+ Translate

Arrhythmogenic inherited heart muscle diseases in children

, : Arrhythmogenic inherited heart muscle diseases in children. Journal of Electrocardiology 34 Suppl: 151-165

The left ventricle (LV) plays a central role in the maintenance of health of children and adults due to its role as the major pump of the heart. In cases of LV dysfunction, a significant percentage of affected individuals develop signs and symptoms of congestive heart failure, leading to the need for therapeutic intervention. Therapy for these patients include anticongestive medications and, in some, placement of devices such as aortic balloon pump or left ventricular assist device, or cardiac transplantation. In the majority of patients the origin is unknown, leading to the term idiopathic dilated cardiomyopathy. During the past decade, the basis of LV dysfunction has begun to unravel. In approximately 30% to 40% of cases, the disorder is inherited; autosomal dominant inheritance is most common (although X-linked, autosomal recessive and mitochondrial inheritance occurs). In the remaining patients, the disorder is presumed to be acquired, with inflammatory heart disease playing an important role. In the case of familial dilated cardiomyopathy, the genetic basis is beginning to unfold. To date, 2 genes for X-linked familial dilated cardiomyopathy (dystrophin, G4.5) have been identified and 4 genes for the autosomal dominant form (actin, desmin, lamin A/C, delta-sarcoglycan) have been described. In 1 form of inflammatory heart disease, coxsackievirus myocarditis, inflammatory mediators, and dystrophin cleavage play a role in the development of LV dysfunction. This review describes the molecular genetics of LV dysfunction and provide evidence for a "final common pathway" responsible for the phenotype.

(PDF 0-2 workdays service)

Order PDF Full Text


Click here to order any other PDF Full Text

Accession: 045301886

PMID: 11781950

PDF Full Text: Submit

Submit PDF Full Text

No spam - Every submission is manually reviewed

Due to poor quality, we do not accept files from Researchgate

Submitted PDF Full Texts will always be free for everyone
(We only charge for PDFs that we need to acquire)

Select a PDF file:

Other references

W. D. Ehmann, 1997: From a rock to a hard place; journeys of a radiochemist through inner and outer space. Journal of Radioanalytical and Nuclear Chemistry 219(2): 157-163

Kretovich, Vl; Karyakina, Ti; Kazakova, Ov; Sidel'-Nikova, Li, 1983: Biosynthesis of amino acids from metabolites of the Krebs cycle by Rhizobium lupini bacteroids. Doklady: biochemistry Akademiia nauk SSSR (pub 1983) 266(1-6): 282-285

Gomes, C.A.R.V.; Vaz, S.H.; Ribeiro, J.A.; Sebastião, A.M., 2006: Glial cell line-derived neurotrophic factor (GDNF) enhances dopamine release from striatal nerve endings in an adenosine A2A receptor-dependent manner. Both glial cell line-derived neurotrophic factor (GDNF) and adenosine influence dopaminergic function in the striatum. We now evaluated the GDNF effect on dopamine release from rat striatal nerve endings and if this effect of GDNF is modulated by...

Yamauchi, F; Sato, M; Yamazaki, A; Takagi, J., 1975: The effect of screening by chem. Hokkaido Nogyo Shikenjo kenkyu hokoku Research bulletin of the National Agricultural Experiment Station: 111 1-10

Kuroda, M.; Maruyama, K., 1976: Gamma-Actinin, a new regulatory protein from rabbit skeletal muscle. II. Action on actin. The interaction of gamma-actinin and actin was investigated under various conditions. It has been shown that gamma-actinin affects the G-F transformation of actin, causing an increase in the number of actin monomers required to form a nucleus in t...

Bandura, Vi, 1987: The commercial base of Tsimlyansk Reservoir, the current state of raw resources and ways of optimizing trade.. Gosudarstvennyii Nauchno Issledovatel'skii Institut Ozernogo i Rechnogo Rybnogo Khozyaistva Sbornik Nauchnykh Trudov: 4-22 (270)

Ek, M; Lennholm, H; Lindblad, G; Iversen, T; Gray, Dg, 1993: Photochromic behavior of UV-irradiated mechanical pulps. The degree of light-induced photoyellowing measured on mechanical pulps depends to some extent on the time between the actual irradiation and the brightness measurement. For example, if a sheet is kept for a certain time after accelerated irradiat...

Turkula, L.D.; Jackson, I.T., 1990: Functional support for flap reconstruction of the lower lip and mandible. A case of functional support for distant flap reconstruction of the entire lower lip and mandibular symphysis following resection of an aggressive recurrent basal cell carcinoma of the lip is presented. Resection of the entire lower lip and mandib...

Greenwood, B., 1973: The mitosis of sheep blood monocytes in tissue culture. Quarterly Journal of Experimental Physiology and Cognate Medical Sciences 58(4): 369-377

Chen, J-Xin.; Lin, P.; Fan, W.; Wu, Q-Liang.; Xiao, P.; Wang, J-Ye.; Zhang, X.; Li, X-Dong.; Xie, M-Ran., 2007: Internal mammary node biopsy in breast cancer. Ai Zheng 26(2): 172-177