Different Types Of Cardiomyopathies Research Articles

Monogenic diseases associated with cardiomyopathy genes and their phenotypic manifestations

The aim of the present study was to summarize the data on the spectrum of genetic diseases and their phenotypic manifestations in case of structural and functional defects in 75 genes, pathogenic variants of which are associated with the formation of different types of cardiomyopathy (CMP). The search for scientific publications was carried out in foreign (PubMed) and Russian (eLibrary) digital libraries. The data analysis was performed using the Simple ClinVar, An Online Catalog of Human Genes and Genetic Disorders, and STRING databases.It was shown that the vast majority of CMP genes are pleiotropic. Monogenic diseases caused by mutations in CMP genes are characterized by a wide range of pathological manifestations in various organs and systems (cardiovascular, nervous, endocrine, musculoskeletal systems, connective tissue, skin and appendages, organs of vision and hearing, kidneys) as well as by metabolic and immune disorders. Therefore, if a patient (regardless of the primary diagnosis) has pathogenic / likely pathogenic variants or variants of uncertain significance in the CMP genes, we recommend a detailed and comprehensive clinical examination. This is important for clarifying the effects of rare genetic variants, identifying significant clinical and prognostic features for CMP and monogenic diseases associated with CMP genes, and identifying risk groups and controllable triggers that contribute to the manifestation of pathogenic genetic variants.

Intestinal microbiota and metabolome perturbations in ischemic and idiopathic dilated cardiomyopathy

BackgroundVarious clinical similarities are present in ischemic (ICM) and idiopathic dilated cardiomyopathy (IDCM), leading to ambiguity on some occasions. Previous studies have reported that intestinal microbiota appeared dysbiosis in ICM, whether implicating in the IDCM remains unclear. The aim of this study was to assess the alterations in intestinal microbiota and fecal metabolites in ICM and IDCM.MethodsICM (n = 20), IDCM (n = 22), and healthy controls (HC, n = 20) were enrolled in this study. Stool samples were collected for 16S rRNA gene sequencing and gas chromatography-mass spectrometry (GC–MS) analysis.ResultsBoth ICM and IDCM exhibited reduced alpha diversity and altered microbial community structure compared to HC. At the genus level, nine taxa including Blautia, [Ruminococcus]_torques_group, Christensenellaceae_R-7_group, UCG-002, Corynebacterium, Oceanobacillus, Gracilibacillus, Klebsiella and Citrobacter was specific to ICM, whereas one taxa Alistipes uniquely altered in IDCM. Likewise, these changes were accompanied by significant metabolic differences. Further differential analysis displayed that 18 and 14 specific metabolites uniquely changed in ICM and IDCM, respectively. The heatmap was generated to display the association between genera and metabolites. Receiver operating characteristic curve (ROC) analysis confirmed the predictive value of the distinct microbial-metabolite features in disease status. The results showed that microbial (area under curve, AUC = 0.95) and metabolic signatures (AUC = 0.84) were effective in discriminating ICM from HC. Based on the specific microbial and metabolic features, the patients with IDCM could be separated from HC with an AUC of 0.80 and 0.87, respectively. Furthermore, the gut microbial genus (AUC = 0.88) and metabolite model (AUC = 0.89) were comparable in predicting IDCM from ICM. Especially, the combination of fecal microbial-metabolic features improved the ability to differentiate IDCM from ICM with an AUC of 0.96.ConclusionOur findings highlighted the alterations of gut microbiota and metabolites in different types of cardiomyopathies, providing insights into the pathophysiological mechanisms of myocardial diseases. Moreover, multi-omics analysis of fecal samples holds promise as a non-invasive tool for distinguishing disease status.Graphical

