Cardiomyopathies–From Basic Research To Clinical Management

Classification and Definitions of Cardiomyopathies (Classification, Evaluation and Management of Cardiomyopathies) Part 1

Introduction Cardiomyopathies are an important and heterogeneous group of diseases. The awareness and knowledge of these diseases in both the public and medical communities historically has been impaired by persistent confusion surrounding definitions and nomenclature. Classification schemes, of which there have been many, (Thiene et al., 2000, 2004; Richardson et al., 1996) are potentially useful […]

Classification and Definitions of Cardiomyopathies (Classification, Evaluation and Management of Cardiomyopathies) Part 2

Primary cardiomyopathies Genetic Hypertrophic cardiomyopathy HCM is a condition of the heart in which a part of the myocardium or the muscle of the heart is enlarged without any obvious reasons. It is very common and affects people of all ages. HCM is a clinically heterogeneous but relatively common autosomal dominant genetic heart disease (1:500 […]

Management of Hypertrophic Obstructive Cardiomyopathy with a Focus on Alcohol Septal Ablation (Classification, Evaluation and Management of Cardiomyopathies) Part 1

Introduction Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease with unique pathophysiological characteristics and a great diversity of morphological, functional, and clinical features. HCM is defined as primary myocardial hypertrophy in the absence of aortic valve disease or significant hypertension (Elliott, 2008) Fig. 1. Magnetic resonance imaging; hypertrophic left and right ventricle. Several observations suggest […]

Management of Hypertrophic Obstructive Cardiomyopathy with a Focus on Alcohol Septal Ablation (Classification, Evaluation and Management of Cardiomyopathies) Part 2

Complications In-hospital death, the most significant complication of ASA, is rare. Nevertheless, it ranges in the literature from 1 to 4%. However, some observations suggest that in skilled hands, the mortality rate is close to zero. Historically, the incidence of complete heart block following ASA has ranged from 0 to 40% with a mean value […]

Hypertrophic Cardiomyopathy in Infants and Children (Classification, Evaluation and Management of Cardiomyopathies) Part 1

Introduction The definition and classification of the cardiomyopathies has been traditionally a complex and quite variable subject. In 2006, the American Heart Association issued a scientific statement elaborated by a task force of experts that contemplated the important development of molecular genetics in recent years, to explain the etiology of the diseases of cardiac muscle, […]

Hypertrophic Cardiomyopathy in Infants and Children (Classification, Evaluation and Management of Cardiomyopathies) Part 2

Clinical findings Aside from the genetic and phenotypic heterogeneity already mentioned in hypertrophic cardiomyopathy, the age, form of presentation, and outcome, are also quite diverse. The age of the patient at presentation is a determinant of prognosis (Colan et al., 2007). Newborn and infants are more likely to be referred for congestive heart failure while […]

Hypertrophic Cardiomyopathy in Infants and Children (Classification, Evaluation and Management of Cardiomyopathies) Part 3

Echocardiography Echocardiography associated with color flow Doppler is the most effective test for the diagnosis of hypertrophic cardiomyopathy (B.J. Maron et al., 2003a). It allows detection of the disease, follow-up of progression, and risk stratification for sudden death (B.J. Maron et al., 1986, Ostman-Smith et al., 2005). The wall thickness echocardiographic criteria for the diagnosis […]

Hypertrophic Cardiomyopathy in Infants and Children (Classification, Evaluation and Management of Cardiomyopathies) Part 4

Computed tomography and magnetic resonance imaging Cardiac computed tomography and magnetic resonance imaging yield superior anatomic data than echocardiography since they allow better definition of the anterolateral wall and tip of the left ventricle and the right ventricle. However, these procedures are more costly and the former exposes the patient to radiation (M. Maron et […]

Hypertrophic Cardiomyopathy in Infants and Children (Classification, Evaluation and Management of Cardiomyopathies) Part 5

Neuromuscular disorders Friedreich’s ataxia is an autosomic recessive hereditary disorder with spinocerebellar degeneration and frequently associated with hypertrophic cardiomyopathy (Gottdiener et al., 1982). A mutation of the frataxin gene (FXN) alters the energy production through mitochondrial iron dysmetabolism resulting in mitochondrial damage producing muscle fiber fibrosis (Michael et al., 2006). The cardiomyopathy may precede the […]

Quality of Life in Dilated Cardiomyopathy with Refractory Chronic Heart Failure Undergoing Devices Implantation (Classification, Evaluation and Management of Cardiomyopathies)

Introduction Heart failure is the final stage of most of cardiac diseases. It is a complex syndrome in which the patients should have the following features: symptoms of heart failure, typically shortness of breath at rest or during exertion, and/or fatigue; signs of fluid retention such as pulmonary congestion or ankle swelling; and objective evidence […]

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