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Primary Cardiomyopathy

Disease Primary Myocardial


Presentation

  • We found no evidence of primary skeletal muscle involvement; however, neuropathic features and biopsy changes of denervation were present in both groups.[ncbi.nlm.nih.gov]
  • Reversible defects were present in three patients in group II, three in group III, and none in group I. Abnormal right ventricular thallium uptake was observed in 27% of patients in group I, 25% in group II, and 40% in group III (p NS).[ncbi.nlm.nih.gov]
  • Delayed enhancement may or may not be present in dilated cardiomyopathies, but when present it involves the subepicardium or mid-myocardium as opposed to the subendocardium.[med-ed.virginia.edu]
  • There is a familial association with hypertrophic cardiomyopathy, although the true incidence and full expression of the association is not well understood at present.[ncbi.nlm.nih.gov]
  • […] failure, as many as one third of patients with idiopathic RCM may present with thromboembolic complications.[emedicine.medscape.com]
Fatigue
  • Common symptoms of heart failure include shortness of breath, fatigue and swelling of the ankles, feet, legs, abdomen and veins in the neck.[heart.org]
  • You might have: shortness of breath fatigue light headedness or feeling faint heart palpitations swelling in the legs due to fluid retention chest pain In children, you might notice they find it hard to keep up with their friends and become breathless[healthdirect.gov.au]
  • Affected individuals may also experience excessive sweating, fatigue, wheezing, and paleness of the skin (pallor).[rarediseases.org]
  • […] death, Cardiomegaly Functional AV valve regurgitation S3 and/or S4 Exertional dyspnea, angina, syncope, sudden death Systolic murmur mitral regurgitation murmur, S4 Bifid carotid pulse with a brisk upstroke and rapid downstroke Exertional dyspnea and fatigue[msdmanuals.com]
  • Call your doctor or other health care provider when you notice: Weight gain of 2-3 pounds in one day Shortness of breath Increase in fatigue Swelling in your ankles or belly Faintness or dizziness Changes in pulse and blood pressure Pounding heart beat[columbiasurgery.org]
Congestive Heart Failure
  • heart failure. hypertrophic cardiomyopathy an increase in heart muscle weight, particularly of the left ventricle and often involving the interventricular septum; it may affect the flow of blood from an atrium into the ventricle or out from the ventricle[medical-dictionary.thefreedictionary.com]
  • Cardiac MRI Pathology Cardiomyopathies Dilated Cardiomyopathy Dilated Cardiomyopathy Dilated cardiomyopathies (DCM) are a common cause of congestive heart failure (CHF) characterized by fibrosis and a decreased number of myocytes.[med-ed.virginia.edu]
  • The recent advent of vasodilator therapy has provided additional help to the patient with refractory congestive heart failure. Hypertrophic cardiomyopathy appears to be much more frequently recognized since the advent of echocardiography.[ncbi.nlm.nih.gov]
  • heart failure (chf) with cardiomyopathy Congestive heart failure due to cardiomyopathy Primary cardiomyopathy Secondary cardiomyopathy Tachycardia induced cardiomyopathy Clinical Information A disease of the heart muscle or myocardium proper.[icd10data.com]
  • Complications : 1) Atrial fibrillation with mural thrombosis ; 2) Embolization ; 3) Infective endocarditis ; 4) Congestive heart failure. Arrhythmias represent the major cause of death in these patients causing sudden death.[histopathology-india.net]
