Experimental models of restrictive cardiomyopathy

Abstract

Restrictive cardiomyopathy (RCMP) is characterized by the isolated diastolic ventricular dysfunction due to increased myocardial stiffness. Animal models of RCMP include the models of acquired and hereditary RCMP as well as the models in large animals. Acquired RCMP is observed in exogenous iron overload, radiation-induced myocardial fibrosis, eosinophilic myocarditis, systemic sclerosis, and amyloidosis. Genetic models of RCMP mimic clinical scenarios of hereditary hemochromatosis and sarcomeric protein mutations. A new approach to modeling RCMP is genetic modification of fruit flies. There are several critical characteristics of successful animal model of RCMP: 1) severe increase in left ventricular end-diastolic pressure in response to volume load; 2) increased ventricular stiffness in the in vitro settings; 3) biatrial enlargement; 4) lack of left ventricular dilation and advanced myocardial hypertrophy.