Arrhythmogenic right ventricular dysplasiacardiomyopathy arvdarvc is an inherited cardiomyopathy characterized by structural and functional abnormalities in the right ventricle rv resulting in ventricular arrhythmias. Jan 22, 2018 anatomical evidence in several species shows highly heterogeneous fat distribution in the atrial and ventricular myocardium. It is an important cause of sudden cardiac death scd in young adults, accounting for 11% of all cases and 22% of cases. Fatty replacement of the myocardium is a peculiar condition that has been reported to occur primarily in the right side of the heart.
In fact, it is well known that a certain amount of intramyocardial fat is present. The myocytes adjacent to the adipose tissue infiltrate showed multiple sarcoplasmic vacuoles, as would be expected for arvc. Frontiers fatty infiltration of the myocardium and. This prospective study included 996 subjects who underwent ecggated 16slice ct for determination of coronary calcium scores. Atrial appendages have fat deposits, and more so on the posterior left atrium. This fat is beneath the pericardium, lying between it and the muscular substance of the wall. Cardiac rupture due to severe fatty infiltration in the right.
The left ventricle and ventricular septum are usually spared. In this condition, the myocytes seem to be pushed apart rather than replaced and they appear structurally normal. Pdf fat in the right ventricle of the normal heart. Arrhythmogenic right ventricular dysplasia arvd, also called arrhythmogenic right ventricular cardiomyopathy arvc, is a rare form of cardiomyopathy, where the heart muscle of the right ventricle rv is replaced by fat andor fibrous tissue. Nevertheless, the fatty pattern originally described should be kept distinct from the pure fatty infiltration of the right ventricle, reported by burke et al. The heart showed mild hypertrophy of the left ventricle, and severe dilation of the right ventricle. The fatty replacement characteristically affects the right ventricle. The mechanism by which fatty infiltration promotes arrhythmogenicity andor. Mri may show fatty infiltration in the right ventricle and occasionally in the left ventricle 8, but this can also be seen in normal myocardium 3 and is no longer part of the diagnostic criteria 9. Anatomical evidence in several species shows highly heterogeneous fat distribution in the atrial and ventricular myocardium. Arrhythmogenic right ventricular cardiomyopathy and fatty. The cmr confirmed the normal biventricular size and systolic function movie ii in the onlineonly data supplement and, interestingly, revealed a region of intramyocardial fatty infiltration in the inferoseptal and septal walls of the left ventricle lv. Sep 23, 2014 axial black blood image of the right ventricle showing fatty infiltration in the free wall of the right ventricle. Of all cases of cardiac lipoma, only 10 tumors were found in the right ventricle 4.
Some of the patients 32%, 1753 also had right ventricle fibrofatty infiltration with mild to moderate rv dysfunction. A corrugated pattern to the right ventricular wall may be seen, known as the accordion sign. The first, fatty infiltration, is confined to the right ventricle. Fatty infiltration of right ventricle adipositas cordis.
Backgroundthe relationship between arrhythmogenic right ventricular cardiomyopathy arvc and pure fat replacement of the right ventricle is unclear methods and resultsmyocardial thickness, epicardial fat thickness, percent fibrosis, and intramyocardial fat infiltration were measured in 16 sections each from 25 hearts with typical fibrofatty arvc, 7 hearts with fat replacement of the. Human autopsy reports show that despite the fact that left ventricular mass far exceeds that of the right ventricle, total amount of fat tissue was. The normal pericardium, white arrow normal epicardial fat orange arrowhead and fat infiltrating the rv yellow arrow are shown in b ashley davidoff md. Mr imaging show fat in left ventricular myocardium that is of normal thickness. Marked lipomatous infiltration of the right ventricle. Right ventricle myocardial fat ct scan through the rv shows fatty infiltration in the right ventricle. Although such fat distributions are considered normal, fatty infiltration is regarded arrhythmogenic, and various cardiac pathophysiological conditions show excess myocardial fat deposits. Previous cmri based studies from patients with myotonic dystrophy have reported evidence of fatty infiltrations and fibrosis schmacht et al. Right ventricular fat infiltration in asymptomatic.
Fat in the right ventricle of the normal heart tansey. Adipositas cordis, fatty infiltration of the right ventricle. Right ventricular hypertrophy doesnt always cause symptoms. The normal hearth well known to present on its surface a certain amount of adipose tissue see fig. Fibrofatty replacement of the right ventricle wall, often with associated inflammation, is the hallmark of arrhythmogenic right ventricular cardiomyopathy arvc, a rare but established cause of sudden cardiac death in young adults. Arrhythmogenic right ventricular dysplasia is a rare genetic defect where the right ventricles muscle is replaced with fat and scar tissue. Mar 20, 2015 arrhythmogenic right ventricular dysplasia is a rare genetic defect where the right ventricles muscle is replaced with fat and scar tissue.