Epigenetics of Cardiomyopathy: Histone Modifications and DNA Methylation

Cardiomyopathy is clinically and genetically heterogeneous group of pathologies of myocardium that are being actively studied by researchers. It is now generally accepted that, along with genetic factors, epigenetic mechanisms can be significant in both risk for cardiomyopathy and different clinical manifestations of the disease. This article provides an overview of scientific publications devoted to the study of histone modifications and chromatin remodeling, as well as DNA methylation changes in different types of cardiomyopathy. Most of the reports focused on epigenome profiling of myocardium of patients with dilated cardiomyopathy. The development of cardiomyopathy (dilated, hypertrophic, ischemic, arrhythmogenic, and restrictive) is associated with epigenetic changes of myocardium and this leads to gene expression alteration and metabolic pathways imbalance with pathogenetic significance for heart diseases. The genes of cardiomyopathies (LMNA, TNNI3, ANKRD1, SLC25A4, EYA4, GATAD1, PRDM16, and DMD) are also involved in epigenetic changes of myocardium. Epigenetic modifications, and enzymes that regulate epigenetic processes, are promising for the identification of new molecular markers and metabolic pathways significant for cardiomyopathies, as well as for the development of diagnostic panels and new drugs. At the same time, the high clinical and etiological heterogeneity of cardiomyopathies, a large number of diverse and interrelated epigenetic processes that occur both under physiological conditions and during the pathogenesis of the disease indicate the need to expand epigenetic studies in various forms of cardiomyopathies, including epigenome, transcriptome, and epitranscriptome levels using omics analysis of single cells of myocardium in humans and model animals, as well as in cell lines in disease modeling.

Arrhythmias : Update on ablations and devices

The field of invasive electrophysiology is technically evolving and especially the catheter ablation treatment of symptomatic atrial fibrillation (AF). The technically innovative method of so-called electroporation (pulsed field ablation, PFA) is characterized by arapid and effective treatment of AF. The current study data confirm ahigh success rate for ablation and a good safety profile in the treatment of paroxysmal and persistent AF. In the field of radiofrequency ablation (RF) of AF the modified form of energy transfer, the very high-power short-duration (vHPSD) protocols, show good results and can reduce the procedural time. There are also technical innovations in other single-shot devices. There is adevice based on the RF technique that could show good clinical results in an initial study and can combine the targeted delivery of RF energy with the advantages of asingle-shot device. For ventricular tachycardia (VT) there are innovations in the diagnostics and clarification in the new European guidelines that were presented in August 2022. These make individual recommendations for different types of cardiomyopathy. There are also technical developments in the field of active rhythm implants. In cardiac pacemaker treatment and specifically for conduction system pacing (CSP) there is evidence for a targeted stimulation of the bundle of His or left bundle branch pacing (LBBP). This form of stimulation is particularly advantageous for patients with heart failure and abroad QRS complex. For leadless pacemakers (leadless pacing) there are now good long-term results and also atwo-chamber approach.

P2X7 purinergic receptor: A potential target in heart diseases (Review).

The P2X7 purinergic receptor (P2X7R) is a non‑selective cation channel activated by high levels of adenosine triphosphate that are commonly present in serious conditions. Activation of this purinergic receptor is closely related to the development of various disease states including inflammatory and neurodegenerative disorders, orthopedic diseases and types of cancer. Accumulating evidence has shown that the P2X7R plays a crucial role in the development of various heart diseases. For example, activation of P2X7Rs may alleviate myocardial ischemia‑reperfusion injury by releasing endogenous cardiac protective substances. In contrast to these findings, activation of P2X7Rs can promote the development of acute myocardial infarction and myocarditis by inducing inflammatory responses. Activation of these receptors can also contribute to the development of different types of cardiomyopathies including diabetic cardiomyopathy, dilated cardiomyopathy and hypertrophic cardiomyopathy by inducing cardiac hypertrophy, fibrosis and apoptosis. Notably, inhibition of P2X7Rs can improve cardiac structure and function abnormalities following acute myocardial infarction, reduction of inflammatory responses following myocarditis and attenuation of the cardiomyopathy process. Furthermore, recent evidence has demonstrated that P2X7Rs are highly active in patients infected with coronavirus disease‑2019 (COVID‑19). Hyperactivation of P2X7Rs in COVID‑19 may induce severe myocardial injury through the activation of several signaling pathways. The present study reviewed the important role of the P2X7R in cardiac dysfunctions and discusses its use as a possible new therapeutic approach for the prevention and treatment of several myocardial diseases.

