sudden death is highly variable and ranges from 2-42%. Various types of myocarditis are associated with arrhythmias, including lymphocytic myocarditis which was the likely diagnosis in our patient. Despite lack of guideline recommendations, we opted to protect our patient with a wearable cardioverter-defibrillator given case reports of successful use for recurrent malignant arrhythmias in myocarditis. Follow-up TTE two months later was normal with EF 60%. Although syncope in the younger population is often appropriately attributed to a neurally-mediated etiology, the current case highlights the need for clinicians to be attuned to the possibility of subclinical myocarditis causing malignant arrhythmias in this setting, in which case the patient may be at risk for recurrent arrhythmia and sudden death and may benefit from the use of a wearable cardioverter-defibrillator.

Endomyocardial biopsy: Yesterday, today and tomorrow

The major goals of this presentation are to give the updated knowledge of primary heart muscle disease, cardiomyopathies (CMP), their classification, the diagnostic possibilities using endomyocardial biopsy (EMB) and to estimate the value of EMB in the choice of the right therapeutic approach. Despite many controversies, EMB is today a widely accepted method, with low percentage of complications for analysing CMPs, and is considered to be a routine procedure in many cardiological centers. Inspite the fact that we started with the use of EMB in 80's, it is not performed often today in Belgrade, like many years ago (2). It's difficult to say why ? The CMPs may be subdivided, according to the functional and structural features, into two groups: idiopathic and specific, or primary and secundary. Idipathic group is consisted of 5 subgroups: hypertrophic, dilated, restrictive, arrithmogenic right ventricular CMP and unclassified. Specific CMPs may be subdivided into 8 groups mainly based on etiopathogenetic characteristics: infective, with viral myocarditis as the most common entity, metabolic, including endocrine disorders and infiltration and storage diseases. Deficiency disorders and heart involvement in connective tissue disorders are also included. Granulomas, neoplasms and neuromuscular disorders are also very often presented with cardiac dysfunction and structural abnormalities. Sensitivity and toxic reactions with long list of substances which may affect the heart are probably the most present today. Finally, the last group represent miscellaneous systemic syndromes with heart affection. EMB has been increasingly used in the diagnosis of CMPs, with special influence on diagnosis of heart transplant rejection, myocarditis, treatment modalities of different types of myocarditis, with low complication rate, considered effective, useful and safe procedure.

Do clinical diagnoses correlate with pathological diagnoses in cardiac transplant patients? The importance of endomyocardial biopsy

Heart transplantation remains the last treatment option for patients with end-stage cardiac disease. Such diseases include ischemic cardiomyopathy, nonischemic cardiomyopathy and other conditions such as arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and cardiac amyloidosis. To review the changes that have occurred over time in the etiology of heart disease in patients requiring heart transplantation, and to compare the clinical and histological diagnoses of explanted hearts from patients with progressive cardiac disease. The pathological findings of 296 surgically excised hearts over a 20-year period (January 1987 to July 2006) at one institution were examined. Patients were separated into groups based on year of heart transplantation. The tissue was examined to determine the underlying cardiac pathology leading to congestive heart failure. Patient records were reviewed for preoperative clinical diagnoses and other relevant data, including pretransplant endomyocardial biopsy (EMB) results, information regarding left ventricular assist devices and, finally, evidence of disease recurrence in the grafted heart. A shift in the underlying etiology was found in patients who underwent heart transplantation from 1992 to 1996, and 1997 to 2001. Between 1987 and 1997, the majority of transplant cases consisted of ischemic cardiomyopathies. From 1997 to 2001, the majority of patients had nonischemic cardiomyopathies, and this trend continued to 2006. A majority of patients with ischemic and hypertrophic cardiomyopathy were diagnosed correctly (96.5% and 82%, respectively) before transplantation. Most patients diagnosed post-transplant with lymphocytic (viral, 15%), hypersensitive/ eosinophilic (25%) and giant cell (100%) myocarditis, arrhythmogenic right ventricle dysplasia (100%), cardiac sarcoidosis (83%) and iron overload toxicity- associated cardiomyopathy (100%) had been misdiagnosed in pretransplantation investigations. Investigations before transplantation did not include an EMB. Of all 296 patients, 51 patients (17%) were misdiagnosed. Excluding the patients with ischemic cardiomyopathy, 46 of 152 patients (30%) were misdiagnosed before transplantation. Although cardiac transplantation is a viable treatment option for patients with a variety of cardiac diseases, accurate diagnosis of patients before transplantation remains a priority. Accurate diagnosis of particular diseases (sarcoidosis, myocarditis, iron toxicity-associated cardiomyopathy and others) allows for proper treatment before transplantation, which may slow down disease progression and improve patient outcomes. Furthermore, it is important to accurately diagnose patients with diseases such as sarcoidosis, amyloidosis and particular types of myocarditis because these can readily recur in the grafted heart. The risk for recurrence must be known to practitioners and, most importantly, to the patient. We strongly recommend the use of EMB if a nonischemic cardiomyopathy is suspected, because the results may alter the diagnosis and modify the treatment strategy.

Pathological changes of the heart in sudden infant death.

There are more than 120 different theories on the possible causes of sudden infant death (SID). In particular, dysfunctions of the central nervous system, cardiorespiratory insufficiency due to infections including atypical immune reactions, and cardiac dysregulation have been discussed during the previous decade. Reports on disturbances of the cardiac rhythmogenic function due to LQTS were among the most speculative. Based on gross histological, immunohistochemical and molecular genetic investigations of SID cases, the most important and most frequent findings of the heart are shown. The significance of different types of myocarditis, hypoxia-related changes, disturbances of the rhythmogenic function, cardiomyopathy, and other changes is discussed with regard to the cause of death. In conclusion, most of the changes reported in the literature are not sufficient to explain the cause of death. Problems in the diagnosis are shown which influence the classification of these disturbances as well as the classification of SID.

Microvascular Involvement in Cardiac Pathology

Abnormalities of the microvasculature are centrally involved in the pathogenesis of some forms of heart disease, but in others are consequences of it. Microvascular abnormalities may contribute to the progression of viral myocarditis and Chagas' disease. Focal abnormalities may occur early in some cardiomyopathies and do occur later in most types of myocarditis. The thickening of arteriolar walls in chronic hypertension is likely to contribute significantly to the impairment of coronary haemodynamics associated with adaptive ventricular hypertrophy and the consequent diminution of coronary reserve, increasing diffusion distances and failure of angiogenesis to compensate. However, the resulting myocyte necrosis stimulates inflammatory angiogenesis. When ischemic myocyte injury becomes irreversible there is a concomitant loss of capacity for reperfusion, the no-reflow phenomenon. Less severe temporary ischemia reduces the proportion of functional capillaries. Multiple mechanisms are involved in this microvascular stunning, including: reperfusion injury; leukocyte activation, adhesion and accumulation; and impaired endothelium-dependent vasodilation. Many of the microvascular changes are those of the inflammatory response to cell death and form part of a final common pathway in myocarditis, cardiomyopathy, cardiac hypertrophy and failure, and ischemic heart disease. Stimulation of angiogenesis prior to myocyte necrosis in hypertrophy and control of leukocyte activity in ischemic heart disease could minimize myocyte loss.