Overview of cardiomyopathies merck manuals professional edition. Denise antle, arnp, msn, ccrn, ccns critical care arnpcns. Molecular characterization of pediatric restrictive cardiomyopathy. Infiltrative cardiomyopathies david bejar, paolo c. Apr 19, 2018 tctmd is produced by the cardiovascular research foundation crf. 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. Restrictive cardiomyopathy rcm is another cause of diastolic heart failure. Integration of advanced cardiac imaging modalities, such as cardiac magnetic resonance imaging mri and positron emission tomography. Recent advances in understanding and managing cardiomyopathy.
In rare cases, the muscle tissue in the heart is replaced with scar tissue. Cardiomyopathies are divided into 3 main types based on the pathologic features see figure forms of. In most cases, the heart muscle weakens and is unable to pump blood to the rest of the body as well as it should. Vhd in rcmp and infiltrative cmp conclusions i rcmp and infiltrativestorage cmp are a rare heterogeneous group of heart muscle diseases with distinctive clinical and cardiac imaging features vhd, particularly of m and t valves, is possible, with different mechanisms functional or sometimes organic, according to the disease subtype. Heart failure hf is a common finding in infiltrative storage cmp. A number of genetic causes of restrictive cardiomyopathy have been identified.
Nevertheless, recent reports combining these techniques with. In infiltrative cardiomyopathy there is concentric hypertrophy granular or speckled appearance of myocardium although this feature is known to be a characteristic feature of cardiac amyloidosis, it is not a specific finding and hence should not be used in isolation. Both atria will dilate and often you will see a pericardial or pleural effusion. Restrictive cardiomyopathy is most frequently caused by infiltrative diseases and storage diseases see tab. Ventricular arrhythmias vas are a significant cause of morbidity and mortality in patients with cardiomyopathy cm. Restrictive cardiomyopathy heart and blood vessel disorders.
Infiltrative cardiomyopathies focus on cardiac amyloidosis. Cardiomyopathy kardeomiopahthee refers to diseases of the heart muscle. Hybrid petcmr imaging systems, which promise to combine the. Spectrum of restrictive and infiltrative cardiomyopathies jacc. Treatment with enalopril and furosemide was initiated. Alcoholic cardiomyopathy usually develops about 10 years after sustained, heavy alcohol consumption. Restrictive cardiomyopathy rcm is the least common among cardiomyopathies. Restrictive cardiomyopathy rcm is characterized by nondilated left or right.
In addition to restrictive cardiomyopathy, there are dilated cardiomyopathy and hypertrophic cardiomyopathy see also overview of cardiomyopathy. Treatment must combine the underlying plasma dyscra. The aims of this article are to present the main features of mri of cardiomyopathy and to show selected images of cardiomyopathies. Authored by corinne bottsilverman of the cleveland clinic. Ppt restrictive cardiomyopathy powerpoint presentation. Since a nuclear medicine scan can better determine damage to the heart caused by. In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. 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.
Spectrum of restrictive and infiltrative cardiomyopathies. 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. These diseases have many causes, signs and symptoms, and treatments. A free powerpoint ppt presentation displayed as a flash slide show on id. Gallego delgado m, montserrat l, ruizcano mh, et al. Infiltrative cardiomyopathy article about infiltrative. Cardiomyopathy refers to progressive impairment of the structure and function of the muscular walls of the heart chambers. Description restrictive cardiomyopathy is the least common type of cardiomyopathy in the united.
Genetic spectrum of idiopathic restrictive cardiomyopathy uncovered by nextgeneration sequencing. Treatment of rcm includes treating the underlying cause. Likewise, infiltrative disease that presents with a dilated left ventricle with global or regional wall motion abnormalities and aneurysm formation e. Restrictive cardiomyopathy associated with skeletal. Treatment must combine the underlying plasma dyscrasia and the. The treatment of cardiac amyloidosis involves the management of heart failure that results from restrictive cardiomyopathy and therapy. Many of these involve the same family of sarcomeric genes that is associated with dilated and hypertrophic cardiomyopathy, and in some instances the same mutation can manifest as more than one of these phenotypes. Deposition of amyloid protein causes changes of tissue composition and architecture, and is associated with changes in signal intensity on t1weighted images. Thus the heart is restricted from stretching and filling with blood properly. Restrictive cardiomyopathies are the least common form of heart muscle disease.
The most important causative diseases are amyloidosis, prior radiotherapy, sarcoidosis, hemochromatosis, and fabry disease. Genetic spectrum of end stage idiopathic restrictive cardiomyopathy. The detection of infiltrative cardiomyopathies, including lysosomal and glycogen storage disorders, iron overload, and amyloidosis both light chain amyloidosis and transthyretin amyloidosis variants, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via diseasespecific therapies. However, as hf represents the final clinical pathway of many cardiac diseases, the recognition of many other multisystem manifestations e. Lowvoltage qrs complex was the sine qua non of infiltrative cardiomyopathy i. Infiltrative cardiomyopathies are characterized by the deposition of abnormal substances that cause the ventricular walls to become progressively rigid, thereby impeding ventricular filling. Restrictive cardiomyopathy rcm is the least common among. Cardiomyopathy caused by heart attacks referred to as ischemic cardiomyopathy results from scarring in the heart muscle. Readers must therefore always check the product information and clinical procedures with the most up to date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. Dilated and restrictive cardiomyopathies online medical reference covers diseases of the myocardium associated with cardiac dysfunction. We investigated the potential role of highsensitivity cardiac troponin t hsctnt for differentiation of infiltrative cardiomyopathy from hcm.
