Role of cardiac mri in assessment of myocardial viability. Lists nomenclature distinguishing hcm from other genetic diseases. In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. Cardiac magnetic resonance in hypertrophic cardiomyopathy. Present and explain mri protocols to best optimise imaging of the different cardiomyopathies 3. The severity of lvot obstruction determines therapy and is an. Mri in the diagnosis of hypertrophic cardiomyopathy an article from the ejournal of the esc council for cardiology practice. Cardiomyopathy kardeomiopahthee refers to diseases of the heart muscle. These diseases have many causes, signs and symptoms, and treatments. The characteristic abnormalities seen on mri are impaired wall thickening, dilation of heart chambers, and impaired myocardial fiber shortening. To evaluate the capability of mri to differentiate cardiac amyloidosis ca, endstage hypertrophic cardiomyopathy hcm, and hypertensive heart disease hhd, which are important etiologies of left ventricular hypertrophy lvh and heart failure. Imaging studies provide solutions for most clinical needs, from diagnosis, anatomical and functional assessment, family screening. Aug 31, 20 to evaluate the capability of mri to differentiate cardiac amyloidosis ca, endstage hypertrophic cardiomyopathy hcm, and hypertensive heart disease hhd, which are important etiologies of left ventricular hypertrophy lvh and heart failure. Clinically, restrictive cardiomyopathy is difficult to distinguish from constrictive pericarditis, which is treatable.
Primary idiopathic restrictive cardiomyopathy is a rare condition that may present in both children and adults. Mohamed elsakka in arabic imaging findings of covid19. Markedly increased septal thickness is noted, with consequent increased tracer uptake resulting in an apparent fixed defect in the inferolateral wall. Cardiac magnetic resonance imaging value of cardiac cmr. Restrictive cardiomyopathy rcm is characterized by restrictive filling and reduced diastolic volume of either or both ventricles, with normal or nearnormal systolic function and wall thickness. Global hypokinesia of the left ventricle useful tip. Compiled consensus on the most important diagnostic modalities and genetic testing tools for the treatment of hypertrophic cardiomyopathy hcm. Hypertrophic cardiomyopathy radiology reference article. Mri can be used for accurate identification of the site and extent of ventricular hypertrophy and subaortic stenosis, and for quantification of myocardial mass. Recommended reading on cardiovascular magnetic resonance. Hypertrophic cardiomyopathy hcm is the most common inheritable myocardial disorder, with an estimated prevalence of 1. Echocardiogram, cardiac mri, cardiac ct, and nuclear medicine imaging are. Hypertrophic cardiomyopathy hcm is a relatively common form of heart disease due to genetic disorders and it is the most frequent cause of sudden death in young individuals 12.
Symptoms vary from none to feeling tired, leg swelling, and shortness of breath. Hypertrophic cardiomyopathy morbidity mortality mortality 1%3% per year some remain stable or improve. Hypertrophic cardiomyopathy hcm is the most common monogenic cardiovascular disorder, affecting one of every 500 adults. Cardiomyopathies cmps are myocardial diseases associated with cardiac dysfunction. Describe and illustrate the mr imaging features of each cardiomyopathy 4. Imaging studies provide solutions for most clinical needs, from diagnosis, anatomical and functional assessment, family screening, risk. These mutations are thought to cause an increase in myocyte stress and impaired function that eventually leads to left ventricular hypertrophy lvh and fibrosis. Cardiomyopathies are a heterogenic group of diseases of the myocardium. Review article the role of magnetic resonance imaging in hypertrophic cardiomyopathy edward t. Hypertrophic cardiomyopathy hcm is a type of cardiomyopathy and is the leading cause of sudden death from arrhythmias in infants, teenagers and young adults.
Hypertrophic cardiomyopathy hcm is a common genetic cardiovascular disease that affects approximately one out of every 500 persons. Mri differentiation of cardiomyopathy showing left. Jeroschherold m, sheridan dc, kushner jd, nauman d, burgess d, dutton d, alharethi r, li d, hershberger re. Role of cardiac mri in the assessment of cardiomyopathy. Cardiomyopathy dilated cmo lv too big lv too big function poor cardiomyopathy. Myocardial perfusion imaging in hypertrophic cardiomyopathy. Lv morphology and function myocardial fibrosis differential diagnosis cmr should be considered in patients with hcm at their baseline assessment if local resources and expertise permit. There are many different pheonotypes ranging from asymmetric to concentric hypertophy of. Constrictive pericarditis and restrictive cardiomyopathy. The long axis localizer can be obtained by using the following scan planes methods as pictured below. Hypertrophic cardiomyopathy hcm is a genetic cardiac disease. Restrictive cardiomyopathy cxr ct mri echocardiogram dilated atria, increased early lv filling velocity, decreased atrial filling velocity, and decreased isovolumetric relaxation time endomyocardial biopsy.
