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American Family Physician Nov 2017The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired)... (Review)
Review
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Dilated cardiomyopathy can be genetic or acquired and typically presents with classic symptoms of heart failure with reduced ejection fraction. Restrictive cardiomyopathy is much less common and often associated with systemic disease. Family physicians should be alert for acquired variants of cardiomyopathy, including peripartum and stress-induced cardiomyopathy, as well as rare variants, such as arrhythmogenic right ventricular dysplasia and left ventricular noncompaction. In addition to history and physical examination, diagnosis of cardiomyopathy includes electrocardiography and echocardiography testing. Treatment may include appropriately staged therapy for heart failure, appropriate activity restriction, evaluation for implantable cardioverter-defibrillator placement, and consideration of heart transplantation in refractory cases. Genetic testing of families is an emerging modality with some potential to augment traditional screening performed by family physicians.
Topics: Cardiomyopathies; Diagnosis, Differential; Heart; Humans; Risk Factors
PubMed: 29431384
DOI: No ID Found -
Heart (British Cardiac Society) Oct 2019Cardiomyopathy is a group of disorders in which the heart muscle is structurally and functionally abnormal in the absence of other diseases that could cause observed... (Review)
Review
Cardiomyopathy is a group of disorders in which the heart muscle is structurally and functionally abnormal in the absence of other diseases that could cause observed myocardial abnormality. The most common cardiomyopathies are hypertrophic and dilated cardiomyopathy. Rare types are arrhythmogenic right ventricular, restrictive, Takotsubo and left ventricular non-compaction cardiomyopathies. This review of cardiomyopathies in pregnancy shows that peripartum cardiomyopathy is the most common cardiomyopathy in pregnancy. Peripartum cardiomyopathy develops most frequently in the month before or after partum, whereas dilated cardiomyopathy often is known already or develops in the second trimester. Mortality in peripartum cardiomyopathy varies from <2% to 50%. Few reports on dilated cardiomyopathy and pregnancy exist, with only a limited number of patients. Ventricular arrhythmias, heart failure, stroke and death are found in 39%-60% of high-risk patients. However, patients with modest left ventricular dysfunction and good functional class tolerated pregnancy well. Previous studies on >700 pregnancies in 500 women with hypertrophic cardiomyopathy showed that prognosis was generally good, even though three deaths were reported in high-risk patients. Complications include different types of supraventricular and ventricular arrhythmias, heart failure and ischaemic stroke. Recent studies on 200 pregnancies in 100 women with arrhythmogenic right ventricular cardiomyopathy have reported symptoms, including heart failure in 18%-33% of pregnancies. Ventricular tachycardia was found in 0%-33% of patients and syncope in one patient. Information on rare cardiomyopathies is sparse and only presented in case reports. Close monitoring by multidisciplinary teams in referral centres that counsel patients before conception and follow them throughout gestation is recommended.
Topics: Cardiomyopathies; Female; Humans; Patient Care Management; Pregnancy; Pregnancy Complications, Cardiovascular; Prognosis; Puerperal Disorders
PubMed: 31308064
DOI: 10.1136/heartjnl-2018-313476 -
Journal of the American College of... Jan 2020Peripartum cardiomyopathy is a form of systolic heart failure affecting young women toward the end of pregnancy or in the months following delivery. Incidence is higher... (Review)
Review
Peripartum cardiomyopathy is a form of systolic heart failure affecting young women toward the end of pregnancy or in the months following delivery. Incidence is higher in African-American women and in women with older maternal age, hypertensive disorders of pregnancy, and multiple gestation pregnancies. Symptoms of heart failure mimic those of normal pregnancy, often resulting in a delay in diagnosis and preventable complications. Echocardiography showing decreased myocardial function is essential for the diagnosis. Medical management is similar to heart failure with reduced ejection fraction of other etiologies, but adjustments during pregnancy are necessary to ensure fetal safety. Variable outcomes include complete recovery, persistent heart failure, arrhythmias, thromboembolic events, and death. Subsequent pregnancy confers substantial risk of relapse and even death if there is incomplete myocardial recovery. Additional research about the etiology, optimal therapy including the use of bromocriptine, long-term outcomes, and duration of treatment after recovery are needed.
Topics: Cardiomyopathies; Cardiovascular Agents; Female; Humans; Peripartum Period; Pregnancy; Pregnancy Complications, Cardiovascular; Puerperal Disorders; Review Literature as Topic
PubMed: 31948651
DOI: 10.1016/j.jacc.2019.11.014 -
Current Cardiology Reports May 2020To briefly review epidemiology and pathophysiology of SICM and provide a more extensive review of the data on diagnostic and management strategies. (Review)
Review
PURPOSE OF REVIEW
To briefly review epidemiology and pathophysiology of SICM and provide a more extensive review of the data on diagnostic and management strategies.