Cardiomyopathy is a frequent reason for cardiac mri evaluation, which is now considered the most appropriate imaging technique for the diagnosis and followup of this wide range of myocardial diseases. Restrictive and infiltrative cardiomyopathy recognition by. Pediatric restrictive cardiomyopathy rcm is a genetically heterogeneous. Oxford university press makes no representation, express or implied, that the drug dosages in this book are correct.
Pdf infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. Differentiation of infiltrative cardiomyopathy from. Restrictive cardiomyopathy circulation research aha journals. Differentiation of infiltrative cardiomyopathy from hypertrophic cardiomyopathy using highsensitivity cardiac troponin t. It can be challenging to differentiate amyloidosis from hypertrophic cardiomyopathy, sarcoid and infiltrative lymphoma on echocardiography but easy to differentiate on cardiac mr. The infiltrative cardiomyopathies are a diverse group of cardiac diseases, which are characterized by the deposition of abnormal substances within heart tissue that cause the ventricular walls to develop either diastolic dysfunction or, less commonly, systolic dysfunction. Definition and classification of the cardiomyopathies uptodate. Restrictive cardiomyopathy rcm is the least common form of all the cardiomyopathies which is defined as a myocardial disorder with stiffened ventricles leading to impaired diastolic filling but. Restrictive cardiomyopathy rcm is a form of cardiomyopathy in which the walls of the heart are rigid but not thickened. Idiopathic restrictive cardiomyopathy is very rare. Some infiltrative cardiac diseases increase ventricular wall thickness, while others cause chamber enlargement with secondary wall thinning. Its principal abnormality is diastolic dysfunctionspecifically, restricted ventricular filling.
They portend an adverse prognosis, with only a few exce. Mar 25, 2020 clinicians should also consider infiltrative cardiomyopathy ie, glycogen storage disease, fabry disease, hurler disease, and others when evaluating patients with suspected cardiac amyloidosis. Restrictive cardiomyopathy rcm is the most infrequent of the known cardiomyopathies. Learn cardiomyopathy with free interactive flashcards. Infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations.
It causes inflammatory masses or nodules called granulomas. Fabrys disease early diagnosis can result in potentially curative treatment. Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction, often resulting in the clinical syndrome of heart failure. Protocol for assessment of restrictive cardiomyopathy. Since this infiltrative process causes restrictive cardiomyopathy, your ea waves mitral inflow will show a restrictive pattern. How to evaluate for and manage inflammatory and infiltrative. Merge cardio for nuclear cardiology nuclear medicine is an emerging area for providers in the cardiology space, facilitating better diagnoses, especially for conditions such as coronary artery disease and cardiomyopathy. The most common infiltrative causes of rcm include amyloidosis, hemochromatosis, and sarcoidosis. They portend an adverse prognosis, with only a few exceptions ie, glycogen storage disease, where early diagnosis can result in potentially curative treatment. Restrictive and infiltrative cardiomyopathy recognition by echo. Infiltrative cardiomyopathy definition of infiltrative. The lack of effective medical treatment for children with this disease. 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.
Dilated and restrictive cardiomyopathies cleveland clinic. Infiltrative cardiomyopathy can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. 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. They usually portend an adverse prognosis, although in rare instances e. To avoid the technical challenges of merging such datasets, given different. Restrictive cardiomyopathy is a form of cardiomyopathy in which the walls of the heart become rigid.
It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders. It is the least common of the three original subtypes of cardiomyopathy. The infiltrative cardiomyopathies are a diverse group of cardiac diseases that are characterized by the deposition of abnormal substances within the heart tissue that causes the ventricular walls to develop either diastolic dysfunction or, less commonly and more of a late presentation of the disease, systolic dysfunction. Treatment can be effective in the early stages of disease. Aug 30, 2017 restrictive cardiomyopathy rcm is a rare disease of the myocardium and is the least common of the 3 clinically recognized and described cardiomyopathies. Larger scars or more numerous heart attacks increases the risk that ischemic cardiomyopathy will develop. Coronary artery catheterization revealed normal coronary vessels with an ejection fraction of 39%.
Find out information about infiltrative cardiomyopathy. A cardiomyopathy is a primary disorder of the heart muscle. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. A contemporary definition for cardiomyopathy is a myocardial disorder in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease sufficient to explain the observed myocardial abnormality. Hypertrophic cardiomyopathy distribution of lvh 600 patients anterior and inferior septum 31% anterior septum only 25% klues hg, jacc 1995. O outline definition types symptoms and signs investigation treatment and prognosis the world health organization who defines. Jul 08, 2015 infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. Dilated cardiomyopathy sa is a 53 year old diabetic, hypertensive black male who was diagnosed with a dilated cardiomyopathy in 1998. The myocardial wall in rcm is too rigid to allow for relaxation due to fibrosis or infiltration by other tissues. Definition cardiomyopathy is an ongoing disease process that damages the muscle wall of the lower chambers of the heart. Restrictive cardiomyopathy definition of restrictive. Cardiomyopathy is a progressive disease of the myocardium, or heart muscle. Choose from 500 different sets of cardiomyopathy flashcards on quizlet.
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