Dilated and restrictive cardiomyopathies cleveland clinic. Genetic spectrum of end stage idiopathic restrictive cardiomyopathy. Restrictive cardiomyopathies constitute a heterogenous group of heart muscle conditions that all have, in common, the symptoms of heart failure. Cmp primary disease intrinsic to cardiac muscle dilated cmp hypertrophic cmp infiltrative cmp left ventricular noncompaction arrythmogenic right ventricular cmp takotsubo cardiomyopathy. Tctmd is produced by the cardiovascular research foundation crf. Gallego delgado m, montserrat l, ruizcano mh, et al. Infiltrative cardiomyopathy definition of infiltrative. However, lowvoltage qrs complex is not a uniform finding with the infiltrative cardiomyopathies. Cureus a novel case of idiopathic restrictive cardiomyopathy. Dilated cardiomyopathy arising as primary myocardial disease of unknown etiology or as disorders of toxic, familial, or infective origin. Noninvasive imaging in cardiac deposition diseases shah.
The much more rare diagnosis of idiopathic restrictive cardiomyopathy is supported only by the absence of specific pathology on either endomyocardial biopsies or at postmortem. They usually portend an adverse prognosis, although in rare instances e. Rare case of infiltrative cardiomyopathy secondary to. Ppt cardiomyopathies powerpoint presentation free to view.
Infiltrative cardiomyopathy and pericardial disease. The topic infiltrative restrictive cardiomyopathy you are seeking is a synonym, or alternative name, or is closely related to the medical condition restrictive cardiomyopathy. Pdf cardiovascular magnetic resonance in hypertrophic. Hypertrophic cardiomyopathy is the second most common cause of sudden cardiac death in the. Chart and diagram slides for powerpoint beautifully designed chart and diagram s for powerpoint with visually stunning graphics and animation effects. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of. Echocardiographic manifestations of infiltrative cardiomyopathy. Echocardiography in infiltrative cardiomyopathy sciencedirect. The weakened heart muscle loses the ability to pump blood effectively, resulting in irregular heartbeats arrhythmias and possibly even heart failure. Hypertrophic cardiomyopathy is the second most common cause of sudden cardiac death in the adolescent population and the. Recent advances in the imaging assessment of infiltrative.
Restrictive cardiomyopathies are the least common form of heart muscle. Echocardiography has been useful in the evaluation of congestive and hypertrophic cardiomyopathies. View enhanced pdf access article on wiley online library. Restrictive cardiomyopathy american heart association.
Restrictive and infiltrative cardiomyopathies are the least frequently encountered form of primary heart muscle disease in adults within the developed world. Secondary and infiltrative cardiomyopathies springerlink. The following are summary points from part 1 of a 2part series on the spectrum of restrictive and infiltrative cardiomyopathies. Denise antle, arnp, msn, ccrn, ccns critical care arnpcns. Cardiomyopathy kardeomiopahthee refers to diseases of the heart muscle. Author links open overlay panel rebecca perry phd a b c joseph b. The combination of increased lv mass in the absence of high electrocardiograph ecg voltages may be more specific for cardiac infiltrative diseases, of which amyloidosis is the most common table 1. Infiltrative cardiomyopathies david bejar, paolo c. Amyloidosis is a systemic and progressive disease and frequently involves more than. Spectrum of restrictive and infiltrative cardiomyopathies jacc. Restrictive cardiomyopathies are the least common form of heart muscle disease.
For more information on the infiltrative cardiomyopathy program, please call farooq sheikh, md, facc, or selma mohammed, md, phd, at 2028778085. Infiltrative cardiomyopathy represents a heterogeneous group of diseases of the heart tissue with similar phenotypic expression. The rapid development of idiopathic restrictive cm immediately following a liver transplant is unusual. The hearts ventricles become rigid because abnormal tissue, such as scar tissue, replaces the normal heart muscle. It progressed within a few months to the point where the patient required. Consequently, the ventricles cant relax normally and fill with blood, and the atria become enlarged. Our new crystalgraphics chart and diagram slides for powerpoint is a collection of over impressively designed datadriven chart and editable diagram s guaranteed to impress any audience. P5402comparison of cardiopulmonary exercise test performance.
