An irregular heart beat and fainting may occur. Dilated cardiomyopathy is the most common type of cardiomyopathy.Although most cases are idiopathic, a number of conditions (e.g., coronary artery disease, wet beriberi), infections … Emerging additional categories include arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and unspecified CM. Rapidly progressive HF and tachyarrhythmias, Comprise 10 - 20% of cases of biopsy positive myocarditis, Path on endomyocardial biopsy: extensive eosinophilic infiltration, A/w thyomomas, thyroiditis, pernicious anemia, other AID, Rapid deterioration, urgent transplantation. Cardiac output is decreased which activates the renin-angiotensin-aldosterone system, the sympathetic autonomic nervous system, and increase the release of the atrial natriuretic peptide. Int J Cardiol. Study by van Waning et al. diuretics) or decreased afterload (e.g. Unfortunately, because some people don’t experience any symptoms, the first sign can be sudden death. Often asymptomatic. Early on there may be few or no symptoms. RVR is poorly tolerated (decreased preload, decreased filling, increased myocardial O, Beta-blockers, Non-dihydropyridine-CCB (verapamil, diltiazem), Rate control may be necessary to sustain atrial kick - Amiodarone, Disopyramide, If refractory to medical therapy: AVN Ablation, Also, patient's have supranormal exercise capacity, mild ventricular dilation, and normal diastolic function, Mildly decreased contractility & EF (usually >30-50%). DOI:10.15347/wjm/2014.009. Cardiomyopathy is a group of diseases that affect the heart muscle. Kearns-Sayre syndrome)/Arrhythmogenic ventricular dysplasia/Hemochromatosis/associated with other systemic disease/Susceptibility to immune-mediate myocarditis, "Minimally dilated CM"/Hemochromatosis/Amyloidosis/Hypertrophic CM, Arrhythmogenic Ventricular (RV>LV) dysplasia, Hemochromatosis, Inherited metabolic defects (Fabry's, Gaucher's), Radiation (breast/lung CA, Mediastinal lymphoma), Possibly related fibrotic disease (Tropical endomyocardial fibrosis, Hypereosinophilic syndrome (Löffler's endocarditis)), Hypertrophic cardiomyopathy/"pseudohypertrophic", Encodes Myosin (thick filament 15nm), Actin (thin filament 7nm),Titin (elastic elements 1nm), associated regulatory proteins, Stabilizing and connecting the cell membrane to intracellular structures, Mutations impair transmission of force and signaling for cardiac & skeletal muscle → combined cardiac & skeletal, Defects impair energy production, causing symptoms ranging from impaired cognitive function to skeletal, Phenotype depends on the distribution of maternal mitochondria during embryonic development, Prior to HAART, HIV represented 1-2% of cases of dilated cardiomyopathy. Conduction disease lasting 1-2 weeks after Abx, MOA: secondary to Antibodies & Cytokines from prior physical injury & viral infection, Patients with Pulmonary Sarcoidosis at higher risk for cardiac involvement, Thought to have an infectious or environmental trigger, as regional clustering of cases. Etiologies varied, including Eosinophilic granulomatosis with polyangiitis (EGP, formerly Churg-Strauss), antecedent infection (Mediterranean & African countries), and malignancies. MOA: Perivascular infiltration → Myocarditis & heart failure with frequent arrhythmias. V122I transthyretin mutation is present in 10% of African Americans with heart failure and approximately ~4% of the African Americans population. This overlap in classification underscore the limitations of phenotypic classification system. Fatigue 5. Incidence <1%. This often improves with appropriately aggressive supportive care. inherited metabolic disorders, sarcoidosis, hemachromatosis, etc.) Jenni echocardiographic criteria most frequently used. Differentiating Cardiomyopathy from other Diseases, Natural History, Complications and Prognosis, Cardiomyopathy pathophysiology On the Web, American Roentgen Ray Society Images of Cardiomyopathy pathophysiology, Cardiomyopathy pathophysiology in the news, Directions to Hospitals Treating Cardiomyopathy, Risk calculators and risk factors for Cardiomyopathy pathophysiology, Editor-In-Chief: C. Michael Gibson, M.S., M.D. Same mutation in related individuals can produce different phenotype, Various patterns of pattern of hypertrophy concentric vs. asymmetric, presence or absence of outflow obstruction, development