Heart Disease
  • […] cardiomyopathy [ kahr″de-o-mi-op ah-the ] a general diagnostic term designating primary myocardial disease. alcoholic cardiomyopathy a congestive cardiomyopathy resulting in cardiac enlargement and low cardiac output occurring in chronic alcoholics ; the heart[medical-dictionary.thefreedictionary.com]
  • Understand Your Risk for Cardiomyopathy Symptoms and Diagnosis of Cardiomyopathy Prevention and Treatment of Cardiomyopathy Cardiomyopathy in Children Learn more: Family History and Heart Disease Alcohol and Heart Disease Cocaine, Marijuana and Other[heart.org]
  • Epidemiology In contrast to coronary heart disease having a higher incidence in the elderly, cardiomyopathies can occur at younger ages.[patient.info]
  • Cardiomyopathy, or heart muscle disease, is a type of progressive heart disease in which the heart is abnormally enlarged, thickened, and/or stiffened.[webmd.com]
Tachycardia
  • Takotsubo cardiomyopathy ) cardiomyopathy in peripartum and post-partum cardiomyopathy tachycardia-induced cardiomyopathy cardiomyopathy in infants of diabetic mothers secondary cardiomyopathies (these conditions can have multi-organ involvement) infiltrative[radiopaedia.org]
  • […] heart muscle) Cardiomyopathy associated with another disorder Cardiomyopathy, primary (heart condition) Congestive heart failure (chf) with cardiomyopathy Congestive heart failure due to cardiomyopathy Primary cardiomyopathy Secondary cardiomyopathy Tachycardia[icd10data.com]
  • Specific cardiac abnormalities - eg, chronic uncontrolled tachycardia, hypertension, ischaemic heart disease, valvular dysfunction or abnormalities of the pericardium.[patient.info]
  • In some affected individuals, no symptoms may be present (asymptomatic); in many people, cardiomyopathy is a progressive condition that may result in an impaired ability of the heart to pump blood; fatigue; heart block; irregular heartbeats (tachycardia[rarediseases.org]
Cardiomegaly
  • Pulmonary venous congestion No cardiomegaly No or mild cardiomegaly Hemodynamics Normal or high EDP, low EF, diffusely dilated hypokinetic ventricles AV valve regurgitation Low CO High EDP outflow subvalvular gradient mitral regurgitation Normal or low[msdmanuals.com]
Heart Murmur
  • Cardiomyopathy may lead to heart failure, blood clots, a heart murmur, and cardiac arrest. The patient may not exhibit any signs and symptoms in the early stages of cardiomyopathy.[fortherecordmag.com]
Systolic Murmur
  • murmur mitral regurgitation murmur, S4 Bifid carotid pulse with a brisk upstroke and rapid downstroke Exertional dyspnea and fatigue LV RV failure Functional AV valve regurgitation ECG Nonspecific ST- and T-wave abnormalities Q waves BBB LV hypertrophy[msdmanuals.com]
Purpura
  • […] subacute myelo-optico-neuropathy SMON 再生不良性貧血 aplastic anemia AA サルコイドーシス sarcoidosis - 筋萎縮性側索硬化症 amyotrophic lateral sclerosis ALS 全身性強皮症 Systemic Scleroderma SSc 皮膚筋炎 Dermatomyositis DM 多発性筋炎 polymyositis PM 特発性血小板減少性紫斑病 Idiopathic Thrombocytopenic Purpura[nanbyou-soudan.jp]
  • 血栓性血小板減少性紫斑病 Thrombotic thrombocytopenic purpura (TTP) 血栓性血小板減少性紫斑病 1 不要 有 HPS1966 HPS1967 HPS1968 HPS1969 HPS1970 HPS1971 65.[cell.brc.riken.jp]
Muscle Weakness
  • The clinical examination revealed peripheral neuropathies in six patients, but no evidence of muscle weakness or atrophy.[ncbi.nlm.nih.gov]

