title: Cardiac interstitium in health and disease: the fibrillar collagen network.
abstract: Composed of type I and III collagens, the valve leaflets, chordae tendineae and collagen matrix of the myocardium form a structural continuum. Synthesized by cardiac fibroblasts, these fibrillar collagens support and tether myocytes to maintain their alignment, whereas their respective tensile strength and resilience resist the deformation, maintain the shape and thickness, prevent the rupture and contribute to the passive and active stiffness of the myocardium. An acquired or congenital defect in this collagen network can lead to abnormalities in myocardial architecture, mechanics or valve function. In the hypertrophic process that accompanies a pressure overload, for example, increased collagen synthesis, fibroblast proliferation and a structural and biochemical remodeling of the matrix are seen. This includes distinctive patterns of reparative and reactive myocardial fibrosis, each of which alters diastolic and systolic myocardial stiffness and may lead to pathologic hypertrophy. Alternatively, a loss of collagen tethers or decline in matrix tensile strength can be responsible for regional or global transformations in myocardial architecture and function seen in the reperfused ("stunned") myocardium and in dilated (idiopathic) cardiopathy. Inherited disorders in the transcriptional and posttranslational processing of collagen can also alter the biophysical properties of the network. Future studies into collagen gene regulation, gene switching events and the control of collagen synthesis and degradation are needed to develop a more complete understanding of the relation between the collagen network and acquired and inherited forms of heart disease and to utilize therapeutics that will prevent, retard or regress abnormal collagen matrix remodeling.
authors.affiliation: Cardiovascular Institute, Michael Reese Hospital, University of Chicago Pritzker School of Medicine, Illinois 60616.
authors.forename: K T
authors.initials: KT
authors.lastname: Weber
mesh_terms: D002815:Chordae Tendineae / Q000033:anatomy & histology*; D003094:Collagen; D006321:Heart / Q000033:anatomy & histology*; D006331:Heart Diseases / Q000473:pathology*; D006351:Heart Valves / Q000033:anatomy & histology*; D006801:Humans; D008855:Microscopy, Electron, Scanning; D009206:Myocardium / Q000473:pathology*
chemical_list: D003094:Collagen