of Afib or malignant arrhythmia, SCD), A majority of the work on the genetics of cardiomyopathy was initially completed in this population, With advances in genetics, linkage analyses identified, >1400 mostly missense mutations in 9 different genes. Takotsubo's = older women after sudden intense emotional or physical stress + Global Ventricular dilatation with basal contraction, Early animal studies suggest MOA: Intense sympathetic activation with heterogeneity of myocardial autonomic innervation, diffuse microvascular spasm, &/or direct catecholamine toxicity, RF for CM: Dose dependent (occurring once cumulative life-time dose >500mg/m, Figure 5, below, depicts the MOA of Anthracycline toxicity. Inflammation resolves into areas of fibrosis that can serve as nidus for reentrant circuits. Symptoms and signs of PPCM are similar to those in patients with idiopathic dilated cardiomyopathy. Those affected are at an increased risk of sudden cardiac death. Table 1 below lists the various gene products implicated in cardiomyopathy. 5-FU, Cisplastin, & other Alkylating agents → coronary spasm → depressed contractility in some patients, anti-CTLA-4 monoclonal antibodies (Ipilimumab, the original checkpoint inhibitor. Sarcomeric genes identified associated with mutations causing DCM & HCM (e.g. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Therefore, the discussion below will focus on this classification. Atrial Arrhythmias, conduction disease, & CM : Formerly arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC), as first described in right ventricle. At present, HIV may interact with other viruses to produce "multiple-hits" to the myocardium and increase susceptibility to disease. 2 phases of eosinophilic myocardial disease: Cardiac inflammation replaced by fibrosis with superimposed thrombosis. Diphtheria releases a toxin that impairs protein synthesis & disrupts the conduction system. may cause more than one type of CM. WikiJournal of Medicine 1 (2). [2] Genetics will continue to play an increasing role in diagnosis and management of CM. Orthopnea, paroxysmal nocturnal dyspnea 4. Preservation of CO until late in the disease. [1] Genetic cause include HCM, ARVD/C, ion channel disorders, storage and infiltrative diseases. Fatigue 2. Infective and non-infective-related causes must be distinguished. The mutations in sarcomere genes (coding for actin, myosin, titin, etc.) nhlbi.nih.govCardiomyopathy refers to diseases of the heart muscle. Intestinal dysfcn (diarrhea, s/p resection), Most common reason for thyroid abnormalities in patient with cardiac dyscrasias are treatment of tachyarrhythmias with amiodarone. Chronic myocarditis is the oldest known cause of cardiomyopathy, described in literature as 'heart muscle disease,' as far back as the mid-1850s. Figure 4, below depicts the specific mechanism with which common viruses above lead to dilated CM. Dilated and Hypertrophic CM, which share symptoms of left heart failure, can be distinguished by a patient's ejection fraction (EF), left ventricular (LV) wall thickness, and LV end diastolic volume (LVEDV). X-linked. The genetic basis of hypertrophic cardiomyopathy (HCM) is well established. It usually starts in the left ventricle, where the heart muscle begins to dilate or stretch and become thinner. Dilated CM is the most common CM, comprising approximately 90% of all cardiomyopathies. The most common modes of transmission is Autosomal Dominant, followed by X-linked inheritance. In severe disease, the fibrotic layer can: extend to the AV valve leaflets causing fibrotic, thickened valvular apparatus, Unclear risk factors or triggers to mark the transition from hypereosinophilic syndrome to extensive fibrosis, Symptoms: CHF (Right>Left), Embolic events, Atrial arrhythmias, Only occurs with liver mets, as liver unable to metabolize serotonin → more serotonin released to venous circulation → Systemic Serotonin → fibrous plaques in the endocardium & heart valves (Right >>> Left), Associated symptoms of flushing & wheezing, Pseudohypertrophy refers to thickened myocardium secondary to infiltration of products between or within cells, Restrictive phenotype predominates, but may also concurrently have mildly dilated CM, Partial recovery from dilated cardiomyopathy, This page was last edited 13:26, 30 September 2019 by. For this reason, and with improving technology, the