Ventricular tachycardia and sudden death in myotonic dystrophy

Hiromasa, Shuichi; Ikeda, Takayuki; Kubota, K; Hattori, Nobu; Coto, Humberto; Maldonado, Claudio; Kupersmith, Joel

doi:10.1016/0002-8703(88)90901-5

Cited by 62 publications

(24 citation statements)

References 6 publications

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“…SCD is a major cause of mortality (Hiromasa et al, 1988;Palakurthy et al, 1989). Approximately 65% of SCD cases occur in patients with underlying acute or chronic heart disease.…”

Section: Introductionmentioning

confidence: 99%

Arrhythmia and neuronal/endothelial myocyte uncoupling in hyperhomocysteinemia

Rosenberger

Moshal

Kartha

et al. 2006

Archives of Physiology and Biochemistry

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Elevated levels of homocysteine (Hcy) known as hyperhomocysteinemia (HHcy) are associated with arrhythmogenesis and sudden cardiac death (SCD). Hcy decreases constitutive neuronal and endothelial nitric oxide (NO), and cardiac diastolic relaxation. Hcy increases the iNOS/NO, peroxynitrite, mitochondrial NADPH oxidase, and suppresses superoxide dismutase (SOD) and redoxins. Hcy activates matrix metalloproteinase (MMP), disrupts connexin-43 and increases collagen/elastin ratio. The disruption of connexin-43 and accumulation of collagen (fibrosis) disrupt the normal pattern of cardiac conduction and attenuate NO transport from endothelium to myocyte (E-M) causing E-M uncoupling, leading to a pro-arrhythmic environment. The goal of this review is to elaborate the mechanism of Hcy-mediated iNOS/NO in E-M uncoupling and SCD. It is known that Hcy creates arrhythmogenic substrates (i.e. increase in collagen/elastin ratio and disruption in connexin-43) and exacerbates heart failure during chronic volume overload. Also, Hcy behaves as an agonist to N-methyl-D-aspartate (NMDA, an excitatory neurotransmitter) receptor-1, and blockade of NMDA-R1 reduces the increase in heart rate-evoked by NMDAanalog and reduces SCD. This review suggest that Hcy increases iNOS/NO, superoxide, metalloproteinase activity, and disrupts connexin-43, exacerbates endothelial-myocyte uncoupling and cardiac failure secondary to inducing NMDA-R1.

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“…SCD is a major cause of mortality (Hiromasa et al, 1988;Palakurthy et al, 1989). Approximately 65% of SCD cases occur in patients with underlying acute or chronic heart disease.…”

Section: Introductionmentioning

confidence: 99%

Arrhythmia and neuronal/endothelial myocyte uncoupling in hyperhomocysteinemia

Rosenberger

Moshal

Kartha

et al. 2006

Archives of Physiology and Biochemistry

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“…Most cardiac events consist of impairment of the cardiac conduction system including bundle branch block or atrioventricular block. These are few reports of ventricular tachycardia (VT), whereas ventricular arrhythmias play a major role in the mortality of these patients (9)(10)(11)(12)(13). Unfortunately, pharmacological therapies have not significantly improved the prognosis (14), possibly due to the massive fatty fibrosis in the cardiac muscle.…”

Section: Introductionmentioning

confidence: 99%

Re-entry Circuit in Ventricular Tachycardia Due to Focal Fatty-fibrosis in a Patient with Myotonic Dystrophy

Muraoka¹,

Negoro²,

Terasaki

et al. 2005

Intern. Med.

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“…1970). Hiromasa et al (1988) établit quant à lui à 30% la fréquence des décès de ces personnes par mort subite; Floreck et al (1990) établit cette fréquence entre 0 et 4%; Nguyen et al (1988) dans une étude portant sur 19 personnes décédées établissent cette fréquence à 33% et font mention d'une autre étude de 30 cas autopsiés où 17% des décès étaient attribuables à la mort subite. Dans la maladie, le pronostic vital est relié à l'atteinte cardiaque (Maloisel et al 1988).…”

Section: Causes De Décèsunclassified

Étude descriptive de la mortalité dans la dystrophie myotonique au Saguenay-Lac-Saint-Jean /

Potvin¹

1994

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Ventricular tachycardia and sudden death in myotonic dystrophy

Cited by 62 publications

References 6 publications

Arrhythmia and neuronal/endothelial myocyte uncoupling in hyperhomocysteinemia

Arrhythmia and neuronal/endothelial myocyte uncoupling in hyperhomocysteinemia

Re-entry Circuit in Ventricular Tachycardia Due to Focal Fatty-fibrosis in a Patient with Myotonic Dystrophy

Étude descriptive de la mortalité dans la dystrophie myotonique au Saguenay-Lac-Saint-Jean /

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