In all cases, efforts should be made to establish a specific diagnosis because the removal or avoidance of the causative agent eg, alcohol, cocaine, persistent tachycardia holds the best promise for reversal of ventricular dysfunction. Infiltrative cardiomyopathy icm refers to a heterogeneous group of myocardial disorders mainly cardiac amyloidosis, sarcoidosis, and hemochromatosis with characteristic deposition of abnormal substances within cellular and intercellular spaces, which leads to the development of ventricular diastolic dysfunction, systolic dysfunction or both. Restrictive cardiomyopathies european heart journal. They should be essential in everyday clinical decision making. They usually portend an adverse prognosis, although in rare instances eg, fabry disease early diagnosis can result in potentially curative treatment. The prognostic role of cardiopulmonary exercise cpx testing in infiltrative cardiomyopathy is unknown. Cardiomyopathies and pericardial disease tintinallis. Lowvoltage qrs complex was the sine qua non of infiltrative cardiomyopathy i.
Infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. Jul 08, 2015 infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. Differentiation of infiltrative cardiomyopathy from. Fabrys disease early diagnosis can result in potentially curative treatment. City of worcester, ma mobile strain screening program 4.
The treatment of cardiac amyloidosis involves the management of heart failure that results from restrictive cardiomyopathy and therapy. Find out information about infiltrative cardiomyopathy. The heart cavity is enlarged and stretched cardiac dilation, which results in weak and slow pumping of the blood, which in turn can result in the formation of blood clots. Infiltrative cardiomyopathy with conduction disease and. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Amyloidosis is a systemic and progressive disease and frequently involves more than one organ. Infiltrative cardiomyopathy with conduction disease and ventricular arrhythmia. O outline definition types symptoms and signs investigation treatment and prognosis the world health organization who defines.
Cardiovascular magnetic resonance in hypertrophic cardiomyopathy and infiltrative cardiomyopathy. Restrictive cardiomyopathy rcm is the most infrequent of the known cardiomyopathies. Novel treatment modal ities will be briefly discussed when applicable. Infiltrative cardiomyopathies focus on cardiac amyloidosis. Dilated cardiomyopathy dcm accounts for 60% of all cardiomyopathies and is defined as an intrinsic myocardial disease process characterized by progressive myocyte hypertrophy, dilation, and contractile dysfunction of one or both ventricles. Crf is committed to igniting the next wave of innovation in research and education that will help doctors save and improve the quality of their patients lives. If left untreated, the disorder could ultimately lead to.
Electrophysiologic and pathologic correlations saroja bharati, maurice lev, pablo denes, james modlinger, christopher wyndham, robert bauernfeind, melvin greenblatt, kenneth m. Apr 19, 2018 tctmd is produced by the cardiovascular research foundation crf. Restrictive cardiomyopathy rcm is characterized by nondilated left or right ventricle with diastolic dysfunction. A free powerpoint ppt presentation displayed as a flash slide show on id. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Infiltrative cardiomyopathy can have a variable prognosis depending on its etiology. The purpose of this study was to demonstrate the clinical features of rcm patients and evaluate the outcome and prognostic predictors of these patients. Dilated and restrictive cardiomyopathies online medical reference covers diseases of the myocardium associated with cardiac dysfunction. They portend an adverse prognosis, with only a few exceptions ie, glycogen storage disease, where early diagnosis can result in potentially curative treatment. Discoveries in molecular genetics and the description of ion channelopathies as diseases have resulted in new definitions and classification of cardiomyopathies. Restrictive cardiomyopathy tends to affect older adults. Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction, often resulting in the clinical syndrome of heart failure. Thus the heart is restricted from stretching and filling with blood properly.
Since this infiltrative process causes restrictive cardiomyopathy, your ea waves mitral inflow will show a restrictive pattern. Infiltrative cardiomyopathy article about infiltrative. Likewise, infiltrative disease that presents with a dilated left ventricle with global or regional wall motion abnormalities and aneurysm formation e. Arrhythmogenic right ventricular cardiomyopathy dysplasia. Rcm should be classified according to its etiology as either primary or. We present echocardiographic findings in seven patients with infiltrative cardiomyopathy due to amyloid. Classification arrhythmogenic right ventricular cm dilated hypertrophic. They portend an adverse prognosis, with only a few exceptions ie, glycogen storage disease, where early.
Restrictive cardiomyopathy rcm is a form of cardiomyopathy in which the walls of the heart are rigid but not thickened. These diseases have many causes, signs and symptoms, and treatments. Cardiac amyloid remains the archetypal infiltrative cardiomyopathy and is discussed in most detail in. Ppt restrictive cardiomyopathy powerpoint presentation. Restrictive cardiomyopathy cm usually develops and progresses slowly, over a course of years. Objective restrictive cardiomyopathy rcm is among the five major cardio myopathies. Genetic spectrum of idiopathic restrictive cardiomyopathy uncovered by nextgeneration sequencing.