Retrospective Molecular Analysis in Five Cases Probably Associated with Sudden Death the National Institute of Legal Medicine and Forensic Sciences of Colombia

In recent years, significant advances have been made in understanding the genetic factors that predispose to sudden cardiac death, finding multiple affected genes that cause arrhythmic disorders, which could trigger sudden death in structurally normal hearts, to determine these genetic variants. It has an important role as a complement in autopsies of deaths to be determined. Cases and controls were analyzed through the study of the exome by next-generation sequencing. The variants were filtered following international recommendations, different software was used to determine the possible variants associated with cardiomyopathies and cardiac channelopathies. Twelve structural variants were found in six genes associated with different types of cardiomyopathies. Seven variants were found in six genes associated with cardiac channelopathies, and additionally, twenty-one variants were found in twelve genes of uncertain significance, four variants may be of clinical relevance. In deaths whose causes remain to be determined after performing the autopsy and considering negative toxicological, virologic, and microscopy results, it is extremely important to carry out a molecular analysis because the cause of death is possibly due to a channelopathy or an arrhythmia that is difficult to detect during the autopsy.

T1 and T2 mapping – basic principles and clinical application

Cardiac magnetic resonance tomography is widely used method in the diagnosis of cardiovascular diseases. In the last decade, new techniques have been developed to obtain quantitative parameters of myocardial changes. T1 and T2 mapping are part of the routine CMRT protocol and allow direct quantification of T1 and T2 relaxation times in the myocardium as well as the calculation of extracellular volume. These are important biomarkers both for the diagnosis of various myocardial diseases and for monitoring treatment follow-up and determining prognosis. The purpose of this article is to provide a brief overview of the basic principles of the T1 and T2 mapping techniques, as well as their main applications in different types of cardiomyopathies.

Mortality and predictors of death in women and men with congestive heart failure of different types of cardiomyopathies

Abstract Background Congestive heart failure (CHF) is one of the leading causes of death from cardiovascular disease and years lived with disability. Studies showed that women had better survival than men despite higher hospitalizations in women. However, there is evidence of a gender-associated risk of dying of heart failure of different types of cardiomyopathies (CMP). Purpose To analyze the mortality of CHF due to different types of CMP in women and men Methods From February 2017 to September 2020, we analyzed the mortality and the predictors of death in women and men with CHF (Framingham criteria) in five types of CMP (ischemic, idiopathic, hypertensive, Chagas, and valve disease). Baseline data included clinical characteristics and echocardiographic findings. Statistical analyses used the Kaplan-Meier (K-M) method and the Cox proportional hazards methods to analyze death rates and search for predictors of death for women and men for each CMP. Results We studied 12,015 patients, mean of 63.8±14.3 years, 6637 (55%) males. For all patients, death occurred in 27.5%, 15.3%, 17.5%, 40.1%, and 24.4%, respectively, for ischemic, idiopathic, hypertensive, Chagas, and valve CMPs (p<0.0001). HFrEF was more prevalent in idiopathic (51.2%) and Chagas (49.9%), and for HFpEF valve disease (80.4%), hypertensive (51.9%), and ischemic (44.4%). Compared to men, women were older, had a higher baseline mean left ventricular ejection fraction (LVEF), and a lower left ventricular diastolic diameter (LVDD) for all five types of CMP (p<0,001). Over a 3-years follow-up period, the cumulative incidence of death was higher in men with ischemic CMP (38% vs. 31%; p=0.037) and Chagas CMP (48% vs. 38%; p<0,001) but similar for idiopathic, hypertensive, and valve disease CMP (figure). Cox regression analysis for death, for each CMP, adjusted for confounders such as age, sex, previous myocardial infarction, diabetes, previous stroke, chronic kidney disease (CKD), atrial fibrillation (AF), any cardiac surgery, cardiac pacemakers, and LVEF showed that men were an independent predictor of death only for Chagas CMP (HR=1,28; 95% CI: 1.08–1.43; p=0.009). Conclusion Men were at a higher risk of death in ischemic and Chagas CMP but not for idiopathic, hypertensive, and valve CMP. The other main predictors of death were similar for all types of CMP, namely CKD, stroke, diabetes, and AF. Funding Acknowledgement Type of funding sources: None.

