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The paper describes the investigation of matrix metalloproteinase type 3 (MMP-3) -1171 5A/6A gene polymorphic alleles variants (rs35068180) in patients with dilated cardiomyopathy. A allele and 6А6А genotype of MMP-3 -1171 5A/6A gene (rs35068180) were determined as new genetic predictors of dilated cardiomyopathy development. Aim. To study the association of polymorphism -1171 5A/ of the MMP-3 gene with dilated cardiomyopathy of various origins. Material and methods. The main study group comprised 221 patients with dilated cardiomyopathy (DCM) of different origin. Their average age was 55.309.69 years. Among them there were 111 persons with DCM of ischemic origin, including 99 (89.2%) men and 12 (10.8%) women. The average age of the subjects with DCM was 51.739.74 years, the age of the male subgroup was 51.008.96 years, and the age of the female subgroup was 57.753.71 years. A total of 110 patients with idiopathic cardiomyopathy were included in the study. Among 221 patients, 110 persons did not demonstrated idiopathic dilated cardiomyopathy as the cause of myocardium dilation. This group comprised 100 (91.5%) male patients and 10 (8.5%) female patients. The control group of subjects (221 persons) was represented by healthy people without diseases of the cardiovascular system. The average age of control subjects was 53.64.8 years. We examined all patients in the main group using routine laboratory and instrumental methods, as well as coronary angiography. If myocarditis was suspected, we did an MRI of the heart. Genotyping of polymorphism -1171 5A/6A (rs35068180) of the MMP-3 gene was performed using PCR. Results. Among patients of the main group with dilated myocardial remodeling of various Genesis, the allele was documented in 65.8% of cases against 59.3% among the control group, p=0.044. The homozygous genotype of the MMP-3 gene in patients of the main group was verified in 42.1% of patients against 32.6% of cases in relatively healthy individuals (p=0.099). Conclusion. We have proved the predominance of 6A allele and 6А6А genotype of the MMP-3 gene in the group of patients with DCM. It seems that it is homozygous 6A allele that causes a decrease in the activity of the transcription process and change in the level of stromelysin in arterial walls. This contributes to the activation of type 1 procollagenase, extracellular matrix deposition and cardiac muscle remodeling.

Background The epidemiology of diabetes and idiopathic cardiomyopathy have limited data. We investigated the overall and the age-, sex-, and urbanization-specific incidence and relative hazard of idiopathic cardiomyopathy in association with type 2 diabetes and various anti-diabetic medications used in Taiwan. Methods A total of 474,268 patients with type 2 diabetes were identified from ambulatory care and inpatient claims in 2007–2009 from Taiwan’s National Health Insurance (NHI) database. We randomly selected 474,266 age-, sex-, and diagnosis date-matched controls from the registry of NHI beneficiaries. All study subjects were linked to ambulatory care and inpatient claims (up to the end of 2016) to identify the possible diagnosis of idiopathic cardiomyopathy. The person-year approach with Poisson assumption was used to estimate the incidence, and Cox proportional hazard regression model with Fine and Gray’s method was used to estimate the relative hazards of idiopathic cardiomyopathy in relation to type 2 diabetes. Results The overall incidence of idiopathic cardiomyopathy for men and women patients, respectively, was 3.83 and 2.94 per 10,000 person-years, which were higher than the corresponding men and women controls (2.00 and 1.34 per 10,000 person-years). Compared with the control group, patients with type 2 diabetes were significantly associated with an increased hazard of idiopathic cardiomyopathy (adjusted hazard ratio [aHR]: 1.60, 95% confidence interval [CI]: 1.45–1.77] in all age and sex stratifications except in those men aged > 64 years. Patients with type 2 diabetes aged Conclusions In Taiwan, diabetes increased the risk of idiopathic cardiomyopathy in both sexes and in all age groups, except in men aged > 64 years. Younger patients were vulnerable to have higher HRs of idiopathic cardiomyopathy. Some anti-diabetic medications may reduce the risks of cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy that is caused by heart failure secondary to a dysfunction of the left ventricle at the end of pregnancy or in the first months following childbirth. The diagnosis is performed by electrocardiogram, radiography of the thorax and increase of natriuretic peptides. Bedside radiography can contribute with data that help early diagnosis. Treatment is carried out following clinical guidelines for heart failure, taking into account potentially teratogenic drugs. The importance of this pathology lies in that it affects women at a fertile age and is potentially mortal, which is why there must be a high index of suspicion for its diagnosis and a differential diagnosis with other entities. In this clinical note we present a series of cases of PPCM with the goal of reviewing the diagnosis and treatment of this entity.