American Heart Association proposed a classification of CM emphasizing primary and secondary (to other systemic diseases) etiologies. Most known inherited genetic defects to date are associated with hypertrophic CM. The LV may or may not be dilated. Cardiomyopathy is a general term for diseases of the heart muscle, where the walls of the heart chambers have become stretched, thickened or stiff. This may include heart valves, mural endocardium or the endocardium that covers implanted material, such as prosthetic valves, pacemaker/defibrillator leads and catheters. RadcliffeCardiology article image Figure 2: Pathophysiology of Takotsubo Cardiomyopathy. Summary. Common cause of sudden cardiac death from malignant arrythmias (VTach, VFib). A disease primarily afflicting post-menopausal women, it is frequently mistaken for acute anterior wall myocardial infarction. Chest pain, primarily in patients with amyloidosis or due to angina 9. Palpitations This leads to enlargement of the inside of the ventricle.Source: U.S. Department of Health and Human Services, National Institutes of Health, National Heart Lung and Blood Institute. This process causes problems in the heart's electrical signaling, resulting in arrhythmias.Restrictive cardiomyopathyThe ventricles become stiff and rigid due to replacement of the normal heart muscle with abnormal tissue, such as scar tissue. 2010 European Society of Cardiology's listed below. Restrictive CM is characterized by the following 6 characteristics:[2][3]. Currently, treatment of CM is driven primarily by phenotype. [2] [3] Figure 11 below contrasts Restrictive CM with the two more common etiologies. On the individual sarcomere level, HCM mutations produce modification in regulatory proteins leading to: This changes on a microscopic level lead to disorganized hypertrophy, causing a cycle of: Only 15% derived from sarcomeric mutations, Fibrosis, disorganized hypertrophy, and microvascular disease contribute to, This occurs via drag forces push the anterior mitral leaflet in contact with hypertrophied ventricular septum, Compared to individuals without HCM, the anterior mitral leaflet is anteriorly displaced & thickened from fibrous endocardial plaque deposition), Presyncope & hypotension can result from decreased preload (e.g. Pathophysiology not fully characterized, but evolving. Cardiomyopathies are divided into 3 main types based on the pathologic features (see figure Forms of cardiomyopathy): Patients developing disease later in life have fare better than those with disease in adolescence/young adulthood. Cardiomyopathy: Gross apical slice of left and right ventricles concentric hypertrophy with cavitary obliteration sudden unexpected death obstructive cardiomyopathy, Dilated Cardiomyopathy: Gross natural color close-up view of heart surgically removed for a transplantation shows aortic valve and anterior leaflet of mitral valve with cholesterol deposits endocardium of left ventricle is diffusely thickened, Cardiomyopathy: Gross montage of ventricular slices showing hypertrophy and about normal ventricular lumen size a hypertrophic non-dilated cardiomyopathy, Cardiomyopathy: Gross ventricular slices hypertrophy and extensive myocardial fibrosis a unique case of global fiber disarray with atrophy and fibrosis, Cardiomyopathy: Gross close up view of a ventricle slice, Cardiomyopathy: Gross excellent ventricular slice with hypertrophy and fibrosis a unique case of global fiber disarray with hypertrophy then atrophy and then fibrosis, Cardiomyopathy: Gross external view of globular heart with patchy fibrosis seen through epicardium, Cardiomyopathy: Gross interventricular septum showing asymmetrical hypertrophy in posterior septum, Cardiomyopathy: Gross hypertrophic cardiomyopathy obstructive excellent section through left ventricle outlet to show subvalvular narrowing case of sudden death in a 27 yo male playing basketball no history of disease, Cardiomyopathy: Gross obstructive cardiomyopathy showing aorta outflow tract with marked endocardial thickening mitral valve appears normal (Same case as previous one), Cardiomyopathy: Gross excellent view of mitral valve atrial surface showing thickening which is fibrous in body of valve and myxoid at area of free margin changes presumed secondary to insufficiency due to anterior motion, Cardiomyopathy: Gross dilated left ventricle with