Generation of a heterozygous TPM1-E192K knock-in human induced pluripotent stem cell line using CRISPR/Cas9 system

E192K missense mutation of TPM1 has been found in different types of cardiomyopathies (e.g., hypertrophic cardiomyopathy, dilated cardiomyopathy, and left ventricular non-compaction), leading to systolic dysfunction, diastolic dysfunction, and/or tachyarrhythmias. Here, we generated a heterozygous TPM1-E192K knock-in human induced pluripotent stem cell (iPSC) line using CRISPR/Cas9-based genome editing system. The cells exhibit normal karyotype, typical stem cell morphology, expression of pluripotency markers and differentiation ability into three germ layers. Accordingly, this cell line could provide a useful cell resource for exploring the pathogenic role of TPM1-E192K mutation in different types of cardiomyopathies.

Is the Impact Breast Implants with RFID Have on MRI Quality Investigated Thoroughly Enough?

Is the Impact Breast Implants with RFID Have on MRI Quality Investigated Thoroughly Enough?

Identification of cardiomyopathy-related core genes through human metabolic networks and expression data

BackgroundCardiomyopathy is a complex type of myocardial disease, and its incidence has increased significantly in recent years. Dilated cardiomyopathy (DCM) and ischemic cardiomyopathy (ICM) are two common and indistinguishable types of cardiomyopathy.ResultsHere, a systematic multi-omics integration approach was proposed to identify cardiomyopathy-related core genes that could distinguish normal, DCM and ICM samples using cardiomyopathy expression profile data based on a human metabolic network. First, according to the differentially expressed genes between different states (DCM/ICM and normal, or DCM and ICM) of samples, three sets of initial modules were obtained from the human metabolic network. Two permutation tests were used to evaluate the significance of the Pearson correlation coefficient difference score of the initial modules, and three candidate modules were screened out. Then, a cardiomyopathy risk module that was significantly related to DCM and ICM was determined according to the significance of the module score based on Markov random field. Finally, based on the shortest path between cardiomyopathy known genes, 13 core genes related to cardiomyopathy were identified. These core genes were enriched in pathways and functions significantly related to cardiomyopathy and could distinguish between samples of different states.ConclusionThe identified core genes might serve as potential biomarkers of cardiomyopathy. This research will contribute to identifying potential biomarkers of cardiomyopathy and to distinguishing different types of cardiomyopathy.

Survival of patients with cardiomyopathies

Cardiomyopathies are a heterogeneous group of diseases. The main pathogenetic mechanism is myocardial damage due to genetic mutations. Cardiomyopathies are one of the leading causes of heart failure, sudden cardiac death, and life-threatening arrhythmias. Certain factors associated with poor prognosis determined the prognosis in this group of patients. Survival in different types of cardiomyopathies depends on the time of diagnosis and initial treatment. The types of cardiomyopathies discussed in this review are hypertrophic cardiomyopathy, dilative cardiomyopathy, restrictive cardiomyopathy, left ventricle non-compaction, and arrhythmogenic right ventricular cardiomyopathy.

Cardiomyopathy Management and In-Hospital Outcomes in a Tertiary Care Center: Clinical Components and Venues of Advanced Care.