Infiltrating lipoma of the right ventricle involving the. It is normally most abundant along the course of the coronary arteries, along the inferior border of the right ventricle, at the apex, and at the origins of the great vessels. Often, the left ventricle tries to compensate for the problems with the right ventricle. The right side of the heart appears yellowish or whitish due to fatty or fibrofatty infiltration of the underlying myocardium the right ventricular inflow outflow tract appear lardaceous fatty the right ventricular free wall appears parchment like when held against a light source the changes may be focal in 20% and diffuse in 80% of cases. Arrhythmogenic right ventricular dysplasia arvd workup. Arrhythmogenic right ventricular cardiomyopathy radiology. Cardiac ct with multiplanar reconstruction in short axis plane a reveals right ventricular enlargement with diffuse fatty infiltration of. This involves a partial or nearcomplete substitution of myocardium with fatty tissue without wall thinning. Diagnosis and management of arrhythmogenic right ventricular. In the right ventricular myocardium, muscle fibers were definitely atrophic or absent, with massive fatty replacement. Fat in the right ventricle of the normal heart request pdf. A magnetic resonance imaging study will show l abnormal contraction patterns and enlargement of the right side of heart, as well as fatty infiltration of the muscle. Unenhanced a, b and contrast materialenhanced c, d ct scans obtained at the level of the right ventricular inflow region a, c and the inferior wall of the right ventricle rv b, d show marked dilatation of the right ventricle and conspicuous trabeculae arrows with lowattenuation fatty tissue in the inferior wall of the right. Biventricular arrhythmogenic ventricular cardiomyopathy in a 32yearold man presenting with syncope and familiar history of sudden cardiac death.
Myocardial fat at cardiac imaging rsna publications online. The massive fat infiltration of the right ventricle, without any evidence of fibrosis and myocyte degeneration, does not have a familial tendency. Current teaching is that fatty infiltration into the myocardium lipomatosis or cor adiposum rarely affects cardiac function. This can lead to a rapid heartbeat and other serious. Fatty infiltration of the right ventricle alone without fibrosis may also occur but its relation to sudden. Postmortem findings in a young man with congenital. Senescent fatty infiltration of the right ventricular outflow tract.
Infiltrating lipoma of the right ventricle involving the int. Helical ct features of arrhythmogenic right ventricular. There are characteristic electrocardiographic and echocardiographic features that all physicians need to be aware of. In cases of slight fatty infiltration, rv myocardial fat is typically. Transaxial noncontrast ct image of the heart in an 81yearold asymptomatic female shows extensive fatty infiltration of the right ventricular free wall extending into the right ventricular outflow tract arrow. View media gallery major limitations of cmri include limited experience of readers due to low prevalence and significant interobserver variability, resulting in high falsepositive rates. Fatty infiltration of the right ventricle is usually an incidental finding at postmortem, but may have clinical significance at times of physiologic stress. Oct 30, 2019 massive fatty infiltration of the right ventricle, without any evidence of fibrosis and myocyte degeneration, represents a questionable cause of sudden death and does not have a familial tendency 21 fig. The purpose of this study was to describe the structure and function of the heart in the presence of marked lipomatous infiltration of the right ventricular wall in patients referred for second opinions about fatty infiltration of the right ventricular wall and suspected arrhythmogenic right ventricular dysplasia.
Cardiac rupture due to severe fatty infiltration in the. Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Dilatation of the right ventricle is one criterion for diagnosis of arvc, table, and it is commonly seen in patients with arvc,,, fig 1. It is from a section of the right ventricle whose muscle is dissociated and encroached upon by adipose tissue which extends even to the internal surface under the endocardium. Right ventricular hypokinesis was defined as moderate or severe systolic hypokinesis of the right ventricular free wall by qualitative assessment. Fat infiltration of left ventricle a rare cause of sudden. Fatty infiltration of the myocardium and arrhythmogenesis. Arrhythmogenic right ventricular dysplasia arvd arrhythmogenic right ventricular dysplasia arvd, also called arrhythmogenic right ventricular cardiomyopathy arvc, is a rare form of cardiomyopathy, where the heart muscle of the right ventricle. May 28, 2015 arvd is defined by fatty or fibrofatty replacement of the normal right ventricular myocardium. Fatty or fibrous fatty transmural myocardial replacement may be diffuse to the entire right ventricular wall, but it is more often localized to the triangle of dysplasia, that is, the infundibular region. Arrhythmogenic cardiomyopathy acm, arrhythmogenic right ventricular dysplasia arvd, or arrhythmogenic right ventricular cardiomyopathy arvc, is an inherited heart disease acm is caused by genetic defects of the parts of heart muscle also called myocardium or cardiac muscle known as desmosomes, areas on the surface of heart muscle cells which link the cells together.