Md author and disclosure information pdf pd f pdf read the full article. Restrictive cardiomyopathy is defined as heartmuscle disease that results in. Predictors of prognosis in 107 patients with idiopathic. Restrictive cardiomyopathy is the least common type of cardiomyopathies without uniformly accepted diagnostic criteria. In clinical practice, ischemic cardiomyopathy is frequently viewed as a type of dilated cardiomyopathy, although current major society classification systems exclude it. We investigated the potential role of highsensitivity cardiac troponin t hsctnt for differentiation of infiltrative cardiomyopathy from hcm. Nov 11, 2016 download fulltext pdf download fulltext pdf. Esc clinical practice guidelines aim to present all the relevant evidence to help physicians weigh the benefits and risks of a particular diagnostic or therapeutic procedure on hypertrophic cardiomyopathy. Infiltrative cardiomyopathies such as cardiac amyloidosis and fabry disease are difficult to differentiate from hypertrophic cardiomyopathy hcm because these cardiomyopathies share clinical and hemodynamic features of left ventricular lv hypertrophy and abnormal diastolic function 1.
Infiltrative cardiomyopathies focus on cardiac amyloidosis van n selby, md assistant professor of medicine ucsf advanced heart failure and heart transplant program october 9, 2015 overview infiltrative cardiomyopathies are characterized by the deposition of abnormal substances that cause the ventricular walls to become progressively rigid. Against it was the ecg showing poor rwave progression and echocardiographic findings suggestive of an infiltrative disease, with symmetric. If you continue browsing the site, you agree to the use of cookies on this website. It causes inflammatory masses or nodules called granulomas. Restrictive cardiomyopathies rcms, the least common form of heart muscle disease, are characterized as infiltrative and non infiltrative, storage diseases, and endomyocardial diseases. Conventional treatment should be aimed at relieving congestive symptoms. No pharmacological treatment has been shown to specifically improve diastolic filling or to prolong survival except heart transplantation in eligible. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Our purpose was to describe the performance distribution of patients with infiltrative cardiomyopathies on cpx in relation to age and sexmatched controls and compare the relative performance between cardiomyopathies.
The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. Infiltrative cardiomyopathy can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. Restrictive cardiomyopathy rcm is the least common phenotype of cardiomyopathies with controversy in its exact definition, epidemiology and diagnostic criteria. Cardiomyopathy definition cardiomyopathy is a chronic disease of the heart muscle myocardium, in which the muscle is abnormally enlarged, thickened, andor stiffened. In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having. Genetic diseases commonly present during childhood or adolescence. It is characterized by increased stiffness of the myocardium that causes pressure within the ventricle to rise precipitously with only small increase in volume. Cardiomyopathy denise antle, arnp, msn, ccrn, ccns critical care arnpcns genesis medical center davenport, iowa. Cardiomyopathy denise antle, arnp, msn, ccrn, ccns critical care arnpcns. The clinical presentation, along with functional and morphologic features, often provides. Restrictive cardiomyopathy rcm is characterized by nondilated left or. It is a progressive disorder that, if left untreated, can lead to early mortality.
Rare case of infiltrative cardiomyopathy secondary to scleromyxoedema. Restrictive cardiomyopathy circulation research aha journals. A summary of the pathology, diagnosis, disease course, and therapy is provided, along with the utility of noninvasive testing as a means. Jun 16, 2015 because infiltrative cardiomyopathy and hypertrophic cardiomyopathy hcm share clinical and hemodynamic features of left ventricular lv hypertrophy and abnormal diastolic function, it is often difficult to distinguish these entities. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. It is the least common of the three original subtypes of cardiomyopathy.
Differentiation of infiltrative cardiomyopathy from hypertrophic. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and. Among the causes of cardiomyopathy are nutritional disorders. The term cardiomyopathy is used to describe a heterogeneous group of diseases that directly alter cardiac structure, impair myocardial function, or alter myocardial electrical properties. Authored by corinne bottsilverman of the cleveland clinic.
Oct 09, 2017 echo assessment of cardiomyopathy pdf slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Spectrum of restrictive and infiltrative cardiomyopathies. Treatment is dependent on the etiology and extent of the disease and involves medications, device therapy, and, in some cases, organ. We describe the case of a patient who developed idiopathic restrictive cm fairly rapidly following a liver transplant. The condition is rare, but can be easily mistaken for other more common conditions of the heart. On the other hand, clinical management differs among these cardiomyopathies in terms of prognosis and treatment. Both atria will dilate and often you will see a pericardial or pleural effusion. Treatment can be effective in the early stages of disease. Cardiac amyloidosis was documented at autopsy in two patients, and the diagnosis was suggested by clinical, echocardiographic, tissue, or hemodynamic findings in the other five.