BackgroundThere are few reports on the prevalence of different types of cardiomyopathy, clinical presentation, severity, short-term outcomes, and implementation of advanced heart failure treatment. This study aimed to assess the prevalence, clinical background of different types of cardiomyopathy and to identify the candidate for advanced treatment in a tertiary care cardiac center with many advantages MethodA single-center retrospective cohort study included 1069 patients admitted to our center and diagnosed with cardiomyopathy during 2019 and 2020 ResultsOut of 1069 cardiomyopathy patients admitted and diagnosed at our center between 2019 and 2020, 62% had ischemic cardiomyopathy (ICM), 36% had dilated cardiomyopathy (DCM), and 2% had hypertrophic cardiomyopathy (HOCM). ICM patients were older, showed a higher prevalence of both male gender and pilgrims, and they had more frequent cardiovascular risk factors compared to dilated cardiomyopathy group of patients. However, DCM patients with more severe heart failure symptoms (NYHA class III/IV), much worse LVEF, were subsequently considered deemed for aggressive diuretic therapy, and further advanced therapy (Sacubitril-Valsartan and device therapy) compared to ICM patients. ICM patients showed poor in-hospital outcomes compared to DCM group of patients (0.05 and <0.001) for an indication for mechanical ventilation and in-hospital mortality, respectively). Increased age, presence of renal dysfunction and lower LVEF were found the independent predictors of in-hospital mortality among our studied patients ConclusionThere are discrepancies between DCM and ICM patients. Although DCM patients were younger at age and had fewer cardiovascular risk factors, they presented with severe symptoms and dysfunction, hence more eligible candidates for advanced heart failure treatment, and finally showed a lower mortality rate. Increased age, presence of renal dysfunction and lower LVEF were found the independent predictors of in-hospital mortality.

Myocardial and Arrhythmic Spectrum of Neuromuscular Disorders in Children.

Neuromuscular disorders (NMDs) are highly heterogenous from both an etiological and clinical point of view. Their signs and symptoms are often multisystemic, with frequent cardiac involvement. In fact, childhood onset forms can predispose a person to various progressive cardiac abnormalities including cardiomyopathies (CMPs), valvulopathies, atrioventricular conduction defects (AVCD), supraventricular tachycardia (SVT) and ventricular arrhythmias (VA). In this review, we selected and described five specific NMDs: Friedreich’s Ataxia (FRDA), congenital and childhood forms of Myotonic Dystrophy type 1 (DM1), Kearns Sayre Syndrome (KSS), Ryanodine receptor type 1-related myopathies (RYR1-RM) and Laminopathies. These changes are widely investigated in adults but less researched in children. We focused on these specific topics due their relative frequency and their potential unexpected cardiac manifestations in children. Moreover these conditions present different inheritance patterns and mechanisms of action. We decided not to discuss Duchenne and Becker muscular dystrophies due to extensive work regarding the cardiac aspects in children. For each described NMD, we focused on the possible cardiac manifestations such as different types of CMPs (dilated-DCM, hypertrophic-HCM, restrictive-RCM or left ventricular non compaction-LVNC), structural heart abnormalities (including valvulopathies), and progressive heart rhythm changes (AVCD, SVT, VA). We describe the current management strategies for these conditions. We underline the importance, especially for children, of a serial multidisciplinary personalized approach and the need for periodic surveillance by a dedicated heart team. This is largely due to the fact that in children, the diagnosis of certain NMDs might be overlooked and the cardiac aspect can provide signs of their presence even prior to overt neurological diagnosis.

Could native T1 mapping replace late gadolinium enhancement in the assessment of myocardial fibrosis in patients with cardiomyopathy?

