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Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.

Among many of the autoimmune diseases observed in patients with myasthenia gravis (MG), myocarditis is one of the most critical. The goal of this review is to systematically describe and investigate the characteristics of MG complicated with myocarditis. We identified 183 records in PubMed (MEDLINE), Web of Science, and EMBASE from 1948 to September 10, 2020. Studies were included if they presented clinical data on MG complicated with myocarditis. Of the 35 patients from 28 studies in this review, 57.14% (20/35) were males, with a mean age of 59.11 ± 15.87. Dyspnea was the most common cardiac symptom accounting for over 60% in the study. Among the 35 patients, 13 cases of myocarditis occurred concomitantly with MG and the longest interval between MG and myocarditis was 7 years. Forty percent of patients developed myocarditis caused by immune checkpoint inhibitors (ICI). Among the patients with myocarditis, over half of the patients were diagnosed by myocardial biopsy. After active immune regulation and symptomatic treatment, only 15 of 35 patients with MG complicated with myocarditis improved, 18 patients died during hospitalization, one patient died due to tumor progression and 1patient died 5 years later. The prognosis of patients with MG complicated with myocarditis is poor, and myocardial enzymes and other indexes need to be monitored for patients taking ICI drugs. Patients with dyspnea who are still not ideally treated by mechanical ventilation should be vigilant against the occurrence of MG complicated with myocarditis.

Restrictive cardiomyopathy (RCM) is a rare primary myocardial disease, and its pathological features are yet to be determined. Restrictive cardiomyopathy with MHY7 mutation was diagnosed in a 65-year-old Japanese woman. Electron microscopy of a myocardial biopsy revealed electron-dense materials resulting from focal myocyte degeneration and necrosis as well as tubular structures and pseudo-inclusion bodies in some nuclei. These features may be associated with the pathogenesis of RCM.

Background: We aim to evaluate the value of Cardiac magnetic resonance (CMR) feature tracking (CMR-FT) in addition to Task Force Criteria(TFC) in patients with (arrhythmogenic cardiomyopathy) AC biopsy-proved. Methods: Thirty-five patients with AC histologically proven who performed CMR with late gadolinium enhancement (LGE) acquisition were enrolled. The study population was divided in Group1 (negative CMR TFC and LV ejection fraction≥55%) and Group2 (positive CMR TFC and/or LVEF

Multisystem Inflammatory Syndrome (MIS) is a novel hyperinflammatory syndrome associated with SARS-CoV-2 infection. It predominantly affects children (MIS-C) a few weeks after a usually asymptomatic SARS-CoV-2 infection and is only rarely seen in adults above 21 years (MIS-A). Only scarce data on histological findings in both pediatric and adult patients has been published so far. An 18-year-old male patient was admitted to hospital in a febrile state, which progressed to severe cardiogenic shock and multi-organ failure requiring extracorporeal life support. Myocardial biopsy revealed small vessel-associated immune cell infiltrates. Diagnosis of MIS-C was made after ruling out all potential differential diagnosis. Use of immunosuppressive treatment with steroids, interleukin-1 blockade and high-dose intravenous immunoglobulins resulted in the patient's full recovery. Multisystem Inflammatory Syndrome (MIS) is a new differential diagnosis of cardiac dysfunction in pediatric and adult patients. The lack of myocardial necrosis differentiates the disease from other viral myocarditis and offers an explanation for the fast response to immunomodulatory therapy and the favorable prognosis. The preceding SARS-CoV-2 infection might only have been mildly symptomatic or even asymptomatic., publishedVersion

The aim of this research is to integrate spectral tissue sensing technology inside a cardiac bioptome for real-time measurements of tissue characteristics. Bioptome tip and handle components were designed and manufactured to house and guide optical fibers. The designed components were assembled on a cardiac bioptome together with optical fibers. A technical feasibility test was carried out to study the functionality of the instrument and the effect of the optical technology on the biopsy performance. Biopsy samples were taken from five different tissue types in a porcine heart and the resulting optical spectra were compared. Spectral tissue sensing fibers were successfully integrated inside a conventional cardiac bioptome. The integrated instrument allowed differentiation between ventricular tissue, blood, and cardiac fat tissue based on blood and fat percentage and amount of scattering. Moreover, differences between scarred and nonscarred tissue were clearly visible. A first step has been made in the use of spectral tissue sensing for the detection of different tissue structures for endo-myocardial biopsy (EMB). The instrument was able to differentiate between various tissues, as well as between healthy and diseased cardiac tissues. Future research should focus on measurements of naturally diseased cardiac tissue, repeated measurements with statistical value, and improvements to the instrument design. Having the ability to measure tissue characteristics prior to acquiring a biopsy sample will not only allow easier positioning of the bioptome at the correct location but can also prevent sampling undesired tissue or scar tissue from previous biopsies.