This book is distributed under the terms of the creative commons. They are classified as dilated cmp, hypertrophic cmp, restrictive cmp, arrhythmogenic right ventricular rv cmp, specific cmp, and nonclassified cmp 1. Highlight key features which are helpful for the cardiologist and establishing a diagnosis background there are two different classifications for cardiomyopathies. Lecture 16 heart failure part 2 cardiomyopathies medicine. Barry maron in collaboration with lisa salberg of the hypertrophic cardiomyopathy association. Myocardial perfusion imaginggated spect images were performed as per the american society of nuclear cardiology guidelines. A diagnosis of hypertrophic cardiomyopathy is based upon a number of features of the disease process. In figure 1 and 2 a case of hypertrophic cardiomyopathy. As the yield of genetic testing is only about 3560%, the diagnosis of hcm is still clinical and based on the demonstration of unexplained and usually asymmetric left ventricular lv hypertrophy by imaging modalities. To illustrate the importance of mri in the management and diagnosis of patients with nonischemic cardiomyopathy, i will discuss two very distinct conditions and the current role of mri in each disease. Oct 18, 2018 hypertrophic cardiomyopathy hcm is the most common genetic cardiomyopathy, affecting approximately 1 in 500 people. This includes physical examination, echocardiography, mri, and cardiac catheterization.
Cardiovascular mri is now accepted as a valuable tool in the initial assessment and followup of many acquired and congenital disorders. For patients, their families and interested physicians, second edition isbn. Aug 05, 20 hcmr novel markers of prognosis in hypertrophic cardiomyopathy hcmr the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. New haven connecticut cardiologist doctors physician directory read about how hypertrophic cardiomyopathy hcm is associated with thickening of the heart muscle, most commonly at the septum between the ventricles, below the aortic valve. The role of magnetic resonance imaging in hypertrophic. While there is use of echocardiography, cardiac catheterization, or cardiac mri in the diagnosis of the disease, other important considerations include ecg, genetic testing although not primarily used for diagnosis, and any family history of hcm or unexplained sudden death in otherwise. Restrictive cardiomyopathy no satisfactory medical therapy treat. Hypertrophic cardiomyopathy hcm is a genetically acquired condition that results in hypertrophic myocardium. Most popular is the genetic disorder, which can lead to sudden death, especially in young people. European association of cardiovascular imaging eacvi, european association of percutaneous cardiovascular. Guidelines and protocols for cardiovascular magnetic resonance in. Denise antle, arnp, msn, ccrn, ccns critical care arnpcns.
Our radiologists work closely with ohsu mri technologists in the art of creating. J am coll by cardiac magnetic resonance imaging magnetic resonance in hypertrophic cardiol 2004. It outlines all sequences and protocols currently applied in our mri section. Increased myofilament sensitivity to calcium, as well as increased accumulation of desmin and collagen type iii, has been implicated in the pathophysiology of this condition. The disease is characterized by left ventricular lv hypertrophy in the absence of another systemic or cardiac disease to. Cardiac imaging in hypertrophic cardiomyopathy intechopen. Hallmark myocardial hypertrophy thickening that is inappropriate and asymmetric. Constrictive pericarditis versus restrictive cardiomyopathy. Reduced systolic function but normal diastolic function.
Mri is helpful in visualizing the asymmetric thickening of the. Listing a study does not mean it has been evaluated by. Cardiomyopathy is a frequent reason for cardiac mri evaluation, which is now considered the most appropriate imaging technique for the diagnosis and follow up. Hcm results in diastolic heart failure due to impairment of myocardial relaxation during diastole. Imaging in hypertrophic cardiomyopathy american college. The diagnosis of hypertrophic cardiomyopathy involves several types of tests. The mode of inheritance is autosomal dominant in approximately 5060% of cases with over 600 mutations identified in sarcomeric genes to date. Recent research demonstrates the incremental utility of cardiac magnetic resonance in the diagnosis, therapeutic planning, and prognostication of this disease. Cardiac mri in idiopathic dilated cardiomyopathy, the left heart is markedly dilated and thinned, and mid wall enhancement, especially in the septum, is present in more than 50% of patients 4. Cardiac magnetic resonance imaging is an alternative approach which offers tissue contrast and a border definition that are often superior to what is achievable with echocardiography 3. Hypertrophic cardiomyopathy hcm is a condition in which a portion of the heart becomes thickened without an obvious cause. The regions in which 2d echocardiography led to a tendency to underestimate the degree of hypertrophy are the anterolateral wall 4 and the apical region 5.
Cardiac mr with lge can predict recovery of left ventricular systolic. Hcmr novel markers of prognosis in hypertrophic cardiomyopathy hcmr the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. More than just ground glass opacities, by henry guo, md, phd duration. In rare cases, the muscle tissue in the heart is replaced with scar tissue. Jan 06, 2003 this unique, succinct book was written by dr. However, hcm is often a dilemma for clinicians because it manifests with diverse phenotypic expressions and clinical courses.
