Consequently, the ventricles cant relax normally and fill with blood, and the atria become enlarged. 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. Dilated and restrictive cardiomyopathies online medical reference covers diseases of the myocardium associated with cardiac dysfunction. Dilated cardiomyopathy arising as primary myocardial disease of unknown etiology or as disorders of toxic, familial, or infective origin. A free powerpoint ppt presentation displayed as a flash slide show on id. Infiltrative cardiomyopathy can have a variable prognosis depending on its etiology. The following are summary points from part 1 of a 2part series on the spectrum of restrictive and infiltrative cardiomyopathies. Infiltrative cardiomyopathy definition of infiltrative.
The prognostic role of cardiopulmonary exercise cpx testing in infiltrative cardiomyopathy is unknown. Infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. They portend an adverse prognosis, with only a few exceptions ie, glycogen storage disease, where early. Ppt cardiomyopathies powerpoint presentation free to view. Arrhythmogenic right ventricular cardiomyopathy dysplasia. Secondary and infiltrative cardiomyopathies are the least common forms of cardiomyopathy and often are the most difficult to treat. Restrictive cardiomyopathies european heart journal. A summary of the pathology, diagnosis, disease course, and therapy is provided, along with the utility of noninvasive testing as a means. Restrictive cardiomyopathies are the least common form of heart muscle disease. Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction, often resulting in the clinical syndrome of heart failure. Genetic spectrum of end stage idiopathic restrictive cardiomyopathy. 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. The hearts ventricles become rigid because abnormal tissue, such as scar tissue, replaces the normal heart muscle.
Author links open overlay panel rebecca perry phd a b c joseph b. 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. Lowvoltage qrs complex was the sine qua non of infiltrative cardiomyopathy i. Restrictive cardiomyopathy american heart association. Infiltrative cardiomyopathy with conduction disease and.
However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of. Restrictive cardiomyopathy rcm is the least common phenotype of cardiomyopathies with controversy in its exact definition, epidemiology and diagnostic criteria. However, lowvoltage qrs complex is not a uniform finding with the infiltrative cardiomyopathies. Md author and disclosure information pdf pd f pdf read the full article. Spectrum of restrictive and infiltrative cardiomyopathies jacc. It is a progressive disorder that, if left untreated, can lead to early mortality. Predictors of prognosis in 107 patients with idiopathic. Cardiomyopathies and pericardial disease tintinallis. Likewise, infiltrative disease that presents with a dilated left ventricle with global or regional wall motion abnormalities and aneurysm formation e. Cardiomyopathy denise antle, arnp, msn, ccrn, ccns critical care arnpcns. The topic infiltrative restrictive cardiomyopathy you are seeking is a synonym, or alternative name, or is closely related to the medical condition restrictive cardiomyopathy. It causes inflammatory masses or nodules called granulomas. The treatment of cardiac amyloidosis involves the management of heart failure that results from restrictive cardiomyopathy and therapy. Discoveries in molecular genetics and the description of ion channelopathies as diseases have resulted in new definitions and classification of cardiomyopathies.
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. Restrictive cardiomyopathy rcm is characterized by nondilated left or right ventricle with diastolic dysfunction. Echocardiography in infiltrative cardiomyopathy sciencedirect. For more information on the infiltrative cardiomyopathy program, please call farooq sheikh, md, facc, or selma mohammed, md, phd, at 2028778085. Hypertrophic cardiomyopathy is the second most common cause of sudden cardiac death in the. The condition is rare, but can be easily mistaken for other more common conditions of the heart. Cardiomyopathy denise antle, arnp, msn, ccrn, ccns critical care arnpcns genesis medical center davenport, iowa. Classification arrhythmogenic right ventricular cm dilated hypertrophic. They portend an adverse prognosis, with only a few exceptions ie, glycogen storage disease, where early diagnosis can result in potentially curative treatment. Cardiomyopathy definition cardiomyopathy is a chronic disease of the heart muscle myocardium, in which the muscle is abnormally enlarged, thickened, andor stiffened. Echocardiography has been useful in the evaluation of congestive and hypertrophic cardiomyopathies.
Objective restrictive cardiomyopathy rcm is among the five major cardio myopathies. Differentiation of infiltrative cardiomyopathy from. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Dilated and restrictive cardiomyopathies cleveland clinic. Cmp primary disease intrinsic to cardiac muscle dilated cmp hypertrophic cmp infiltrative cmp left ventricular noncompaction arrythmogenic right ventricular cmp takotsubo cardiomyopathy.