RECENT FINDINGS
SICM is likely underdiagnosed and that has mortality implications. Current evidence supports speckle tracking echocardiography to identify decreased contractility irrespective of left ventricular ejection fraction for the diagnosis of SICM. There continues to be a dearth of large clinical trials evaluating the treatment of SICM and current consensus focuses on supportive measures such as vasopressors and inotropes. Sepsis is a significant cause of mortality, and sepsis-induced cardiomyopathy has both prognostic and management implications for these patients. Individualized work-up and management of these patients is crucial to improving outcomes.
Topics: Cardiomyopathies; Echocardiography; Heart; Humans; Sepsis; Stroke Volume; Ventricular Dysfunction, Left; Ventricular Function, Left
PubMed: 32377972
DOI: 10.1007/s11886-020-01277-2 -
Journal of Veterinary Internal Medicine May 2020Cardiomyopathies are a heterogeneous group of myocardial disorders of mostly unknown etiology, and they occur commonly in cats. In some cats, they are well-tolerated and...
Cardiomyopathies are a heterogeneous group of myocardial disorders of mostly unknown etiology, and they occur commonly in cats. In some cats, they are well-tolerated and are associated with normal life expectancy, but in other cats they can result in congestive heart failure, arterial thromboembolism or sudden death. Cardiomyopathy classification in cats can be challenging, and in this consensus statement we outline a classification system based on cardiac structure and function (phenotype). We also introduce a staging system for cardiomyopathy that includes subdivision of cats with subclinical cardiomyopathy into those at low risk of life-threatening complications and those at higher risk. Based on the available literature, we offer recommendations for the approach to diagnosis and staging of cardiomyopathies, as well as for management at each stage.
Topics: Animals; Cardiomyopathies; Cat Diseases; Cats; Consensus; Heart; Practice Guidelines as Topic; Societies, Veterinary
PubMed: 32243654
DOI: 10.1111/jvim.15745 -
Journal of the American College of... May 2019Arrhythmias coexist in patients with heart failure (HF) and left ventricular (LV) dysfunction. Tachycardias, atrial fibrillation, and premature ventricular contractions... (Review)
Review
Arrhythmias coexist in patients with heart failure (HF) and left ventricular (LV) dysfunction. Tachycardias, atrial fibrillation, and premature ventricular contractions are known to trigger a reversible dilated cardiomyopathy referred as arrhythmia-induced cardiomyopathy (AiCM). It remains unclear why some patients are more prone to develop AiCM despite similar arrhythmia burdens. The challenge is to determine whether arrhythmias are fully, partially, or at all responsible for an observed LV dysfunction. AiCM should be suspected in patients with mean heart rate >100 beats/min, atrial fibrillation, and/or premature ventricular contractions burden ≥10%. Reversal of cardiomyopathy by elimination of the arrhythmia confirms AiCM. Therapeutic choice depends on the culprit arrhythmia, patient comorbidities, and preferences. Following recovery of LV function, patients require continued follow-up if an abnormal myocardial substrate is present. Appropriate diagnosis and treatment of AiCM is likely to improve quality of life and clinical outcomes and to reduce hospital admission and health care spending.
Topics: Arrhythmias, Cardiac; Cardiomyopathies; Humans; Patient Care; Recovery of Function; Ventricular Dysfunction, Left
PubMed: 31072578
DOI: 10.1016/j.jacc.2019.02.045 -
International Journal of Molecular... Nov 2021With the development and advancement of next-generation sequencing (NGS), genetic analysis is becoming more accessible. High-throughput genetic studies using NGS have... (Review)
Review
With the development and advancement of next-generation sequencing (NGS), genetic analysis is becoming more accessible. High-throughput genetic studies using NGS have contributed to unraveling the association between cardiomyopathy and genetic background, as is the case with many other diseases. Rare variants have been shown to play major roles in the pathogenesis of cardiomyopathy, which was empirically recognized as a monogenic disease, and it has been elucidated that the clinical course of cardiomyopathy varies depending on the causative genes. These findings were not limited to dilated and hypertrophic cardiomyopathy; similar trends were reported one after another for peripartum cardiomyopathy (PPCM), cancer therapy-related cardiac dysfunction (CTRCD), and alcoholic cardiomyopathy (ACM). In addition, as the association between clinical phenotypes and the causative genes becomes clearer, progress is being made in elucidating the mechanisms and developing novel therapeutic agents. Recently, it has been suggested that not only rare variants but also common variants contribute to the development of cardiomyopathy. Cardiomyopathy and genetics are approaching a new era, which is summarized here in this overview.