Restrictive cardiomyopathy, or restrictive cardiac disease, is defined as abnormal diastolic function in association with relatively wellpreserved systolic function at least in the early stages of the disease. Accf and aha update guidelines on the diagnosis and. Hypertrophic cardiomyopathy is a genetic disease characterized by abnormal myocardial hypertrophy, which can lead to a wide clinical spectrum, including sudden cardiac death and heart failure. Cardiac mri in restrictive cardiomyopathy sciencedirect. Systemic hypertension is a highly prevalent potentially modifiable cardiovascular risk factor. Its early detection is important because it is the most common cause of sudden cardiac death among young people. It is meant to be a guide for those living with andor caring for those with hypertrophic cardiomyopathy hcm. In particular, assessment of cardiomyopathies with mri has been successful owing to its unique ability to depict different enhancement patterns in diseased myocardium on inversion recovery delayed gadoliniumenhanced images. Cardiovascular magnetic resonance in systemic hypertension. Mri most common finding is increased septal thickness but can have thickening anywhere along the lv, does not need to be uniform or symmetric high resolution cardiac mri superior to echocardiography for characterizing the phenotype, including the presence and magnitude of lvh in various segments, identification of. Although hypertrophic cardiomyopathy can generally describe a hypertrophied and nondilated left ventricle due to any cause, this article focuses on hypertrophic cardiomyopathy in the absence of another systemic or cardiac disease. This results in the heart being less able to pump blood effectively. Mri of nonischemic cardiomyopathy pubmed central pmc. Cardiac mri evaluation of hypertrophic cardiomyopathy.
Late myocardial enhancement has been associated with myocardial fibrosis and may allow for earlier detection of hypertrophic cardiomyopathy hcm than is currently available with echocardiography and ecg. Cardiac mri plays an important and useful role here. Hypertrophic cardiomyopathy hcm is the most common inheritable cardiac disorder, with an estimated prevalence of 1. Dilated cardiomyopathy radiology reference article. In hypertrophic cardiomyopathy hcm, the wall of the left ventricle is abnormally thickened. The primary finding on physical exam is often a murmur an abnormal heart sound caused by the rushing of blood across the obstruction if present. Lateenhancement mr images may demonstrate areas of fibrosis within the myocardium, characteristically in the mid or subepicardial myocardium, allowing. Look for ischaemia coronary artery disease as this is a treatable cause for dilated cardiomyopathy. Mri with electrocardiographic gating provides excellent depictions of cardiac anatomy in hypertrophic cardiomyopathy. The histologic basis of late gado resonance imaging in a canine myo inferoseptal left ventricular wall detected linium enhancement cardiovascular cardial infarction model. With the sagittal localizer the coil positioning can be checked. The aims of this article are to present the main features of mri of cardiomyopathy and to show selected images of cardiomyopathies.
Apr 23, 2017 imaging of cardiomyopathy discussed by dr. Cardiac magnetic resonance imaging of myocardial contrast uptake and blood flow in patients affected with idiopatic or dilated cardiomyopathy. In this patient the familiar history was negative, the ecg was doubtful figure 1, the echocardiogram resulted inconclusive due to an inadequate acustic window figure 2, left panel and the mri findings led to the final diagnosis figure 2, right panel. About half of cases are idiopathic, but the rest are usually due to metabolic, infectious, inflammatory, or toxic etiology. Hypertrophic cardiomyopathy is a complex genetic cardiovascular disorder with substantial variability in phenotypic expression and natural progression. Hypertrophic cardiomyopathy hcm is the most common genetic cardiomyopathy, affecting approximately 1 in 500 people. Assessment of restrictive cardiomyopathy of amyloid or idiopathic etiology by magnetic resonance imaging. We enrolled 26 patients presenting with both lvh and heart failure. Hypertrophic cardiomyopathy hcm is the most common inherited cardiomyopathy, with frequent features of asymmetric left ventricular hypertrophy, systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction.
This article provides guidelines for the performance of cmr in children and adults with congenital heart disease. Mr echo can be used for localization of all cardiac scan planes. It was published in december 2006 by blackwell futura, and is 128. Combining the diagnostic utilities of cardiac structures, myocardial perfusion, and various tissue characterizing pulse sequence methods in matching scan planes within a single imaging session, cardiac magnetic resonance imaging cmr provides a novel interrogation of myocardial physiology and abnormal anatomy from various forms of cardiomyopathy. The diagnosis and prognosis of hypertrophic cardiomyopathy hcm is generally clarified by noninvasive imaging, including screening by echocardiography and often refinement by cardiac magnetic resonance cmr. The relationship between regional wall thickness and function in these patients was studied by three dimensional 3d tagged magnetic resonance imaging mri utilizing the volumeelement approach. It is found across all racial groups and is the most common cause of sudden death in young athletes. Hoey1, mohamed elassaly 2, arul ganeshan1, richard w. This site focused on cardiomyopathy in children and has information about the disease, caring for a child with cardiomyopathy, and tending to a child in the hospital. From a dedicated orthogonal plane a long axis localizer of the heart is obtained. 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. Imaging plays an important role in the diagnosis of underlying causes for hypertension, in assessing cardiovascular complications of hypertension, and in understanding the pathophysiology of the disease process. Cardiac mri in clinical practice american college of physicians. Listing a study does not mean it has been evaluated by the u.