Familial and sporadic cases are both described, and in familial cases, a skeletal myopathy may also be present. Stephanie Acosta, MD. Restrictive Cardiomyopathy. Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls of the heart are rigid (but not thickened). Restrictive cardiomyopathy, or restrictive cardiac disease, is defined as abnormal diastolic function in association with relatively well-preserved systolic function (at least in the early stages of the disease). It is the least common of the three original subtypes of cardiomyopathy: hypertrophic, dilated, and restrictive.. Primary restrictive cardiomyopathy is a rare form of cardiomyopathy that is characterized by normal or decreased ventricular volumes with bi-atrial enlargement, normal left ventricular wall thickness with impaired ventricular filling with restrictive physiology, and normal (or near normal) systolic function. Restrictive cardiomyopathy is diagnosed based on medical history, physical exam, and diagnostic tests. 5 Restrictive Cardiomyopathy in Clinical Practice 2017 . Restrictive cardiomyopathy is a primary disease of the myocardium, definition of RCM is on the basis of anatomic, histological, and physiological criteria. Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy, and is characterized by diastolic dysfunction with restrictive ventricular filling with normal or near-normal systolic function and wall thickness. Apical ballooning, akinetic of the apex. City of Worcester, MA Mobile Strain Screening Program 4 . 88 year old initial 6 80 year old man with DOE and new onset AF . Echo Differentiation of Restrictive Cardiomyopathy and Constrictive Pericarditis 1. 74 year old man s/p TAVR Still has HF . Clinically, restrictive cardiomyopathy is difficult to distinguish from constrictive pericarditis, which is treatable. A small number of cases are related to a true primary idiopathic restrictive cardiomyopathy, which can present in either childhood or adulthood. Garcia JACC 2016 2 . Echokardiographie Asymmetrisch verdickte Wände, Flussbeschleunigung im Ausflusstrakt Mitralklappeninsuffizienz, SAM (systolic anterior movement) ... Restriktive Kardiomyopathie mit diastolischer Funktionsstörung Auch Koronarienbeteiligung möglich Systemerkrankung In addition to restrictive cardiomyopathy, there are dilated cardiomyopathy and hypertrophic cardiomyopathy (see also Overview of Cardiomyopathy). Cardiomyopathy: Recognition by Echo . Restrictive cardiomyopathy is the least common subtype of cardiomyopathy and is characterized by a marked decrease in ventricular compliance. Restriction vs Constriction Stiff Muscle Thick Skin 3. There are three main types of cardiomyopathy. Diagnostic work-up may include electrocardiogram, chest X-ray, echocardiogram, exercise stress test, cardiac catheterization, CT scan, MRI and radionuclide studies. Myocardial echo during and amyloidosis speckled; Abnormally low É in restrictive cardiomyopathy Tako-tsubo cardiomyopathy Takotsubo is named after the ceramic pots used to trap octopus in Japan. 1 RCM may be idiopathic or associated with other infiltrative diseases, such as amyloidosis, endomyocardial disease, sarcoidosis, iron deposition disease, and … Constrictive vs Restrictive Cardiomyopathy 25 Jan 2017 Koh Choong Hou Supervisor: A/P Ewe See Hooi 2. Cardiomyopathy refers to progressive impairment of the structure and function of the muscular walls of the heart chambers. Echocardiography is crucial for staging the degree of diastolic abnormalities. Namely the presence of abnormal LV diastolic filling associated with intracellular or interstitial … This gives the LV the octupus trap shape. Thus the heart is restricted from stretching and filling with blood properly.