BackgroundCardiomyopathy is a myocardial disease, which usually demonstrates improper ventricular morphology, function, or both. It is classified into two classes based on the organ involved. Primary cardiomyopathy is confined mainly to the myocardium and can be genetic, non-genetic, or acquired. Secondary cardiomyopathy is caused by generalized systemic disorder. Myocardial fibrosis produces abnormal myocardial stiffness and increases arrhythmias risk. Native T1-mapping is an innovative technique that provides quantitative assessment of edema, diffuse myocardial fibrosis, and inflammation in a number of disease states. Furthermore native T1 mapping provides a future method for quantifying myocardial fibrosis in advanced chronic kidney disease and dialysis patients without the use of gadolinium-based contrast agents. So our aim is to assess the potential value of segmental quantification of myocardial fibrosis using native T1 mapping in different types of cardiomyopathy in comparison to late gadolinium enhancement (LGE) imaging.ResultsThe native T1 values of a total 1152 segments (16 segments in 72 patients of cardiomyopathy), 192 segments in 12 patients with hypertrophic cardiomyopathy (HCM), 800 segments in 50 patients with dilated cardiomyopathy (DCM), 80 segments in 5 patients with infiltrative cardiomyopathy, and 80 segments in 5 patients with non-compaction were assessed. These were compared with 160 segments of 10 healthy volunteers. Native T1 values were significantly higher in most of myocardial segments with LGE than in those without including the control group; non-contrast T1 values in mid LV septal segments were found the most significant (1130.85 ± 79.79 ms vs 1047.74 ± 42.74 ms; P = 0.001). Also the current study showed T1 values were significantly higher than normal even in segments unaffected by LGE (P<0.01) in both HCM and DCM groups. A receiver operating characteristic (ROC) analysis revealed the required cutoff value of 1070 ms for detecting myocardial fibrosis with a sensitivity 66% and specificity of 68%.ConclusionContrast-free T1-mapping is a new technique for detecting myocardial fibrosis objectively with a high diagnostic performance especially in patients who cannot afford gadolinium contrast agents as patients with end-stage renal disease.

Effect of Ischemic Cardiomyopathy on Perioperative Mortality After Endovascular Abdominal Aortic Aneurysm Repair

One in five patients undergoing elective endovascular abdominal aortic aneurysm repair (EVAR) will have left ventricular dysfunction. Yet, little is known about the perioperative risks of the different types of cardiomyopathy. We compared the outcomes of patients with ischemic cardiomyopathy (ICM) undergoing EVAR with those of patients with non-ICM (NICM).

The Prognostic Importance of Right Ventricular Longitudinal Strain in Patients with Cardiomyopathies, Connective Tissue Diseases, Coronary Artery Disease, and Congenital Heart Diseases.

Right ventricular (RV) systolic function represents an important independent predictor of adverse outcomes in many cardiovascular (CV) diseases. However, conventional parameters of RV systolic function (tricuspid annular plane excursion (TAPSE), RV myocardial performance index (MPI), and fractional area change (FAC)) are not always able to detect subtle changes in RV function. New evidence indicates a significantly higher predictive value of RV longitudinal strain (LS) over conventional parameters. RVLS showed higher sensitivity and specificity in the detection of RV dysfunction in the absence of RV dilatation, apparent wall motion abnormalities, and reduced global RV systolic function. Additionally, RVLS represents a significant and independent predictor of adverse outcomes in patients with dilated cardiomyopathy (CMP), hypertrophic CMP, arrhythmogenic RV CMP, and amyloidosis, but also in patients with connective tissue diseases and patients with coronary artery disease. Due to its availability, echocardiography remains the main imaging tool for RVLS assessment, but cardiac magnetic resonance (CMR) also represents an important additional imaging tool in RVLG assessment. The findings from the large studies support the routine evaluation of RVLS in the majority of CV patients, but this has still not been adopted in daily clinical practice. This clinical review aims to summarize the significance and predictive value of RVLS in patients with different types of cardiomyopathies, tissue connective diseases, and coronary artery disease.