Chronic Chagas cardiomyopathy (CCC) is one of the deadliest cardiomyopathies known and the most severe manifestation of Chagas disease, which is caused by infection with the parasite Trypanosoma cruzi. Idiopathic dilated cardiomyopathies (IDC) are a diverse group of inflammatory heart diseases that affect the myocardium and are clinically similar to CCC, often causing heart failure and death. While T-cells are critical for mediating cardiac pathology in CCC and IDC, the mechanisms underlying T-cell function in these cardiomyopathies are not well-defined. In this study, we sought to investigate the phenotypic and functional characteristics of T-cell subpopulations in CCC and IDC, aiming to clarify whether the inflammatory response is similar or distinct in these cardiomyopathies. We evaluated the expression of systemic cytokines, determined the sources of the different cytokines, the expression of their receptors, of cytotoxic molecules, and of molecules associated with recruitment to the heart by circulating CD4+, CD8+, and CD4-CD8- T-cells from CCC and IDC patients, using multiparameter flow cytometry combined with conventional and unsupervised machine-learning strategies. We also used an in silico approach to identify the expression of genes that code for key molecules related to T-cell function in hearts of patient with CCC and IDC. Our data demonstrated that CCC patients displayed a more robust systemic inflammatory cytokine production as compared to IDC. While CD8+ T-cells were highly activated in CCC as compared to IDC, CD4+ T-cells were more activated in IDC. In addition to differential expression of functional molecules, these cells also displayed distinct expression of molecules associated with recruitment to the heart. In silico analysis of gene transcripts in the cardiac tissue demonstrated a significant correlation between CD8 and inflammatory, cytotoxic and cardiotropic molecules in CCC transcripts, while no correlation with CD4 was observed. A positive correlation was observed between CD4 and perforin transcripts in hearts from IDC but not CCC, as compared to normal tissue. These data show a clearly distinct systemic and local cellular response in CCC and IDC, despite their similar cardiac impairment, which may contribute to identifying specific immunotherapeutic targets in these diseases.

Peripartum cardiomyopathy (PPCM) is defined as idiopathic cardiomyopathy which occurs at the end of pregnancy or in the first few months after delivery, with symptoms of heart failure (HF) secondary to left ventricular dysfunction, and at the same time no other cause for this condition. The pathomechanism of the disease has not yet been fully understood, but it is probably based on the interaction of complex factors. The clinical course of PPCM varies from life-threatening acute heart failure to mild symptoms mimicking the symptoms typical of the perinatal period. In Europe, PPCM is a rare disease but there are areas of the world where it occurs in one in every 300 cases. The treatment of PPCM is similar to that of HF with reduced ejection fraction. However, it is important to exclude drugs with teratogenic effects during pregnancy. The inclusion of bromocriptine in PPCM therapy seems to be justified, and in some cases improves the prognosis.

Advances in the diagnostics and treatment of cancer have led to improved survival of patients and decreased mortality. Cardiovascular complications (CVC) however, are among the most frequent side effects of anticancer treatment, which can have an adverse effect on the prognosis. This may be the result of cardiotoxicity, which involves direct effects of the radiation therapy and /or chemotherapy on the heart function and structure. Heart failure with cancer therapy was associated with a 3.5-fold increase in the risk of mortality compared with idiopathic cardiomyopathy. The cardiotoxicity risk assessment is often difficult, due to the different susceptibility of patients to drugs and their combinations, and coadministration of radiation therapy. Therefore, the likelihood of development and the time of occurrence of cardiotoxicity may vary and not always depend on the initial risk. Strategies for managing such patients are in most cases based on the expert opinion. This article provides recommendations for the prevention and therapy of cardiotoxicity, set out in the consensus of the European Society of Cardiology 2016.