Topics: Cardiomyopathies; Cardiomyopathy, Alcoholic; Cardiomyopathy, Dilated; Cardiomyopathy, Hypertrophic; Genetic Testing; High-Throughput Nucleotide Sequencing; Humans; Mutation; Phenotype
PubMed: 34830403
DOI: 10.3390/ijms222212522 -
International Journal of Molecular... Jul 2021Cardiomyopathies are a heterogeneous group of pathologies characterized by structural and functional alterations of the heart. (Review)
Review
BACKGROUND
Cardiomyopathies are a heterogeneous group of pathologies characterized by structural and functional alterations of the heart.
AIMS
The purpose of this narrative review is to focus on the most important cardiomyopathies and their epidemiology, diagnosis, and management.
METHODS
Clinical trials were identified by Pubmed until 30 March 2021. The search keywords were "cardiomyopathies, sudden cardiac arrest, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy, arrhythmogenic cardiomyopathy (ARCV), takotsubo syndrome".
RESULTS
Hypertrophic cardiomyopathy (HCM) is the most common primary cardiomyopathy, with a prevalence of 1:500 persons. Dilated cardiomyopathy (DCM) has a prevalence of 1:2500 and is the leading indication for heart transplantation. Restrictive cardiomyopathy (RCM) is the least common of the major cardiomyopathies, representing 2% to 5% of cases. Arrhythmogenic cardiomyopathy (ARCV) is a pathology characterized by the substitution of the myocardium by fibrofatty tissue. Takotsubo cardiomyopathy is defined as an abrupt onset of left ventricular dysfunction in response to severe emotional or physiologic stress.
CONCLUSION
In particular, it has been reported that HCM is the most important cause of sudden death on the athletic field in the United States. It is needless to say how important it is to know which changes in the heart due to physical activity are normal, and when they are pathological.
Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Cardiomyopathy, Hypertrophic; Death, Sudden, Cardiac; Humans; Myocardium
PubMed: 34299342
DOI: 10.3390/ijms22147722 -
Journal of Cardiac Failure Jan 2022The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in... (Review)
Review
The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in conduction disease, arrhythmia, and/or heart failure. The diagnosis of cardiac sarcoidosis (CS) can be challenging and requires clinician awareness as well as differentiation from overlapping diagnostic phenotypes, such as other forms of myocarditis and arrhythmogenic cardiomyopathy. Clinical manifestations, extracardiac involvement, histopathology, and advanced cardiac imaging can all lend support to a diagnosis of CS. The mainstay of therapy for CS is immunosuppression; however, no prospective clinical trials exist to guide management. Patients may progress to developing advanced heart failure or ventricular arrhythmia, for which ventricular assist device therapies or heart transplantation may be considered. The existing knowledge gaps in CS call for an interdisciplinary approach to both patient care and future investigation to improve mechanistic understanding and therapeutic strategies.
Topics: Cardiomyopathies; Heart Failure; Heart Transplantation; Humans; Myocarditis; Sarcoidosis
PubMed: 34260889
DOI: 10.1016/j.cardfail.2021.06.016 -
Texas Heart Institute Journal 2013Mitochondrial disease is a heterogeneous group of multisystemic diseases that develop consequent to mutations in nuclear or mitochondrial DNA. The prevalence of... (Review)
Review
Mitochondrial disease is a heterogeneous group of multisystemic diseases that develop consequent to mutations in nuclear or mitochondrial DNA. The prevalence of inherited mitochondrial disease has been estimated to be greater than 1 in 5,000 births; however, the diagnosis and treatment of this disease are not taught in most adult-cardiology curricula. Because mitochondrial diseases often occur as a syndrome with resultant multiorgan dysfunction, they might not immediately appear to be specific to the cardiovascular system. Mitochondrial cardiomyopathy can be described as a myocardial condition characterized by abnormal heart-muscle structure, function, or both, secondary to genetic defects involving the mitochondrial respiratory chain, in the absence of concomitant coronary artery disease, hypertension, valvular disease, or congenital heart disease. The typical cardiac manifestations of mitochondrial disease--hypertrophic and dilated cardiomyopathy, arrhythmias, left ventricular myocardial noncompaction, and heart failure--can worsen acutely during a metabolic crisis. The optimal management of mitochondrial disease necessitates the involvement of a multidisciplinary team, careful evaluations of patients, and the anticipation of iatrogenic and noniatrogenic complications. In this review, we describe the complex pathophysiology of mitochondrial disease and its clinical features. We focus on current practice in the diagnosis and treatment of patients with mitochondrial cardiomyopathy, including optimal therapeutic management and long-term monitoring. We hope that this information will serve as a guide for practicing cardiologists who treat patients thus affected.
Topics: Animals; Cardiomyopathies; DNA, Mitochondrial; Energy Metabolism; Genetic Predisposition to Disease; Humans; Mitochondria, Heart; Mitochondrial Diseases; Phenotype; Predictive Value of Tests; Risk Factors; Treatment Outcome
PubMed: 24082366
DOI: No ID Found