Arrhythmogenic Right Ventricular Cardiomyopathy and Fatty Replacement of the Right Ventricular Myocardium

Burke, Allen; Farb, Andrew; Tashko, Gerti; Virmani, Renu

doi:10.1161/01.cir.97.16.1571

Cited by 331 publications

(219 citation statements)

References 16 publications

(13 reference statements)

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“…There is agreement on the fibrofatty variant, but the adipose variant is considered by some authors as normal 22 and by others as pathological. 23 We consider that isolated adiposis infiltration is pathological if it is associated with disorganized strands of myocytes that might be isolated.…”

Section: Discussionmentioning

confidence: 99%

Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia

et al. 2003

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Background— Sudden death is a possible consequence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Prevalence of ARVC/D in unexpected sudden cardiac death (USCD), however, remains imprecise, as do circumstances of death and ARVC/D-associated gross and microscopic findings, especially His bundle anomalies. Methods and Results— We reviewed 14 000 forensic autopsies required by judicial authorities from January 1980 to January 1999 in a 2 000 000-resident area. Age, gender, and circumstances of death were recorded. Hearts were examined macroscopically and microscopically. In this series, the ARVC/D group accounted for 200 consecutive cases (10.4%) of USCD, including 108 males and 92 females (average age 32.5 and 34.5 years, respectively). Nearly one third of deaths occurred during the fourth decade of life. Circumstances of death were various, but 75.6% occurred during everyday life events (at home, 63.1%; in the street, 6.6%; or at work, 6.1%); only 7 cases (3.5%) occurred during sports activity. Nineteen cases (9.5%) happened during the perioperative period. Adipose infiltration of the right ventricle was either isolated (20%) or associated with fibrosis (74.5%) and lymphocytes (5.5%). A total of 14.5% of cases had cardiac hypertrophy, assessed by an increase in heart weight and/or left ventricular wall thickness. In most cases, the His bundle and its branches were abnormal either because of infiltration of adipose tissue (8.1%), fibrosis (54.3%), or both (5.6%). Conclusions— In ARVC/D, both sexes are equally affected, and there is a peak of risk during the fourth decade. Death most frequently occurs during sedentary activity. His abnormalities and left ventricular hypertrophy may be associated with ARVC/D.

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Section: Discussionmentioning

confidence: 99%

Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia

et al. 2003

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“…If similar degradation occurs in the human heart, it will become a focus for lethal arrhythmia at some stage in life. The most important pathological feature of ARVD is the degeneration of right ventricular cardiomyocytes 22 . Our ARVD mouse model reproduced the specific right ventricular degeneration from the outside inwards.…”

Section: Discussionmentioning

confidence: 99%

Lamr1 functional retroposon causes right ventricular dysplasia in mice

et al. 2004

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“…The water-fat separation DIRT technique removed the black line artifact resulting from this opposed-phase signal cancellation. DIRT may find use as a fat saturation alternative for MR angiography and for the identification of lipid pathology such as lipomas and fatty infiltration of the right ventricle (19). Fluid or edema imaging is often performed using long echo time turbo-spin-echo imaging (20,21).…”

Section: Discussionmentioning

confidence: 99%

Magnetic resonance separation imaging using a divided inversion recovery technique (DIRT)

Goldfarb

2010

Magn. Reson. Med.

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The divided inversion recovery technique is an MRI separation method based on tissue T 1 relaxation differences. When tissue T 1 relaxation times are longer than the time between inversion pulses in a segmented inversion recovery pulse sequence, longitudinal magnetization does not pass through the null point. Prior to additional inversion pulses, longitudinal magnetization may have an opposite polarity. Spatial displacement of tissues in inversion recovery balanced steadystate free-precession imaging has been shown to be due to this magnetization phase change resulting from incomplete magnetization recovery. In this paper, it is shown how this phase change can be used to provide image separation. A pulse sequence parameter, the time between inversion pulses (T180), can be adjusted to provide water-fat or fluid separation. Example water-fat and fluid separation images of the head, heart, and abdomen are presented. The water-fat separation performance was investigated by comparing image intensities in short-axis divided inversion recovery technique images of the heart. Fat, blood, and fluid signal was suppressed to the background noise level. Additionally, the separation performance was not affected by main magnetic field inhomogeneities. Magn Reson Med 63:1007-1014, 2010. V C 2010 Wiley-Liss, Inc. Key words: MRI; separation imaging; fat; lipid; fluid; myocardial infarction; pulse sequence; inversion recovery; bSSFP Tissue characterization in MRI is mostly performed using a visual assessment of image contrast generated by contrast agents or intrinsic magnetic relaxation time differences. MR images with pixel intensities weighted by T 1 or T 2 relaxation times are most commonly used. Additionally, preparation pulses can generate a variety of image contrasts between tissues. For example, fat saturation (1), magnetization transfer contrast radiofrequency pulses (2), or diffusion gradient pulses (3) may be added to impose specific image contrasts. The difference in pixel intensities allows identification of and discrimination between diseased and healthy tissues using a single image.MR image separation techniques provide multiple images of the same imaging plane with pixels separated by an intrinsic tissue parameter. Water-fat separation (4) provides two images. Pixels composed primarily of lipids are displayed in the fat image and the other pixels composed primarily of water are displayed in the second image. Water-fat separation is most commonly performed using the chemical shift between water and fat nuclei. Image separation can also be performed using other parameters such as T 1 relaxation times or T 1 -weighted imaging pixel intensity (5). In T 1 -weighted spoiled gradient echo images, fat usually has the brightest signal and water-fat separation can be performed using a simple image intensity threshold.Recently, an artifact was identified during late gadolinium-enhanced myocardial infarct imaging using an inversion recovery balanced steady-state free precession (IR-bSSFP) technique (6,7). This artifact is a spa...

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Arrhythmogenic Right Ventricular Cardiomyopathy and Fatty Replacement of the Right Ventricular Myocardium

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Cited by 331 publications

References 16 publications

Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia

Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia

Lamr1 functional retroposon causes right ventricular dysplasia in mice

Magnetic resonance separation imaging using a divided inversion recovery technique (DIRT)

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