Isolated left ventricular non-compaction (ILVNC) is a rare, congenital, idiopathic cardiomyopathy that may present in adulthood. There is no true gold standard for the diagnosis of ILVNC. Two-dimensional echocardiography with colour Doppler is the modality of choice to diagnose the condition. However, the diagnosis should be confi rmed with cardiac magnetic resonance imaging (CMRI), as well as either a positive family history, complications of ILVNC or confi rmatory genetic testing. We describe the clinical and echocardiographic features in 4 patients, each with a possible diagnosis of ILVNC, in the setting of potential alternative aetiologies for heart failure. Approval to present these cases was obtained from the institutional ethics committee and the patients also provided consent. Suffi cient transthoracic echocardiographic (TTE) evidence of ILVNC according to previously published criteria was found in all the cases, although it was not confi rmed with CMRI. This case series highlights the importance of routine echocardiography in all patients who present with heart failure – irrespective of associated risk factors. We caution against over-diagnosis of ILVNC with TTE alone, and recommend the use of CMRI as a second-line diagnostic investigation. Screening of family members and prevention of complications of confi rmed cases of ILVNC are important.

Background Barth syndrome (BTHS) is a rare X-linked recessive disorder characterized by clinical features including cardiomyopathy, skeletal myopathy, neutropenia, growth delay, and exercise intolerance. It is often considered to be a paediatric disease, owing to most cases being diagnosed during childhood and mortality being the highest during the first few years of life. Case summary We report a case of dilated cardiomyopathy due to BTHS in a 27-year-old adult male patient, who initially presented with lightheadedness, dyspnoea, orthopnoea, and bilateral lower extremity oedema. Key findings from investigations included leukopenia, prolonged QTc interval, reduced left ventricular ejection fraction (LVEF), global enlargement of all heart chambers, patent coronary arteries, and mild pulmonary hypertension. The patient was diuresed to euvolemia and discharged with a LifeVest. Guideline-directed medical therapy was initiated and uptitrated as an outpatient. A repeat echocardiogram 2 years after initial presentation showed marked improvement in LVEF. Discussion It is possible that there are adult patients with idiopathic cardiomyopathy, which may be attributable to BTHS. In the absence of an obvious underlying cause, with the appropriate historical information, clinical exam, laboratory investigations, and imaging findings, BTHS should be considered as a likely cause of non-ischaemic cardiomyopathy.

Background Pediatric cardiomyopathy is a genetically heterogeneous disease with substantial morbidity and mortality. Current guidelines recommend genetic testing in children with hypertrophic, dilated, or restrictive cardiomyopathy, but practice variations exist. Robust data on clinical testing practices and diagnostic yield in children are lacking. This study aimed to identify the genetic causes of cardiomyopathy in children and to investigate clinical genetic testing practices. Methods and Results Children with familial or idiopathic cardiomyopathy were enrolled from 14 institutions in North America. Probands underwent exome sequencing. Rare sequence variants in 37 known cardiomyopathy genes were assessed for pathogenicity using consensus clinical interpretation guidelines. Of the 152 enrolled probands, 41% had a family history of cardiomyopathy. Of 81 (53%) who had undergone clinical genetic testing for cardiomyopathy before enrollment, 39 (48%) had a positive result. Genetic testing rates varied from 0% to 97% between sites. A positive family history and hypertrophic cardiomyopathy subtype were associated with increased likelihood of genetic testing ( P =0.005 and P =0.03, respectively). A molecular cause was identified in an additional 21% of the 63 children who did not undergo clinical testing, with positive results identified in both familial and idiopathic cases and across all phenotypic subtypes. Conclusions A definitive molecular genetic diagnosis can be made in a substantial proportion of children for whom the cause and heritable nature of their cardiomyopathy was previously unknown. Practice variations in genetic testing are great and should be reduced. Improvements can be made in comprehensive cardiac screening and predictive genetic testing in first‐degree relatives. Overall, our results support use of routine genetic testing in cases of both familial and idiopathic cardiomyopathy. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01873963.