Gallego delgado m, montserrat l, ruizcano mh, et al. Noninvasive imaging in cardiac deposition diseases shah. P5402comparison of cardiopulmonary exercise test performance. Restrictive cardiomyopathies are the least common form of heart muscle. Ppt restrictive cardiomyopathy powerpoint presentation. Thus the heart is restricted from stretching and filling with blood properly. Echocardiographic manifestations of infiltrative cardiomyopathy. Genetic diseases commonly present during childhood or adolescence. Treatment is dependent on the etiology and extent of the disease and involves medications, device therapy, and, in some cases, organ. 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. Secondary and infiltrative cardiomyopathies springerlink. Infiltrative cardiomyopathies focus on cardiac amyloidosis.
We present echocardiographic findings in seven patients with infiltrative cardiomyopathy due to amyloid. Pdf cardiovascular magnetic resonance in hypertrophic. 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 a form of cardiomyopathy in which the walls of the heart are rigid but not thickened. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and. 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. Tctmd is produced by the cardiovascular research foundation crf. The purpose of this study was to demonstrate the clinical features of rcm patients and evaluate the outcome and prognostic predictors of these patients. They should be essential in everyday clinical decision making. Restrictive cardiomyopathies rcms, the least common form of heart muscle disease, are characterized as infiltrative and non infiltrative, storage diseases, and endomyocardial diseases. Denise antle, arnp, msn, ccrn, ccns critical care arnpcns. Among the causes of cardiomyopathy are nutritional disorders.
Conventional treatment should be aimed at relieving congestive symptoms. Cureus a novel case of idiopathic restrictive cardiomyopathy. It is characterized by increased stiffness of the myocardium that causes pressure within the ventricle to rise precipitously with only small increase in volume. Cardiac amyloid remains the archetypal infiltrative cardiomyopathy and is discussed in most detail in. 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. Against it was the ecg showing poor rwave progression and echocardiographic findings suggestive of an infiltrative disease, with symmetric. No pharmacological treatment has been shown to specifically improve diastolic filling or to prolong survival except heart transplantation in eligible. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Both atria will dilate and often you will see a pericardial or pleural effusion. Hypertrophic cardiomyopathy is the second most common cause of sudden cardiac death in the adolescent population and the. Jul 08, 2015 infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations.
Restrictive cardiomyopathy circulation research aha journals. Novel treatment modal ities will be briefly discussed when applicable. Treatment can be effective in the early stages of disease. Rare case of infiltrative cardiomyopathy secondary to. Since this infiltrative process causes restrictive cardiomyopathy, your ea waves mitral inflow will show a restrictive pattern. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. 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.
However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having. The clinical presentation, along with functional and morphologic features, often provides. We describe the case of a patient who developed idiopathic restrictive cm fairly rapidly following a liver transplant. View enhanced pdf access article on wiley online library. Rare case of infiltrative cardiomyopathy secondary to scleromyxoedema. Restrictive cardiomyopathy is defined as heartmuscle disease that results in. Chart and diagram slides for powerpoint beautifully designed chart and diagram s for powerpoint with visually stunning graphics and animation effects. City of worcester, ma mobile strain screening program 4. Authored by corinne bottsilverman of the cleveland clinic. 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 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. Electrophysiologic and pathologic correlations saroja bharati, maurice lev, pablo denes, james modlinger, christopher wyndham, robert bauernfeind, melvin greenblatt, kenneth m. The rapid development of idiopathic restrictive cm immediately following a liver transplant is unusual.
It is the least common of the three original subtypes of cardiomyopathy. 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. Restrictive cardiomyopathy rcm is characterized by nondilated left or. Amyloidosis is a systemic and progressive disease and frequently involves more than. Recent advances in the imaging assessment of infiltrative. These diseases have many causes, signs and symptoms, and treatments. They usually portend an adverse prognosis, although in rare instances eg, fabry disease early diagnosis can result in potentially curative treatment. Rcm should be classified according to its etiology as either primary or.
Infiltrative cardiomyopathy article about infiltrative. If left untreated, the disorder could ultimately lead to. Restrictive cardiomyopathy is the least common type of cardiomyopathies without uniformly accepted diagnostic criteria. 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. 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. Apr 19, 2018 tctmd is produced by the cardiovascular research foundation crf.
On the other hand, clinical management differs among these cardiomyopathies in terms of prognosis and treatment. It progressed within a few months to the point where the patient required. Restrictive and infiltrative cardiomyopathies are the least frequently encountered form of primary heart muscle disease in adults within the developed world. Cardiomyopathy kardeomiopahthee refers to diseases of the heart muscle. 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. Restrictive cardiomyopathy rcm is the most infrequent of the known cardiomyopathies. In clinical practice, ischemic cardiomyopathy is frequently viewed as a type of dilated cardiomyopathy, although current major society classification systems exclude it.1317 1283 1508 1204 533 1299 973 112 1453 1595 906 542 273 1109 824 354 1340 1556 238 341 792 1156 907 244 1589 819 376 1264 452 1187 996 1504 1134 311 673 130 942 453 1235 676 417 102 720 596 449 1312 412 399