marked endocardial thickening this is what has been called adult fibroelastosis, Dilated Cardiomyopathy: Gross good example huge dilated left ventricle, Dilated Cardiomyopathy: Gross dilated left ventricle with marked endocardial sclerosis (an excellent example), Cardiomyopathy: Gross intact globular shaped heart, Dilated Cardiomyopathy: Gross opened left ventricle dilated with endocardial thickening good example, Cardiomyopathy: Gross globular heart external view 10 year old girl with sickle cell anemia, Cardiomyopathy: Gross horizontal sections of ventricles dilation type 10 year old girl with sickle cell anemia, Cardiomyopathy: Intermediate between hypertrophic and dilated, Cardiomyopathy Asymmetrical Septal Hypertrophy, Dilated Cardiomyopathy: Gross opened globular left ventricle natural color (very good example), Diabetic Cardiomyopathy: Gross natural color moderately hypertrophied heart shown in horizontal section hyperemic subendocardium has no microscopic lesion long standing type I diabetic patient, no significant coronary artery lesions, congestive heart failure, Dilated Cardiomyopathy: Gross natural color external view globular heart 500 gm 24yo female seven pregnancies, Cardiomyopathy: Micro H&E high mag excellent example myofiber disarray, Cardiomyopathy: Micro H&E low mag interventricular septum at junction of normal myofiber orientation with asymmetrical hypertrophy (an excellent example), Cardiomyopathy: Micro trichrome low mag bizarre vacuolated fibers with disarray and focal fibrosis excellent low mag epicardial surface, Alcoholic Cardiomyopathy: Micro plastic section lipid in perinuclear area loss of myofibrils. Ischemic Cardiomyopathy. Arrhythmogenic right ventricular dysplasia (ARVD)ARVD develops when the muscle tissue in the right ventricle dies and is replaced with scar tissue. Pathophysiology Flow Chart ( Block Diagram) ... Edit this Diagram. Most common infective cause of cardiomyopathy, Third most common parasitic infection in the world, Named after the Brazilian physician, Dr. Carlos Chagas, who discovered the disease in 1909, Mechansim of action (MOA): Both direct & indirect mechanisms, Direct - Parasite → myocyte lysis & neuronal damage, Indirect - Chronic immune system activation, Same two clinical phases listed above for. arterial dilators), Afib is common in patients with HCM. Path on endomyocardial biopsy: infiltration with lymphocytes ad mononuclear cells with a high proportion of eosinophils. Small vessel spasm and ischemia → small, stiff heart with rEF without dilation, Concomitant Pulmonary HTN accentuates Right HF present in most restrictive diseases, Primarily a dilated CM with restrictive component, Fibrosis limits the degree of dilation possible, Most common cause of Endomyocardial fibrosis near the equator, May comprise up to 25% of CHF in these regions, End stage of prior hypereosinophilic disease triggered by endemic parasites, Fibrosis obliterating the ventricular apex, extending to valvular apparatus, like Löffler's endocarditis, Associated with pericardial effusions, unlike Löffler's endocarditis, Fibrosis obliterating the ventricular apex, extending to valvular apparatus : Tropical endomyocardial fibrosis, Most common cause of Endomyocardial fibrosis outside of equatorial regions, Hypereosinophilic syndrome associated with myeloproliferative disorders are often secondary to chromosomal rearrangements involving platelet-derived growth factor receptor (PDGFR), creating a fusion gene yielding a constitutively active PDGFR tyrosine kinase, Treatment with Imatinib (TKI) has produced hematologic remissions and reversal of endomyocarditis, HAN : most common (MC) identifiable cause of Eosinophilia :: CHINA : most common cause of (MCC) eosinophilia. Pathophysiology depicted below in Figure 7. Hep C is a major cause of myocarditis & DCM, particularly in endemic countries, RNA viruses: enterovirus, echovirus, polioviruses. Over the years, classification of this condition has been updated by the rapid advancement of genetic, imaging, and clinical investigation. 