BackgroundCS constitutes a rare but potentially underdiagnosed and fatal disease. Its diagnosis remains difficult owing to the infrequent and indistinguishable symptoms and the lack of formal diagnostic criteria dependent upon the diagnostic techniques used. Early diagnosis and treatment, however, may help to counter its poor prognosis.We aim to characterize and compare the diagnostic accuracy of cardiac MRI, FDG-PET and myocardial biopsy for the diagnosis of cardiac sarcoidosis and to advance and compare methods for complex diagnostic test accuracy reviews and meta-analysis.MethodsFollowing a systematic review on DTA studies on the aforementioned topic, a four-part approach to meta-analysis will be used: (1) direct comparison of index tests with clinical reference standard, (2) indirect comparison of index tests with clinical reference standard, (3) addition of an alternative test to that indirect comparison (4) and Bayesian meta-analysis using results of part 3 as informative prior for comparisons analogous to part 1 and 2.DiscussionThe most widely recognized diagnostic algorithm for cardiac sarcoidosis is considered out of date, as it precedes the introduction of imaging techniques in diagnostic pathways. These novel imaging techniques, like CMR and FDG-PET scan, have emerged as promising diagnostic tools which may fill the current diagnostic gap. Thus, a systematic review and evaluation of CS diagnosis are much needed. Such an attempt is anticipated to alter the current diagnostic guidelines for CS by shedding more light on the role of sophisticated imaging techniques on prompt CS therapy and follow-up.Trial registrationPROSPERO,CRD42019047126

The idea that performing a proper succession of imaging tests and techniques allows an accurate and early diagnosis of cardiac amyloidosis, avoiding the need to perform the myocardial biopsy, is becoming increasingly popular. Furthermore, being imaging techniques non-invasive, it is possible to perform the follow-up of the pathology through repeated image acquisitions. : In the present review, the various innovative imaging methodologies are presented, and it is discussed how they have been applied for early diagnosis of cardiac amyloidosis (CA), also to distinguish the two most frequent subtypes in CA: immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR); this allows to perform the therapy in a targeted and rapid manner.

Coronary artery fistulae (CAF) represent an abnormal communication bypassing the myocardial capillary bed between a coronary artery and either a cardiac chamber (coronary-cameral CAF) or a vascular structure from systemic or pulmonary circulation (coronary-vascular CAF). CAF represent a congenital anomaly in 0.2% of the population, but are more frequently found in heart transplant recipients (8%), mostly as coronary-right ventricle (RV) CAF caused by endomyocardial biopsy used for monitoring of rejection1,2. Other complications of myocardial biopsy include perforation of the RV wall and tricuspid valve damage2. Several case reports have shown successful transcatheter closure of CAF3,4. We aimed to analyse ...

Aims: Giant-cell myocarditis (GCM) is a rare and often fatal form of myocarditis. Only a few reports have focused on fulminant forms. We describe the clinical characteristics, management and outcomes of GCM patients rescued by mechanical circulatory support (MCS). Methods and results: The clinical features, diagnoses, treatments and outcomes of MCS-treated patients in refractory cardiogenic shock secondary to fulminant GCM admitted to eight French intensive care units (2002-2016) were analysed. We also conducted a systematic review of this topic. Thirteen patients (median age 44 [range 21-76] years, Simplified Acute Physiology Score II55 [40-79]) in severe cardiogenic shock (median [range] left ventricular ejection fraction 15% [15-35%] and blood lactate 4 mmol/L) were placed on MCS 4 [0-28] days after hospital admission. Severe arrhythmic disturbances were frequent (77%), with six (46%) patients experiencing an electrical storm prior to MCS. Venoarterial extracorporeal membrane oxygenation was the first MCS option for 11 (85%) patients. GCM was diagnosed in five (38%) patients before transplant or death and treated with immunosuppressants; infections were the main complication (80%). Four patients died on MCS and no patient presented long-term survival free from heart transplant (nine patients, 69%). All transplanted patients were alive 1 year later and no GCM recurrence was reported after median follow-up of 42 [12-145] months. Conclusion: Outcomes of fulminant GCMs may differ from those of milder forms. In this context, heart transplant might likely be the only long-term survival option. (c) 2017 Elsevier B.V. All rights reserved.