Introduction Peripartum cardiomyopathy (PPCM) is a rare idiopathic cardiomyopathy frequently presenting with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery. Within the published literature, there are case reports extolling the safety of both regional and general anesthetic interventions in PPCM. However, there is an absence of high-quality evidence to define a suitable paradigm for peri-operative care. In the absence of a large prospective case series or clinical trials, the synthesis of clinical data from published case reports provides an opportunity to distil published clinical data and explore the effect of clinical interventions. Evidence acquisition A systematic search of English articles English language case reports published between 1986 and 2020 within multiple databases. Clinical data was extracted and aggregated into a database for analysis. Evidence synthesis Gestational hypertension and pre-eclampsia were pre-partum risk factors. 403 case reports provided 466 individual cases from 48 countries. Neither regional nor general anesthetic interventions in the peripartum period have a discernible impact on the outcome of patients with PPCM. Rapid unpredictable deterioration in the peripartum period, requiring mechanical cardiac support or heart transplantation is described. The mortality of PPCM is 5-6%. Conclusions Patients with PPCM are at risk of rapid unpredictable decline. Management within specialist centers should be considered. Although the data is unsuitable to provide a comprehensive paradigm for the anesthetic and critical care management of PPCM, the observations provide a direction for future clinical audits and trials.

End-stage heart disease can be a result of idiopathic cardiomyopathy, chronic ischemic damage or long-standing volume or pressure overload from valvular pathology. While cardiac transplantation may be the ultimate therapy for the fortunate patient who is eligible, the number of available organs is outweighed by the far larger number of patients who require advanced therapies. Mechanical circulatory support was initially introduced to either replace the native heart (in a total heart configuration) or to assist the weakened left ventricle until a suitable donor organ became available. As the technology advanced and permitted outpatient management, the improved quality of life and more recently the prolonged use of mechanical circulatory support (>5 years) has led to a dramatic increase in the utilization of durable, implantable continuous-flow left ventricular assist devices (LVADs). This chapter will review the perioperative considerations of managing LVAD patients and will briefly discuss important intraoperative issues.

End-stage heart disease can be a result of idiopathic cardiomyopathy, chronic ischemic damage or long-standing volume or pressure overload from valvular pathology. While cardiac transplantation may be the ultimate therapy for the fortunate patient who is eligible, conventional surgical interventions can be considered in highly selected cases. This chapter reviews the current evidence supporting the decision to provide high risk conventional surgery and summarizes the current indications and results of heart transplantation.

Aim. To study the relationship of matrix metalloproteinase-3 (MMP3) genetic polymorphism and dilated ischemic cardiomyopathy (DCM), as well as idiopathic cardiomyopathy (ICM) of unknown etiology.Material and methods. A total of 221 patients with DCM and ICM were examined (mean age, 55,30±9,69 years). The group of ischemic DCM consisted of 111 people (99 men (89,2%) and 12 women (10,8%)). The mean age of DCM subjects was 51,73±9,74 years (male subgroup, 51,00±8,96 years; female subgroup, 57,75±3,71 years). The ICM group consisted of 110 people (100 men (91,5%) and 10 women (8.5%)). The mean age of ICM subjects was 58,68±8.38 years (male subgroup, 58,29±8.,6 years; female subgroup, 62,90±6,29 years). The control group of subjects (n=121) consisted of healthy people without cardiovascular diseases (mean age, 53,6±4,8 years). All patients of the experimental group underwent routine diagnostic tests, as well as coronary angiography. In case of suspected myocarditis, cardiac magnetic resonance imaging was performed. All patients underwent polymerase chain reaction to determine the MMP3-11715A/6A polymorphism (rs35068180).Results. In patients with cardiomyopathy, regardless of the disease origin, significant differences were verified in comparison with the control group. Allele 6A (65,8% vs 59,3%, p=0,044) and genotype 6A/6A (42,1% vs 32,6%, p=0,099) were found significantly more frequently in patients with cardiomyopathy than in the control group. In addition, despite various etiological factors, the pathogenetic involvement of MMP3 is likely to have a general direction.Conclusion. In all patients with cardiomyopathy, the prevalence of MMP3 gene A allele was shown. Due to decrease in the transcription activity in homozygous 6A allele, the stromelysin level in arterial walls also decreases. This promotes the activation of procollagenase-1, the deposition of extracellular matrix and cardiac remodeling