80% of HCM patients have a mutation in either Myosin-binding protein C (. Incidence varies across the world. Weight loss, cardiac cachexia 6. Detailed Causes of Restrictive CM[2][3][4][7], Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology, Cardiomyopathy: Gross excellent view of mitral valve from left atrium anterior leaflet appears to balloon a bit into the atrium. doi: 10.1016/j.ijcard.2011.09.077. Symptoms range from exercise intolerance and progressive heart failure to fatal arrhythmias and sudden cardiac death. Definition - imprecise due to varied criteria across many international societies. Subtypes listed below in order of decreasing severity. highlights the expanding role of genetics, Usually represent mutations on several domains on TTN gene (e.g. Dilated cardiomyopathy has many known and probably many unidentified causes (see table Causes of Dilated Cardiomyopathy). Cardiomyopathy is any structural and functional abnormality of the heart muscle unattributable to specific causes or disease processes such as coronary artery disease (CAD), congenital heart disease, or valvular disease. Symptoms may include the following: 1. Can cause fatal myocarditis, HF, heart block, myocardial fibrosis and cardiomyopathy. Respected digital resource of independent, expert-led cardiovascular education through the provision of a personalised learning experience In Central and South America, Chagas disease due to Trypanosoma cruzi is the most common infectious cause. These diseases have many causes, signs and symptoms, and treatments. Severity of clinical disease is usually commensurate with an increasing number of mutations. Caused by one of >160 mutations (Allelic heterogeneity), Lamellar inclusions of glycosphingolipids, Most are diagnosed early in childhood & die before adulthood, Fibrotic restrictive CM can coexist with constrictive pericarditis. Absence of another identifiable cause for the HF. often displays microinfarcts secondary to small vessel ischemia. Pathophysiology of HCM can be summarized in three aspects: secondary to early activation of profibrotic pathways, Interstitial fibrosis detectable before overt hypertrophy, Focal areas of 'replacement fibrosis' detectable on MRI before hypertrophy present, Myocardial fibroblast exaggerated response to primary myocardial dysfunction, Areas of scarring may represent substrate for malignant ventricular arrhythmias (MCC of death in this population), Over time, fibrosis → diastolic dysfunction, Microinfarction of hypertrophied myocardium proposed mechanism for replacement scar formation. Approximately 30% of Dilated CM (DCM) cases are familial. Cardiomyopathy refers to diseases of the heart muscle. Cardiomyopathy refers to diseases of the heart muscle. The clinical presentation of patients with cardiomyopathy can vary widely, depending on the underlying mechanism of disease. Missense mutations are most common among all forms of CM; nonsense and frameshift mutations also contribute to dysfunctional structural and cellular metabolism proteins (see Table 1 above) causing CM. Fewer granulomas associated with better prognosis. Template:Richard P, Charron P, Carrier L, et al. These diseases have a variety of causes, symptoms, and treatments. In cardiomyopathy, the heart muscle becomes enlarged or abnormally thick or rigid. In cardiomyopathy, the heart muscle becomes enlarged or abnormally thick or rigid. Pathophysiology of Dilated Cardiomyopathy. The importance of timely diagnosis is displayed below in Figure 6. 2012; 154:328–332. - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=2264027, Fabry's disease (alpha-galactosidace A deficiency), https://www.wikidoc.org/index.php?title=Cardiomyopathy_pathophysiology&oldid=1582960, Creative Commons Attribution/Share-Alike License, Viral/Parasitic/Bacterial/Fungal/Spirochetal/Ricketsial/Fungal, Granulomatous inflammatory diseases/Eosinophilic myocarditis/Hypersensitivity myocarditis/Polymyositis-Dermatomyositis/Collagen Vascular Disease/Pregnancy/Transplant Rejection, Most common etiology, comprising >10% of cases of DCM, Amphetamines, Cocaine, Pheochromocytoma, Stress-induced CM (Takotsubo's), Adramycins, Tyrosine Kinase Inhibitors, immune checkpoint inhibitors, Hydroxychloroquine, Chloroquine, Lithium, phenothiazine antipsychotics, antiretroviral therapies, lead; mercury (amalgam fillings, cinnabar); cobalt (tainted beer production), Skeletal & Cardiac myopathy/Dystrophin-related dystrophy (Duchenne's, Becker's) - X-linked/Mitochondrial myopathies (e.g. Alternatively called … In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. Heart failure with labile &/or orthostatic BP and episodic HTN. A cardiomyopathy is a primary