Cardiac transthyretin-related (ATTR) amyloidosis is a severe cardiomyopathy for which therapeutic approaches are currently under development. Because non-invasive imaging techniques such as cardiac magnetic resonance imaging and echocardiography are non-specific, the diagnosis of ATTR amyloidosis is still based on myocardial biopsy. Thus, diagnosis of ATTR amyloidosis is difficult in patients refusing myocardial biopsy. Furthermore, myocardial biopsy does not allow 3D-mapping and quantification of myocardial ATTR amyloid. In this report we describe a Tc-99m-DPD-based molecular imaging technique for non-invasive single-step diagnosis, three-dimensional mapping and semiquantification of cardiac ATTR amyloidosis in a patient with suspected amyloid heart disease who initially rejected myocardial biopsy. This report underlines the clinical value of SPECT-based nuclear medicine imaging to enable non-invasive diagnosis of cardiac ATTR amyloidosis, particularly in patients rejecting biopsy.

Cardiac amyloidosis is a complex and vexing clinical condition that requires a high degree of suspicion for the diagnosis with a substantial amount of discipline to discern the extent of disease and the best available therapy. There is a complex interplay between multiple organ systems, and the clinical presentation may involve a myriad of confusing clinical symptoms. The diagnosis of cardiac amyloidosis can be confirmed with a combination of physical findings, cardiac biomarkers, noninvasive testing, and, if necessary, myocardial biopsy. Genetic testing is critical to establish the type of amyloidosis.

A 63-year-old Japanese woman with an adrenal tumor was transferred to our hospital due to cardiogenic shock. Right and left ventriculography showed severe hypokinesis of the middle segment and the apex in both ventricles, and an endomyocardial biopsy demonstrated a small number of necrotic myocytes and cellular infiltration. She was diagnosed with pheochromocytoma and quickly recovered after treatment with an α-blocker. The functional disability of both the right and left ventricles with less myocardial damage due to an excessive level of catecholamine seemed to be related to the early recovery the present patient with catecholamine-induced cardiomyopathy due to pheochromocytoma., Internal Medicine, 56(6), pp.665-671; 2017

Background Arrhythmogenic cardiomyopathy (ACM) is a genetically inherited cardiomyopathy characterized by myocardial fibro-fatty replacement. A pathogenetic role of viral myocardial infections in ACM natural history has been proposed over the years, although no definitive conclusion has been reached yet. Purpose To describe viral genome presence into a cohort of ACM biopsy proven patients (pts) and its impact on clinical features and outcome. Methods A cohort of all ACM pts undergoing an invasive third level evaluation at our institution was enrolled. All pts underwent a cardiac magnetic resonance (MR), an invasive electrophysiological study (EPS) with endo-cavitary electro-anatomical mapping (EAM), and a EAM guided endo-myocardial biopsy (EMB). Viral genome research through molecular biology techniques was performed on all biopsied samples. According to arrhythmic risk evaluation, a trans-catheter ablation (TCA) and/or an internal cardioverter device (ICD) implant was performed. Clinical arrhythmic presentation, MR data, arrhythmia inducibility at EPS, EAM and EMB characteristic, and arrhythmic events at a 12-month follow up visit were retrieved in all pts and compared between the viral genome positive (v+ACM) and negative group (v-ACM). Results Forty-five pts were enrolled in our study (48±13 years; 66% male); the EMB samples of 7 (15%) pts presented a lymphocytic infiltrate and tested positive for viral genome (n=3 B19 Parvovirus; n=2 for Citomegalovirus; n=2 for Ebstein-Bar Virus) [Figure1]. At arrhythmic presentation, complex ventricular arrhythmias (NSVT, SVT and FV) were more frequent in the v+ACM group (86% vs 50%; p=0.039). Both left and right ventricular ejection fraction at MR resulted more depressed in the v+ACM group (44±7 vs 52±2 and 47±2 vs 52±2; p=0.047 and p=0.041). Complex ventricular arrhythmia inducibility at EPS was more frequent in v+ACM (72% vs 34%; p=0.032), while no differences in pathological potentials rate and extension at unipolar and bipolar EAM were found. TCA was performed in 55% and 57% and an ICD was implanted in 29% and 42% in the v+ACM and v-ACM group respectively. No differences in 12-months arrhythmic event rates (39% vs 42%) between the two groups were described. EBM at different magnifications/stains Conclusion In our cohort a viral infection super-imposed to the fibrofatty infiltration was found in 15% of the patients. ACM pts testing positive for viral genome at the EMB had a more severe arrhythmic disease presentation, a more impaired heart function, and a higher rate of complex ventricular arrhythmias at disease presentation, but seemed to respond as well as viral genome negative ACM to ablative and pharmacological treatment