Coronavirus disease 2019 (COVID-19) is challenging the care for cardiovascular patients, resulting in serious consequences with increasing mortality in pre-diseased heart failure patients. In the current state of the pandemic, the physiopathology of COVID-19 affecting pre-diseased hearts and the management of terminal heart failure in COVID-19 patients remain unclear. We outline the findings of a young COVID-19 patient suffering from idiopathic cardiomyopathy who was treated for acute multi-organ failure and required cardiac surgery with implantation of a temporary right ventricular and durable left ventricular assist device (LVAD). For deeper translational insights, we used in-depth tissue analysis by electron and light sheet fluorescence microscopy revealing evidence for spatial distribution of severe acute respiratory syndrome coronavirus 2 in the heart. This indicates that in-depth analysis may represent a valuable tool in understanding indistinct clinical cases. We conclude that COVID-19 directly affects pre-diseased hearts, but the consequences can be treated successfully with LVAD implantation. OA Förderung 2020

BackgroundPeripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with acute heart failure during the peripartum period. It is common in patients of African ancestry. Currently, there is paucity of data on the burden, predictors and outcomes of PPCM in Uganda. This study aimed to investigate the prevalence, predictors and six-month outcomes of PPCM in an adult cohort attending a tertiary specialised cardiology centre in Kampala, Uganda.MethodsThis study consecutively enrolled 236 women presenting with features of acute heart failure in the peripartum period. Clinical evaluation and echocardiography were performed on all the enrolled women. PCCM was defined according to recommendations of the Heart Failure Association of the European Society of Cardiology Working Group on PPCM. Poor outcome at six months of follow-up was defined as presence of any of the following: death of a mother or her baby, New York Heart Association (NYHA) functional class III-IV or failure to achieve complete recovery of left ventricular function (left ventricular ejection fraction ≤55%).ResultsThe median age, BMI and parity of the study participants was 31.5 (25.5-38.0) years, 28.3 (26.4-29.7) and 3 (2-4) respectively. The prevalence of PPCM was 17.4% (n = 41/236). Multiple pregnancy was the only predictor of PPCM in this study population (OR 4.3 95% CI 1.16-16.05, p = 0.029). Poor outcome at six-months was observed in about 54% of the patients with PPCM (n = 4, 9.8% in NYHA functional class III-IV and n = 22, 53.7% with LVEF ConclusionPPCM is relatively common in Uganda and is associated with multiple pregnancy. Poor outcomes especially absence of complete recovery of left ventricular function are also common. Large studies to further investigate long-term maternal and foetal outcomes in Uganda are justified.

Background Syndromic dilated cardiomyopathy (DCM) includes a group of complex disorders with a very heterogeneous genetic etiology, leading to delay in definitive diagnosis. Conversely, an early genetic diagnosis is very important in determining the disease course, the prognosis, and may guide personalized treatments and family counseling. Methods We analyzed two brothers with a multisystemic disorder, including dilated cardiomyopathy, diabetes, bilateral neurosensorial hearing loss, and optic atrophy, using different genetic approaches, namely mitochondrial DNA sequencing, comparative genomic hybridization‐array (a‐CGH) and whole exome sequencing (WES). Results Sequencing of the wide mitochondrial genome revealed, in both brothers, the known homoplasmic variant rs2853826 in the subunit 3 of the NADH dehydrogenase gene (MT‐ND3), whose pathogenicity was conflicting. Comparative genomic hybridization‐array analysis revealed in both patients and their father two heterozygous deletions in Phosphodiesterase 4d‐Interacting Protein (PDE4DIP) and Protocadherin‐related 15 (PCDH15) genes, respectively. The use of WES detected a pathogenetic mutation in ALMS1, enabling the definitive diagnosis of Alström syndrome. Conclusion We demonstrated how the diagnosis of a complex heterogeneous disease may be difficult, due to several overlapping manifestations and the possible interaction of more genetic variants that could lead to a more severe and complex phenotype. This paper strongly evidences how genomics is revolutionizing the diagnosis of rare complex disease, representing one of the most essential steps to enable a definitive diagnosis and to establish the etiology for diseases, such as syndromic DCM., The diagnosis of a complex heterogeneous disease may be difficult, due to several overlapping manifestations and the possible interaction of more genetic variants. By using different genetic approaches we obtained a definitive diagnosis of Alström syndrome in two brothers with a multisystemic disorder.