Workup

  • The diagnostic workup for CM is relatively invasive and not readily obtainable during pregnancy. In many cases, the etiology remains unknown despite comprehensive workups.[heartfailure.onlinejacc.org]
T Wave Inversion
  • Patients present with an abrupt onset of angina-like chest pain, and have diffuse T-wave inversion, sometimes preceded by ST-segment elevation, and mild cardiac enzyme elevation. 19 Originally described in Japan, the condition is reported in Caucasian[academic.oup.com]

Treatment

  • The Children's Hospital at Montefiore (CHAM) uses a multidisciplinary approach to provide expert care in the diagnosis and management of children with all forms of heart failure, focusing on the diagnosis and treatment of primary cardiomyopathies and[cham.org]
  • Treatment depends on the specific type and cause of cardiomyopathy (see table Diagnosis and Treatment of Cardiomyopathies ).[msdmanuals.com]
  • Some people will not need any treatment. Treatment of secondary cardiomyopathy may involve treating the underlying condition.[mydr.com.au]
  • [ edit ] Treatment may include suggestion of lifestyle changes to better manage the condition.[en.wikipedia.org]
  • Their survival primarily depends on the natural history and progression of the disease as well as the response to treatment with anticongestive drugs.[ncbi.nlm.nih.gov]

Prognosis

  • All three subgroups (Löffler's endocarditis, primary restrictive cardiomyopathy, and endomyocardial fibrosis) have a guarded prognosis.[ncbi.nlm.nih.gov]
  • However, the prognosis appears to be worse in children than in adults. Genetic cases show autosomal dominant inheritance with incomplete penetrance.[emedicine.medscape.com]
  • Hemodynamics Normal or high EDP, low EF, diffusely dilated hypokinetic ventricles AV valve regurgitation Low CO High EDP outflow subvalvular gradient mitral regurgitation Normal or low CO High EDP, dip and plateau diastolic LV pressure curve Normal or low CO Prognosis[msdmanuals.com]
  • Cardiac pathology secondary to these conditions is often reversible (9,10), with a substantially better long-term prognosis compared with primary CM.[heartfailure.onlinejacc.org]
  • Although some pathological findings differ, the clinical picture and prognosis of MDCM are very similar to those of typical DCM. 13 Peripartum cardiomyopathy (PPCM) is a form of DCM that presents with signs of cardiac failure during the last month of[academic.oup.com]

Etiology

  • Despite this, large studies have failed to identify the etiology in the majority of patients.[ncbi.nlm.nih.gov]
  • […] of abnormal substances, as may occur in amyloidosis, hemochromatosis, and other disorders. primary cardiomyopathy that in which the basic pathologic process involves the myocardium itself and not other cardiac structures; the condition is of unknown etiology[medical-dictionary.thefreedictionary.com]
  • […] damage; and 3) idiopathic if no etiology was established.[heartfailure.onlinejacc.org]
  • Cardiomyopathies may be classified as either primary or secondary, on the basis of etiology, or on the pathophysiology of the lesion: hypertrophic, dilated, or restrictive.[icd9data.com]

Epidemiology

  • Epidemiology In contrast to coronary heart disease having a higher incidence in the elderly, cardiomyopathies can occur at younger ages.[patient.info]
  • Accordingly, the purpose of this single-center cohort study was to assess the disease pattern and outcome of disorders in which the primary pathology is the fetal myocardium and to determine epidemiological and hemodynamic markers associated with adverse[heartfailure.onlinejacc.org]
  • […] cardiomyopathy, contact the Children’s Cardiomyopathy Foundation listed in the Resources section of this report or the National Institutes of Health (NIH) clinical trials web site: The Pediatric Cardiomyopathy Registry was established to describe the epidemiologic[rarediseases.org]
  • Association scientific statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology[academic.oup.com]
Sex distribution
Age distribution

Pathophysiology

  • Cardiomyopathies may be classified as either primary or secondary, on the basis of etiology, or on the pathophysiology of the lesion: hypertrophic, dilated, or restrictive.[icd9data.com]
  • On the grounds of their morphology and pathophysiology, primary or idiopathic cardiomyopathies may be classified into a number of disorders; namely, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy,[journals.lww.com]
  • Cardiomyopathies may be classified as either primary or secondary, on the basis of etiology, or on the pathophysiology of the lesion: hypertrophic, dilated, or restrictive. [ NCIT : C34830 ] Synonyms: extrinsic cardiomyopathy, [X]Cardiomyopathy in other[ebi.ac.uk]
  • Society and Federation of Cardiology (ISFC) Task Force on the Definition and Classification of the Cardiomyopathies expanded the classification to include all diseases affecting heart muscle and to take into consideration etiology as well as the dominant pathophysiology[proz.com]

Prevention

  • Is It Possible to Prevent Cardiomyopathy? Cardiomyopathy is a term that describes the end result of many diseases and illnesses.[emedicinehealth.com]
  • Prevention In many cases, cardiomyopathy cannot be prevented. However, there are some types of cardiomyopathy that can be avoided, such as those that are caused by excessive alcohol or drug use.[mydr.com.au]
  • Prevention In many cases, you can't prevent cardiomyopathy. Let your doctor know if you have a family history of the condition.[mayoclinic.org]
  • Management Treatment options are symptomatic and mainly directed towards treatment of heart failure and prevention of thromboembolism and sudden death.[patient.info]
  • Understand Your Risk for Cardiomyopathy Symptoms and Diagnosis of Cardiomyopathy Prevention and Treatment of Cardiomyopathy Cardiomyopathy in Children Learn more: Family History and Heart Disease Alcohol and Heart Disease Cocaine, Marijuana and Other[heart.org]

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