Objectives The purpose of this study was to assess osimertinib-associated cardiac adverse events (AEs) in a real-world setting, using a retrospective single-center cohort study in Japan. Background Cases of osimertinib-associated cardiac AEs have been reported but remain poorly understood. Methods A total of 123 cases of advanced non–small cell lung cancer (NSCLC) with confirmed EGFR mutations who received osimertinib monotherapy from 2014 to 2019 at the Osaka International Cancer Institute (Osaka, Japan) were evaluated. Cardiac AEs were defined according to Common Terminology Criteria for Adverse Events (CTCAE) version 5.0. Changes in left ventricular ejection fraction (LVEF) and rates of cancer therapeutics-related cardiac dysfunction (CTRCD), defined as a ≥10 % absolute decline in LVEF from baseline to a value of, Central Illustration

Цель – выявить предикторы неэффективности медикаментозной терапии при синдроме дилатационной кардиомиопатии (ДКМП) различной этиологии, сопоставить результаты больных с плохим ответом на лечение, умерших и перенесших трансплантацию сердца (ТС), уточнить критерии и сроки отбора на ТС. Материал и методы. В исследование включен 331 больной с синдромом ДКМП, 103 женщины и 228 мужчин (31,1 и 68,9%), средний возраст – 48 (37; 58) лет, от 16 до 77 лет. Критерии включения: возраст от 16 лет, конечно-диастолический размер левого желудочка (КДР ЛЖ) >5,5 см, фракция выброса (ФВ) 2,0), II степень и более трикуспидальной регургитации. ФВ была достоверно ниже у погибших/перенесших ТС в сравнении с выжившими без ТС (33,3±8,9 vs 25,5±10,4%, р, Aim – to identify predictors of the ineffectiveness of drug therapy for dilated cardiomyopathy syndrome (DCM), taking into account the etiology of the disease, to compare patients with poor response to treatment, those who died and those who underwent heart transplantation (HT), to specify criteria and timing of selection for HT. Material and methods. 331 patients with DCM syndrome were included in the study, 103 women and 228 men (31.1 and 68.9%), average age was 48 (37; 58) years old (16 to 77 y.o.). The inclusion criteria were age 16 years, the left ventricle end diastolic size (LV EDS) more than >5.5 cm, left ventricle ejection fraction (LV EF)

Purpose: The aim of this study was to quantify the value of various clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in comparison with morphological studies of the myocardium. Methods: In 100 patients (65 men, 44.7 ± 12.5 years old) with “idiopathic” arrhythmias (n = 20) and dilated cardiomyopathy (DCM; n = 80), we performed the following: 71 endomyocardial biopsies (EMB), 13 intraoperative biopsies, 5 studies of explanted hearts, and 11 autopsies with virus investigation (real-time PCR) of the blood and myocardium. Antiheart antibodies (AHA) were also measured as well as cardiac CT (n = 45), MRI (n = 25), and coronary angiography (n = 47). The comparison group included 50 patients (25 men, 53.7 ± 11.7 years old) with noninflammatory heart diseases who underwent open heart surgery. Results: Active/borderline myocarditis was diagnosed in 76.0% of the study group and in 21.6% of patients in the comparison group (p < 0.001). The myocardial viral genome was observed more frequently in patients in the comparison group than in the study group (65.0 and 40.2%; p < 0.01). We evaluated the diagnostic value of noninvasive markers of myocarditis. The panel of AHA had the greatest importance in the identification of myocarditis: sensitivity was 81.5%, and the positive and negative predictive values were 75.0 and 60.5%. This defined the diagnostic value of noninvasive markers of myocarditis and established a diagnostic algorithm providing an individual assessment of the likelihood of myocarditis development. Conclusion: AHA have the greatest significance in the diagnosis of latent myocarditis in patients with “idiopathic” arrhythmias and DCM. The use of a complex of noninvasive criteria allows the probability of myocarditis to be estimated and the indications for EMB to be determined.