Objective: Exercise-induced premature ventricular complexes (EiPVCs) are often considered as benign arrhythmias, although they are associated with a high risk of all-cause death in the general healthy population. However, an intermediate pathophysiological process remains unclear, particularly in patients with known cardiovascular disease. The aim of this study was to find an association between EiPVCs, the occurrence of life-threatening ventricular arrhythmias (LACO), and all-cause mortality in patients with cardiovascular disease. Methods: This was an observational study of a cohort of patients with coronary artery disease (CAD) or idiopathic cardiomyopathy (ICM). Stress testing was performed as a part of the routine cardiovascular evaluation. The occurrence of EiPVCs was evaluated during exercise testing (ET). At follow-up, long-term occurrence of LACO was evaluated. A bivariate and multivariate analysis was performed. Results: Out of the total of 1442 patients analysed, 700 (49%) had EiPVCs. During 14 years of following-up after ET, 106 LACO outcomes were observed. Long-term all-cause mortality was 4% (n = 61). A bivariate analysis showed that patients with EiPVCs had an increased risk for LACO (RR = 2.81, 95% CI; 1.9–4.3, P

Objectives This study sought to characterize ventricular tachycardia (VT) ablation outcomes across nonischemic cardiomyopathy (NICM) etiologies and adjust these outcomes by patient-related comorbidities that could explain differences in arrhythmia recurrence rates. Background Outcomes of catheter ablation of VT in patients with NICM could be related to etiology of NICM. Methods Data from 2,075 patients with structural heart disease referred for catheter ablation of VT from 12 international centers was retrospectively analyzed. Patient characteristics and outcomes were noted for the 6 most common NICM etiologies. Multivariable Cox proportional hazards modeling was used to adjust for potential confounders. Results Of 780 NICM patients (57 ± 14 years of age, 18% women, left ventricular ejection fraction 37 ± 13%), underlying prevalence was 66% for dilated idiopathic cardiomyopathy (DICM), 13% for arrhythmogenic right ventricular cardiomyopathy (ARVC), 6% for valvular cardiomyopathy, 6% for myocarditis, 4% for hypertrophic cardiomyopathy, and 3% for sarcoidosis. One-year freedom from VT was 69%, and freedom from VT, heart transplantation, and death was 62%. On unadjusted competing risk analysis, VT ablation in ARVC demonstrated superior VT-free survival (82%) versus DICM (p ≤ 0.01). Valvular cardiomyopathy had the poorest unadjusted VT-free survival, at 47% (p Conclusions Catheter ablation of VT in NICM is effective. Etiology of NICM is a significant predictor of outcomes, with ARVC, myocarditis, and DICM having similar but superior outcomes to hypertrophic cardiomyopathy, valvular cardiomyopathy, and sarcoidosis, after adjusting for potential covariates.

Peripartum cardiomyopathy (PPCM) is a rare idiopathic cardiomyopathy having an incidence of less than 0.1%. PPCM is associated with high morbidity and mortality rates ranging from 5 to 32%. In this review, the authors report a series of five PPCM cases. The case reports included pregnant women with PPCM, admitted in the hospital from October 1, 2017 to June 1, 2018 over a period of 9 months who required cesarean section. The authors aim to discuss the presentation, optimization, anesthetic management, and postoperative care of this rare condition. One of the cases was a booked case. The remaining four pregnant patients were referred in late pregnancy with features of congestive cardiac failure. One patient was in acute pulmonary edema, required intubation, and subsequently had cesarean section under general anesthesia. Four patients were managed with incremental epidural anesthesia. The need for proper preoperative optimization and intra- and postoperative management was discussed. The authors had one maternal mortality in our series. There was no neonatal mortality. Early diagnosis of PPCM, prompt treatment of heart failure, planning the delivery, and postpartum care can decrease maternal morbidity and mortality. Incremental epidural dosing can be used for cesarean section and has the advantage of stable hemodynamic status without the risks associated with general anesthesia.