disorder of the heart muscle. Cardiomyopathy can lead to heart failure.The main types of cardiomyopathy include dilated, hypertrophic and restrictive cardiomyopathy. Based on ratio between severely thickened myocardium with noncompacted layer ≥ 2x as thick as compacted layer (measured in short axis view), Very frequent nonsustained Ventricular tachycardia or High PVC burden, Prevalence 1:500 (~Hereditary hemochromatosis), Risk of Sudden Cardiac Death in patients with HCM is 0.5%, Transmitted in an Autosomal Dominant pattern, HCM is age dependent, with incomplete penetrance. Dyspnea on exertion, shortness of breath, cough 3. Time course varies from patient to patient. Studies investigating proangiogenic agents (VEGF with bromocriptine) as potential therapies. Cardiomyopathy Pathophysiology In 2006, the American Heart Association defined cardiomyopathies as: [2] "...a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. associated with Pericarditis, Vasculitis, Pulmonary hypertension, accelerated CAD. V122I gene mutation : Amyloidosis & Heart Failure :: BRCA1/2 gene mutation : Breast CA, 10% of people >80yo & 50% of people >90yo. Create healthcare diagrams like this example called Cardiomyopathy in minutes with SmartDraw. "Medical gallery of David Richfield". Highest in 1:100 in Zaria, Nigeria, due to the Hausa tribe's (predominantly located in Northern Nigeria) custom of eating kanwa, a dry lake salt, for forty days after delivery, around 1 in 1000 to 4000 live births in the United States, Mice lacking cardiac PGC-1α, a regulator of pro-angiogenic factors (e.g. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Cardiomyopathy is usually diagnosed with a chest X-ray, an echocardiogram (ultrasound of the heart), blood tests and a physical examination. Mutations in over 40 different genes have been described (locus heterogeneity). Crossref Medline Google Scholar; 11. Pathophysiology: Dilated cardiomyopathy occurs when the myocardium will dilate, thin, and undergo hypertrophy. Circulation 2003; 107:2227. The different etiologies of cardiomyopathy (CM), resulting in abnormal heart structure and function are myriad. Cyclophosphamide & Ifosfamide cardiotoxic in high doses. Various mutations within those genes have produced CM (allelic heterogeneity), and the same mutation can manifest differently within different family members (incomplete penetrance). Traztuzumab, Imatinib). Takotsubo cardiomyopathy (TC) is characterized by reversible ventricular dysfunction, not limited to the distribution of an epicardial coronary artery. Progressive exercise intolerance 3. In Central and South America, Chagas disease due to Trypanosoma cruzi is the most common infectious cause. Abdominal discomfort or liver tenderness 8. Cardiovascular disease is a common complication of diabetes responsible for 80% of … focal vasoconstriction in coronary artery in the setting of tachycardia, akin to ischemia-reperfusion with subsequent inflammation. While has been described now in both ventricles, RV much more frequently affected, Highly arrhythmogenic. Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders, which may lead to cardiovascular death or progressive heart failure-related disability." Involves interaction with genetics and environment (variable penetrance): Tafazzin gene encodes a protein expressed at high levels in cardiac & skeletal muscle. [3] [4] The many causes of Dilated CM all share the following phenotype: Enlarged heart, decreased systolic function. Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Paroxysmal nocturnal dyspnea 7. With the exception of dystrophinopathies, deletions are relatively rare. Coronary artery disease is the leading cause of death with overall mortality of approximately 245 per 100 000 individuals in 2008. A normal sarcomere is pictured above in Figure 1. ISSN 2002-4436. Increasing edema, weight, or abdominal girth On physical examination, look for signs of heart failure and volume overload Some etiologies (e.g. ß-hemolytic strept associated with Rheumatic Fever → inflammation & fibrosis of cardiac valves and systemic tissue. Others, include Thiazides, Anticonvulsants, Indomethacin, Methyldopa. tap diagram to zoom and pan Copy of New Pathophysiology of Pneumonia--You can edit this template and create your own diagram. Important etiologies of Dilated CM are listed below in Table 2. Duchenne or Becker muscular dystrophy, Danon disease), Dietary-induced fluid retention (e.g. LVEF <45%. As the disease worsens, shortness of breath, feeling tired, and swelling of the legs may occur, due to the onset of heart failure. 1. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Chronic Antibiotics, most common etiology. Abstract: Iron cardiomyopathy remains the leading cause of death in patients with thalassemia major. However, research is uncovering more and more genetic associations with dilated CM. This is caused by viral infections, toxins, connective tissue processes, or genetics. Hypertrophic cardiomyopathy occurs when the ventricle muscle thickens and this causes contraction of the heart to be stiff. The interactive pathophysiology of Restrictive CM is depicted below in Figure 12: Common causes of restrictive CM are categorized below. It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders. Rates of sarcomeric mutations exceed 60% in patients with familial disease & asymmetric septal hypertrophy. In this video the pathophysiology of restrictive, hypertrophic, and dilated cardiomyopathy is discussed. Independent fluid retention in obese people c impaired excretion (Rapid clearance of BNP by adipose tissue) → increase wall stress & secondary adaptive neurohormonal responsive. Gradually worsening shortness of breath 2. As a result, the ventricles cannot relax normally and expand to fill with blood, which causes the atria to become enlarged. Types of Cardiomyopathy Hypertrophic cardiomyopathyoccurs when the heart muscle thickens abnormally.Dilated cardiomyopathy affects the heart's ventricles and atria. Our knowledge of this disease entity has progressed significantly since the term was first used in 1957. In 2006, the American Heart Association defined cardiomyopathies as:[2], "...a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Mutation is present in 10 % of African Americans with heart failure ( HF ) diastolic... Hiv may interact with other viruses to produce `` multiple-hits '' to the myocardium and increase susceptibility to disease of... 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Create healthcare diagrams like this example called cardiomyopathy in minutes with SmartDraw of... That You can edit this template and create your own diagram inherited genetic defects to date are associated hypertrophic... America, Chagas disease due to Trypanosoma cruzi is the most common CM comprising... As coronary artery disease, valvular disorders, storage and infiltrative diseases, below the... Widely, depending on the underlying mechanism of disease for 80 % of HCM patients have a higher burden.. Exercise intolerance and progressive heart failure ( HF ) and diastolic dysfunction, comprising approximately 90 % of etiologies... [ 2 ] [ 3 ] BP and episodic HTN artery in the setting of,. Introduced in 2010 ), PD-L1 monoclonal antibodies ( eg, atezolizumab ) to symptoms. More genetic associations with dilated CM all share the following 6 characteristics [... It has been updated by the U.S. Federal Government as nidus for reentrant..: enlarged heart, decreased systolic function or stretch and become thinner transmitted undercooked! And arrhythmogenic right ventricular cardiomyopathy and this causes contraction of the endocardial surface of the muscle! Only one-third of all etiologies of dilated CM hemachromatosis, etc. of African population... Modify and make your own dilated CM enterovirus, echovirus, polioviruses responsibility of the endocardial of. Later in life have fare better than those with disease in adolescence/young adulthood close inpatient monitoring, decompensation. Acquired, and mixed causes and progressive heart failure ( HF ) and unspecified CM subdivided. An increased risk of sudden cardiac death, restrictive, hypertrophic and restrictive cardiomyopathy template and your. Lists the various gene products implicated in cardiomyopathy, the ventricles can not relax normally and to. Mitral and tricuspid valves from atria, appear normal anatomy characteristics: [ 2 ] [ 3 Figure... Timely diagnosis is displayed below in Figure 1 Dominant, pathophysiology of cardiomyopathy in flow chart by X-linked inheritance the heart.Types of.. Unidentified causes ( see table causes of dilated cardiomyopathy occurs when the heart muscle that affect the muscle!

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