Neglected tropical cardiomyopathies: I. Chagas disease

Yacoub, Sophie; Mocumbi, Ana Olga; Yacoub, Magdi H.

doi:10.1136/hrt.2007.132316

Cited by 35 publications

(55 citation statements)

References 22 publications

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“…The primary target organ for Chagas disease and for some CDGs is the heart, leading to an increased susceptibility to conduction anomalies, cardiac arrhythmias, and heart failure (16)(17)(18)(19). Thus, these diseases lead to aberrant cardiac glycosylation and to symptomatic changes in cardiac excitability.…”

mentioning

confidence: 99%

Regulated and aberrant glycosylation modulate cardiac electrical signaling

Montpetit

Stocker

Schwetz

et al. 2009

Proc. Natl. Acad. Sci. U.S.A.

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Millions afflicted with Chagas disease and other disorders of aberrant glycosylation suffer symptoms consistent with altered electrical signaling such as arrhythmias, decreased neuronal conduction velocity, and hyporeflexia. Cardiac, neuronal, and muscle electrical signaling is controlled and modulated by changes in voltage-gated ion channel activity that occur through physiological and pathological processes such as development, epilepsy, and cardiomyopathy. Glycans attached to ion channels alter channel activity through isoform-specific mechanisms. Here we show that regulated and aberrant glycosylation modulate cardiac ion channel activity and electrical signaling through a cell-specific mechanism. Data show that nearly half of 239 glycosylation-associated genes (glycogenes) were significantly differentially expressed among neonatal and adult atrial and ventricular myocytes. The N-glycan structures produced among cardiomyocyte types were markedly variable. Thus, the cardiac glycome, defined as the complete set of glycan structures produced in the heart, is remodeled. One glycogene, ST8sia2, a polysialyltransferase, is expressed only in the neonatal atrium. Cardiomyocyte electrical signaling was compared in control and ST8sia2 (؊/؊) neonatal atrial and ventricular myocytes. Action potential waveforms and gating of less sialylated voltage-gated Na ؉ channels were altered consistently in ST8sia2 (؊/؊) atrial myocytes. ST8sia2 expression had no effect on ventricular myocyte excitability. Thus, the regulated (between atrium and ventricle) and aberrant (knockout in the neonatal atrium) expression of a single glycogene was sufficient to modulate cardiomyocyte excitability. A mechanism is described by which cardiac function is controlled and modulated through physiological and pathological processes that involve regulated and aberrant glycosylation. action potentials ͉ cardiomyocyte ͉ glycomics ͉ ion channels ͉ sialic acids

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mentioning

confidence: 99%

Regulated and aberrant glycosylation modulate cardiac electrical signaling

Montpetit

Stocker

Schwetz

et al. 2009

Proc. Natl. Acad. Sci. U.S.A.

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“…The surgical mortality varies between 15% and 30%, 3 particularly when ex- tensive endocardial resection or tricuspid valve prostheses are needed. The rate of relapse after surgical correction is not clear.…”

Section: Management and Prognosismentioning

confidence: 99%

“…In severe right EMF, the electrocardiogram shows a tall and broad right atrial wave and a characteristic ''QR'' pattern in V3R or V1. 3 In advanced disease, atrial fibrillation and ventricular ectopic beats are common.…”

Section: Electrocardiographymentioning

confidence: 99%

“…1 It is the most prevalent form of restrictive cardiomyopathy worldwide, showing marked variation in geographic distribution. [2][3][4] The hypotheses postulated to explain the etiology of EMF have not been proven and cannot explain its occurrence worldwide. They include cerium deficiency, magnesium deficiency, cassava, malnutrition, parasitic diseases, viral diseases, autoimmunity, allergy, and ethnicity.…”

mentioning

confidence: 99%

“…They include cerium deficiency, magnesium deficiency, cassava, malnutrition, parasitic diseases, viral diseases, autoimmunity, allergy, and ethnicity. 3 The right ventricle is the cardiac chamber most frequently affected in EMF, either in isolation or as part of bilateral disease. 5 This may reflect better clinical tolerance of right-heart forms of the disease, which allows the patients to reach health facilities.…”

mentioning

confidence: 99%

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Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

Mocumbi

2014

Pulm. circ.

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Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy of unknown origin, affecting predominantly the right side of the heart. Its highest prevalence is in poor regions of sub-Saharan Africa, where it is usually found in children and adolescents. In areas where it is endemic, EMF is a major cause of heart failure and premature death. EMF of the right ventricle has unique clinical, electrocardiographic, and echocardiographic signs. Several features of advanced disease are not fully understood, the most striking being the presence of severe ascites with little or no pedal edema. Echocardiography is the main diagnostic tool and supports management of the disease in most patients, as it allows assessment of the severity and extension of endocardial fibrosis, the quality of the right atrioventricular valve, and the presence of intracardiac thrombi. Pulmonary hypertension in the setting of right EMF is related to chronic thromboembolism, through the chronic thrombi present in the severely enlarged right atrium found in advanced disease. The management of right EMF is difficult. Treatment of symptomatic patients should be surgical. However, as the disease is usually detected in late stages, with severe and advanced lesions, surgery cannot be performed without a very high risk of death and complications. New surgical approaches addressing several components of the structural cardiac abnormalities have been attempted, with promising results. EMF remains one of the most neglected diseases worldwide, and research into its pathophysiological mechanisms will probably improve outcomes and alter the natural history of the disease. This requires improvement in the health systems in areas of endemicity as well as the design of international collaborative research projects.

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Trypanosoma Cruzi

Taylor

2009

Intracellular Niches of Microbes

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Neglected tropical cardiomyopathies: I. Chagas disease

Cited by 35 publications

References 22 publications

Regulated and aberrant glycosylation modulate cardiac electrical signaling

Regulated and aberrant glycosylation modulate cardiac electrical signaling

Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

Trypanosoma Cruzi

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