Eosinophilic infiltration is a rare and underrecognized cause of myocarditis associated with prolonged eosinophilia. Before advanced imaging and routine biopsy, patients were diagnosed with an idiopathic cardiomyopathy with subsequent diagnosis made on autopsy. We present 3 cases of eosinophilic myocarditis diagnosed by cardiac biopsy classified as hypereosinophilic syndrome. Two patients presented with severe left ventricular dysfunction, and 1 patient presented with cardioembolic stroke. All patients were successfully treated with glucocorticoid therapy. Our cases highlight the importance of early diagnosis with endomyocardial biopsy and prompt immunosuppressive treatment. Résumé: L’infiltration par les éosinophiles est une cause rare et non reconnue de myocardite associée à une éosinophilie prolongée. Avant l’imagerie avancée et la biopsie systématique, le diagnostic de cardiomyopathie idiopathique était posé après l’autopsie des patients. Nous présentons 3 cas de myocardite à éosinophiles dont la biopsie cardiaque a permis de poser le diagnostic de syndrome d’hyperéosinophilie. Deux patients ont présenté une dysfonction grave du ventricule gauche, et 1 patient a présenté un accident vasculaire cérébral d’origine cardio-embolique. Le traitement par glucocorticoïdes s’est avéré réussi chez tous les patients. Nos cas montrent l’importance du diagnostic précoce par biopsie endomyocardique et du traitement immunosuppresseur hâtif.

Objectives This study sought to characterize the biatrial substrate in heart failure (HF) and persistent atrial fibrillation (PeAF). Background Atrial fibrillation (AF) and HF frequently coexist; however, the contribution of HF to the biatrial substrate in PeAF is unclear. Methods Consecutive patients with PeAF and normal left ventricular (NLV) systolic function (left ventricular ejection fraction [LVEF] >55%) or idiopathic cardiomyopathy (LVEF ≤45%) undergoing AF ablation were enrolled. In AF, pulmonary vein (PV) cycle length (PVCL) was recorded via a multipolar catheter in each PV and in the left atrial appendage for 100 consecutive cycles. After electrical cardioversion, biatrial electroanatomic mapping was performed. Complex electrograms, voltage, scarring, and conduction velocity were assessed. Results Forty patients, 20 patients with HF (mean age: 62 ± 8.9 years; AF duration: 15 ± 11 months; LVEF: 33 ± 8.4%) and 20 with NLV (mean age: 59 ± 6.7 years; AF duration: 14 ± 9.1 months; p = 0.69; mean LVEF: 61 ± 3.6%; p Conclusions HF is associated with significantly reduced biatrial tissue voltage, fractionation, and prolongation of PVCL. Advanced biatrial remodeling may have implications for invasive and noninvasive rhythm control strategies in patients with AF and HF.

Prediction of follow-up shock is crucial to stratify patients with dilated cardiomyopathy (DCM) requiring implantable cardioverter defibrillator (ICD). The objective of the article is to assess the predictive value of endo-myocardial biopsy (EMB) towards ICD shock and follow-up mortality. A series of patients with DCM scheduled for ICD implantation underwent EMB to further determine the genesis of DCM. Presence of fibrosis and inflammation was documented and related to outcomes. A total of 240 patients were referred for ICD as primary (56%) and secondary (44%) prophylaxis. EMB showed myocardial fibrosis in 55.4%, inflammation in 55.7%, and viral genomic material in 60%. Median follow-up was 39 months (1-209). Appropriate and inappropriate shocks occurred in 29.2 and 20.4%. At logistic regression, determinants of appropriate shock were ICD indication for secondary prophylaxis (direct relationship: p = 0.009, OR 3.4, CI 1.3-8.8) and presence of inflammation at EMB (inverse relationship: p = 0.04, OR 0.4, CI 0.1-0.9). Moreover, the sole determinant of inappropriate shock was age at implant (inverse relationship: p = 0.003, OR = 0.9, CI 0.90-0.98). Overall mean estimated survival was 168 months and 5-year survival was 83%. Degree of improvement in LVEF% was the sole determinant of follow-up mortality (inverse relationship p = 0.02; HR = 0.9; CI 0.88-0.99). Present selection criteria for ICDs implant rely mainly on LVEF% that lacks sensitivity and specificity. EMB can identify the substrate of increased or reduced life-threatening arrhythmias. Presence of inflammation is a positive prognostic factor for reduced arrhythmogenic risk, independently by the ICD implantation indication.

Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure due to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found. We report a case of a woman with PPCM who developed a critical cardiogenic shock with repeated cardiopulmonary resuscitation. We show for the first time that mechanical circulatory support combined with high-dose bromocriptine therapy to suppress systemic prolactin levels may serve as an effective therapeutic option in patients with fulminant PPCM and cardiogenic shock. Myocardial cathepsin D was overexpressed in our patient underscoring a potential role of cathepsin D-induced cleavage of prolactin in the pathophysiology of PPCM.

Aims Identification of metabolic signatures in heart failure (HF) patients and evaluation of their diagnostic potential to discriminate HF patients from healthy controls during baseline and exercise conditions. Methods Plasma samples were collected from 22 male HF patients with non-ischemic idiopathic cardiomyopathy and left ventricular systolic dysfunction and 19 healthy controls before (t0), at peak (t1) and 1 h after (t2) symptom-limited cardiopulmonary exercise testing. Two hundred fifty-two metabolites were quantified by gas chromatography-mass spectrometry (GC-MS) and liquid chromatography (LC)-MS/MS-based metabolite profiling. Results Plasma metabolite profiles clearly differed between HF patients and controls at t0 (P

Introduction Among patients who survive heart transplant, recurrent end-stage heart failure is a major cause of morbidity and mortality secondary to acute rejection, primary graft failure or coronary artery vasculopathy, and heart retransplantation (HRT) offers the potential for long-term survival. However, there is controversy about the appropriateness of retransplantation because of limited organ availability and outcomes that tend to be worse than in primary transplantation. Internationally, between 2 and 4% of the available donor hearts are distributed to retransplant patients.1 The aim of this study was to determine the mid-term survival after heart retransplantation. Methods After obtaining Institutional Review Board approval, the authors retrospectively studied all consecutive adult patients who underwent orthotopic heart transplantation between January 2009 and December 2018 at a tertiary care university hospital and followed them up until December 2019. Patients included in the analysis had undergone HRT and were 18 years or older. Recipient demographic data, hemodynamic profile and postoperative outcomes were analyzed. Results During the study period, 250 patients underwent orthotopic heart transplantation. Among these patients, 2% (n=5) underwent HRT. The end-stage heart failure diagnoses leading to first heart transplant in our study were hypertrophic cardiomyopathy (40%), idiopathic cardiomyopathy (40%) and myocarditis (20%); and the major indications for HRT were chronic rejection (60%) and cardiac allograft vasculopathy (40%). Grafts failed at a median follow-up of 9 (5) years after first heart transplantation. The majority of patients (80%) required combined simultaneous second cardiac and first renal transplantation. Heart-kidney combined transplantation were performed with organs coming from the same donor and with no-staged modality. Actuarial survival rate was 60%, after a median follow-up of 17.8 (39.6) months. Discussion Although HRT survival is still inferior to primary transplantation, it has improved over the decades and is a viable treatment option for patients with late failing allografts. Outcome after HRT strongly depends on the indication for retransplantation and the interval between first and second transplantation. Different studies suggest that a diagnosis of cardiac allograft vasculopathy or chronic rejection and an interval between first and second transplantation greater than 1 year have a significantly favorable impact on mortality.2 3 Judicious patient selection and careful perioperative care are of utmost importance considering limited allograft availability.

Pediatric primary cardiomyopathy is rare but serious, having high mortality; hypertrophic and dilated types are the most common. Its etiology has been mainly considered idiopathic; however, next generation sequencing techniques have revealed nearly half of idiopathic pediatric cases arose from specific genetic mutations. Therefore, our study aimed to identify the genetic causes of primary idiopathic cardiomyopathy. Newborns to 15-year old patients with this condition were recruited between March 2016 and May 2017 at Thammasat University Hospital. Complete patient history and physical examination data were collected by a geneticist with cardiac examinations and echocardiograms by pediatric cardiologists. Whole exome sequencing was performed for all. Of the 12 patients enrolled, 5 cases were dilated type and 7 hypertrophic. Two with dilated type were excluded during follow-up as cause was determined (hypocalcemia and pacemaker induced). A list of 118 genes for cardiomyopathy was analyzed in the remaining 10 cases. Pathogenic and likely pathogenic mutations were identified in 5 patients: HRAS, PTPN11, SOS1, FLNC and TXNRD2; half our patients were not actually idiopathic. Despite its high cost, genetic testing is useful for determining familial risk as well as predicting patient cardiomyopathy progress.