Cough from a cardiologic perspective

Cough from a cardiologic perspective Abstract. A cough is at the efferent end of a complex reflex arc and, due to its well-known mechanical respiratory cleaning function, usually the first symptom prompting a pneumological clarification. However, the chemical and mechanical afferent neuronal parts of the reflex, the cough receptors, are distributed over a variety of organ systems, some of which directly and indirectly affect the heart. Cardiology therefore plays a central role in the clarification of coughs. In cardiology, a cough is most frequently caused by acute and chronic heart failure resulting from different types of cardiomyopathies. It can, however, be caused by other pathologies as well. The connection between cough and cardiac arrhythmia is interesting, although cough can be cause, consequence and therapy. Last but not least, almost all drugs frequently prescribed in cardiology can cause cough in one way or another. In addition, a cough is the current number 1 warning sign when it comes to COVID-19 infection. On the one hand, it must be differentiated from cardiac-induced coughs, but on the other hand it can also be closely related to them.

Evaluation of cardiomyopathy diagnosis in heart transplant recipients: comparison of echocardiographic and pathologic classification

BackgroundDefinite diagnosis of cardiomyopathy types can be challenging in end-stage disease process. New growing data have suggested that there is inconsistency between echocardiography and pathology in defining type of cardiomyopathy before and after heart transplantation. The aim of the present study was to compare the pre-heart transplant echocardiographic diagnosis of cardiomyopathy with the results of post-transplant pathologic diagnosis.ResultsIn this retrospective cross-sectional clinicopathological study, 100 consecutive patients have undergone heart transplantation in Masih-Daneshvari hospital, Tehran, Iran, between 2010 and 2019. The mean age of patients was 40 ± 13 years and 79% of patients were male. The frequency of different types of cardiomyopathy was significantly different between two diagnostic tools (echocardiography versus pathology, P < 0.001). On the other hand, in 24 patients, the results of echocardiography as regard to the type of cardiomyopathy were inconsistent with pathologic findings.ConclusionBased on the findings of the present study, it could be concluded that there is a significant difference between echocardiographic and pathologic diagnosis of cardiomyopathy; therefore, it is necessary to use additional tools for definite diagnosis of cardiomyopathy like advanced cardiac imaging or even endomyocardial biopsy before heart transplantation to reach an appropriate treatment strategy.

Childhood cardiomyopathies: a study in tertiary care hospital of Kashmir

Background: Cardiomyopathy constitutes a group of diseases that directly affect the structural or functional ability of myocardium. They are the most common form of heart diseases that are inherited in children and responsible for sudden deaths in healthy young adults. The Aim of our study was to provide a detailed description of clinical profile, epidemiology and etiology of cardiomyopathies in children.Methods: This was a hospital based prospective observational study, conducted over a period of two and half years from September 2017 to March 2020 in the Post Graduate Department of Pediatrics Government Medical College Srinagar. Study group included all patients aged between 1 month and 18 years diagnosed with cardiomyopathy. They were subjected to a detailed clinical history and physical examination. All the patients underwent echocardiography.Results: During the study period 37 patients were diagnosed with different types of cardiomyopathies. Dilated cardiomyopathy was commonest seen in 19 (51.35%), followed by hypertrophic cardiomyopathy 13 (35%) cases. Fast breathing was most common presenting complaint in dilated cardiomyopathy while most of cases with hypertrophic cardiomyopathy were asymptomatic. There was one case of distinct form of cardiomyopathy isolated left ventricular hypoplasia. Males were more involved 21 (56.76%) cases. Majority of patients 18 (48.64%) were less than one year at time of diagnosis. Parental consanguity was seen in 6 (16.22%) cases. Underlying cause was identified in 10 (27.02%) cases with myocarditis being the commonest cause.Conclusions: Pediatric cardiomyopathy represents a considerable percentage of children with cardiac disorders. Dilated cardiomyopathy is the most common type usually presented with congestive heart failure, majority of cases of hypertrophic CMP were asymptomatic. Male preponderance was seen in dilated and hypertrophic cardiomyopathy. Parental consanguinity was seen in significant number of patients. Myocarditis and inborn error of metabolism was seen in significant number of patients.