Aim. To study the association between morphological changes and comorbidities and atrial fibrillation (AF) catheter ablation efficacy, and to evaluate morphological predictors of AF recurrence in patients with durable pulmonary vein isolation (PVI) after catheter intervention.Material and methods. Fifty-four patients with paroxysmal/persistent AF without severe structural heart disease were enrolled in this study who underwent primary pulmonary vein cryoballoon ablation (CBA) with simultaneous implantation of an electrocardiogram loop recorder (Medtronic Reveal XT) and interatrial/interventricular septum biopsy. The follow-up duration was 12 months with scheduled 3, 6 and 12 month visits. Patients with recurrent AF were referred for redo procedure, where the PVI durability was assessed. The follow-up duration after the second procedure was also 12 months. The patients were divided into two groups: patients without recurrent AF and patients with recurrent AF after two catheter interventions.Results. In interventricular septum biopsy samples we found the morphological criteria of myocarditis in 70% of the patients (n=34). Individual morphological changes were detected in all patients. The most common types were cardiomyocyte hypertrophy — 98,1% (n= 52), overcrossing of cardiomyocytes — 77,4% (n=41) and lymphohistiocytic infiltration of the interstitium by more than 14 cells — 75,5% (n=40). Patients with persistent AF were significantly more likely to have endothelial cell swelling (55% vs 45%, p=0,022). Interstitial tissue edema was the only morphological parameter significantly associated with AF recurrence (p=0,03).Conclusion. In patients with AF and no structural heart disease who underwent drug-resistant AF cryoballoon ablation, morphological changes in the myocardium of the atria and ventricles are detected in 100% of cases. The predominant biopsy diagnosis is myocarditis. Morphological signs of the inflammatory process activity, as interstitial tissue edema are associated with the high incidence of non-venous AF. Further preoperative evaluation is needed to identify patients with non-venous atrial fibrillation, which will increase the effectiveness of interventional approach.

35 year old patient with Ebstein anomaly and operated atrial septal defect (ASD) was referred to our hospital due to VDD pacemaker lead dysfunction. We decided to remove dysfunctional lead via subclavian lead extraction procedure. After pacemaker generator had been removed, lead was cut to insert locking stylet but plucked lead entrapped in subclavian vein. Then 7-F femoral sheath was introduced to femoral vein and plucked lead was pulled back via myocardial biopsy catheter from inferior vena cava. Finally macro snare was used to taken out lead from femoral vein. The procedure was successfully managed without complication.

LncRNA TINCR has been reported to be involved in cardiac hypertrophy, while its involvement in diabetic cardiomyopathy is unknown.In this study, myocardial biopsy and serum collected from patients with diabetic cardiomyopathy, diabetic patients without cardiopathy and healthy controls, and the expression of TINCR in those tissues was detected by quantitative reverse-transcriptase polymerase chain reaction (qRT-PCR). ROC curve analysis was performed to evaluate the diagnostic value of TINCR expression for diabetic cardiomyopathy. Human cardiomyocyte cells (Cat# 12440053, Thermo Fisher Scientific) were treated with high glucose, and the expression of TINCR was detected by qRT-PCR. TINCR expression was transfected into cardiomyocyte cells and cell apoptosis under high glucose treated was detected by cell apoptosis assay.We found that TINCR expression level in myocardial biopsy and serum was significantly lower in patients with diabetic cardiomyopathy than in diabetic patients without cardiopathy and healthy controls, while no significant differences were found between diabetic patients without cardiopathy and healthy controls. TINCR expression level can be used to effective diagnose diabetic cardiomyopathy. High glucose treatment showed no significant effects on the expression of TINCR in human cardiomyocyte cells, and TINCR overexpression inhibited apoptosis of cardiomyocytes under high glucose treatment.Therefore, we conclude that lncRNA TINCR is downregulated in diabetic cardiomyopathy and it can inhibit cardiomyocyte apoptosis.

Introduction. Extracorporeal cardiopulmonary resuscitation (ECPR) is a challenging approach for treating refractory out-of-hospital cardiac arrest (OHCA). Case Presentation. The authors describe a case of a 40-year-old Caucasian female who suffered from refractory OHCA, was admitted to a hospital while receiving ongoing cardiopulmonary resuscitation, and was connected to venoarterial extracorporeal membrane oxygenation 73 minutes after collapse. Ventricular tachyarrhythmias alternating with pulseless electrical activity resolved after eight hours. Following complete cardiac and neurological recovery, only adenoviral genome was found in myocardial biopsy. After 11 months, another episode of identical arrhythmias was rescued by an implantable cardioverter-defibrillator. Conclusion. Adequate prehospital and early hospital logistics is a prerequisite for successfully implementing extracorporeal cardiopulmonary resuscitation for refractory OHCA.