Changing concepts of endocardial fibroelastosis.

Endocardial fibroelastosis is not a disease but a reaction of the endocardium. I review the history of the term with emphasis on the gradual understanding of the many causes of this reaction. I include a comprehensive list of diseases or other cardiac stresses that authors have reported in association, and I try to explain the mechanism of the reaction.

The perspective of ventricular noncompaction as seen by a nonagenarian.

The persistence of so-called spongy myocardium was first reported in humans in the 1960s. Subsequently, a few reports described the condition, utilizing inconsistent facts and terminology. In 1990 the first report appeared using the term "noncompaction", and detailed its clinical implications.

Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry.

Background: Current information on the epidemiology and outcomes of hypertrophic cardiomyopathy (HCM) in children is limited by disease diversity and small case series.

Methods And Results: The Pediatric Cardiomyopathy Registry has collected prospective and retrospective data on children diagnosed with HCM since 1990. We identified the various causes of HCM in childhood and determined the relationship between outcomes, cause, and age at presentation.

Incidence, causes, and outcomes of dilated cardiomyopathy in children.

Context: Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and cause of cardiac transplantation in children. However, the epidemiology and clinical course of DCM in children are not well established.

Objective: To provide a detailed description of the incidence, causes, outcomes, and related risk factors for DCM in children.

Factors associated with establishing a causal diagnosis for children with cardiomyopathy.

Objective: The goal was to identify the clinical variables associated with establishing a cause of cardiomyopathy in children.

Methods: The Pediatric Cardiomyopathy Registry contains clinical and causal testing information for 916 children who were diagnosed as having cardiomyopathy in North America between 1990 and 1995. Children with a causal diagnosis were compared with those without with respect to several demographic, clinical, and causal testing variables.

Mutations in the muscle LIM protein and alpha-actinin-2 genes in dilated cardiomyopathy and endocardial fibroelastosis.

Dilated cardiomyopathy (DCM) is a major cause of morbidity and mortality. Two genes have been identified for the X-linked forms (dystrophin and tafazzin), while mutations in multiple genes cause autosomal dominant DCM. Muscle LIM protein (MLP) is a member of the cysteine-rich protein (CRP) family and has been implicated in both myogenesis and sarcomere assembly.

The incidence of pediatric cardiomyopathy in two regions of the United States.

Background: Population-based data on the incidence of pediatric cardiomyopathy are rare because of the lack of large, prospective studies.

Methods: Since 1996 the Pediatric Cardiomyopathy Registry sponsored by the National Heart, Lung, and Blood Institute has collected data on all children with newly diagnosed cardiomyopathy in New England and the Central Southwest region (Texas, Oklahoma, and Arkansas) of the United States. We report on all children in these regions who received this diagnosis between 1996 and 1999.