Adipositas cordis, fatty infiltration of the right. Oct 06, 2010 adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. We can determine whether alvc has right ventricular involvement by rvef. Although such fat distributions are considered normal, fatty infiltration is regarded arrhythmogenic, and various cardiac pathophysiological conditions show excess myocardial fat. We report a case of fatal right ventricular dysfunction immediately after cardiac transplantation secondary to massive fatty infiltration of the donor right ventricle. There are characteristic electrocardiographic and echocardiographic features that all physicians need to be. The normal pericardium, white arrow normal epicardial fat orange arrowhead and fat infiltrating the rv yellow arrow are shown in b. Ct scan through the rv shows fatty infiltration in the right ventricle. Pure fatty infiltration of the right ventricle vsarvcd. Fatty infiltration of the myocardium, if severe, can be a cause of serious cardiac dysfunction or, occasionally, sudden death. Arvc should be kept distinct from both fatty infiltration of the right ventricle and adipositas cordis. Magnetic resonance mr is an excellent tool for visualising the right ventricle, it allows a threedimensional evaluation of ventricular anatomy, volumes and when compared against other techniques it can better recognise the replacement of myocardial fatty and fibrofatty tissue, although fatty infiltration of the right ventricle is not. Cardiac ct with multiplanar reconstruction in short axis plane a reveals right ventricular enlargement with diffuse fatty infiltration of the right ventricular free wall arrows. Jan 17, 2020 some of the patients 32%, 1753 also had right ventricle fibro fatty infiltration with mild to moderate rv dysfunction.
Pathology outlines arrhythmogenic right ventricular. Fibrofatty arvc was characterized by right ventricular myocardial thinning, fat infiltration of the anterobasal and posterolateral apical right ventricle, subepicardial. Massive fatty infiltration of the right ventricle, without any evidence of fibrosis and myocyte degeneration, represents a questionable cause of sudden death and does not have a familial tendency 21 fig. Rupture during acute myocardial infarction has certainly been shown to be more common in the fatty heart. Of course, in such cases, there is great loss of the muscular power of the heart, the right ventricle being more affected than the left. Fat infiltration of left ventricle a rare cause of. Arrhythmogenic right ventricular cardiomyopathy arvc, also known as arrhythmogenic right ventricular dysplasia, is a disorder of the myocardium characterized by fatty or fibrofatty infiltration of the right ventricle rv, dilatation and dysfunction of the right ventricle as well as electrical instability, ventricular arrhythmia of right ventricular origin, heart failure and sudden death. Fibrofatty arvc was characterized by right ventricular myocardial thinning, fat infiltration of the anterobasal and posterolateral apical right ventricle, subepicardial left ventricular fibrofatty replacements 64%, myocyte atrophy 96%, and lymphocytic myocarditis 80%.
It involves predominantly the apical and infundibular regions of the rv. Arrhythmogenic right ventricular dysplasia is an inherited, progressive condition. Our patient demonstrated novel cardiac pathology i. Adipositas cordis, fatty infiltration of the right ventricle, and. Jan 22, 2018 characteristically, during af both left and right atrial myocardial walls have extensive fatty infiltration, patchy inflammation and interstitial fibrosis tanaka et al. Furthermore, there was extensive fatty infiltration of the right atrium, right ventricle and left ventricle. Nevertheless, it must be noted that although this epicardial fat distribution is considered normal, fatty infiltration of. Human autopsy reports show that despite the fact that left ventricular mass far exceeds that of the right ventricle, total amount of fat tissue was similar in both ventricles, corradi et al. Enlarged right heart ventricle heart disease medhelp. Solitary fatty infiltration within the left ventricle. To analyze the computed tomography ct findings of fatty replacement in the right ventricle rv of asymptomatic subjects and to correlate the ct findings with electrocardiogram ecg abnormalities. Lipomatous infiltration with right ventricular thickness. Fatty infiltration of the right ventricle alone without fibrosis may also occur but its relation to sudden death is not well established. Characterised by fatty infiltration of the right ventricle, it frequently results in life threatening cardiac arrhythmias, and is one of the important causes of sudden cardiac death in the young.
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