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1.
MRI assessment of right ventricular dysplasia   总被引:3,自引:0,他引:3  
Right ventricular dysplasia is a new entity of unknown origin in the classification of cardiomyopathies. Also known as arrhythmogenic right ventricular cardiomyopathy (ARVC) or arrhythmogenic right ventricular dysplasia, it is a disease of the heart muscle characterised by fibroadipose atrophy mainly involving the right ventricle and responsible for severe ventricular arrhythmias and sudden death also in young people. Magnetic resonance imaging provides evidence of ventricular dilatation at the outflow tract, thinning and thickening of the wall, diastolic bulging areas (especially located at the level of the right ventricle outflow tract) and fatty substitution of the myocardium mainly at the level of the right ventricle. Many radiologists erroneously consider the previously described fatty substitution as the main sign of ARVC, even though an evaluation of fat substitution alone may be a source of error for two reasons: firstly, because isolated areas of fatty replacement are not synonymous with ARVC since small non-transmural focal fatty areas of fat are also present in the normal patients; and secondly, because the MRI detection of fat may be overestimated due to partial-volume artefacts with normal subepicardial fat. Cardiac MRI can also be employed for the diagnosis of idiopathic right ventricular outflow tract tachycardia. Considering the evolutive nature of the disease, the non-invasiveness of MRI allows the follow-up of these patients and may be considered an excellent screening modality for the diagnosis of ARVC in family members. Finally, MRI can be employed in electrophysiological studies to locate the arrhythmogenic focus and reduce sampling errors. Electronic Publication  相似文献   

2.
Helical CT features of arrhythmogenic right ventricular cardiomyopathy.   总被引:5,自引:0,他引:5  
Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a disorder of the heart muscle of unknown origin. It is characterized by electrical instability of the heart as a result of replacement of the right ventricular myocardium with fatty or fibrous fatty tissue. Dilatation of the right ventricle; fatty tissue in conspicuous trabeculae of the right ventricle, especially in the anterior wall, apex, and inferior (diaphragmatic) wall; and a scalloped appearance (bulging) of the right ventricular wall are characteristic findings at helical computed tomography (CT) that may be used to diagnose ARVC. Fatty tissue in the left ventricle and ventricular septum is seen relatively frequently in ARVC, and fat in the ventricular septum is another useful finding for diagnosis of ARVC with helical CT. ARVC is usually diagnosed on the basis of clinical or pathologic findings, and electron-beam CT is superior to nongated helical CT in assessment of abnormal right ventricular function. However, with knowledge of the characteristic findings, standard nongated helical CT can be helpful in diagnosing ARVC.  相似文献   

3.
Right ventricular arrhythmogenic dysplasia is a rare cardiomyopathy which involves the right ventricle either totally or partially. Up to now diagnoses have been based on Ecg, hemodynamics, angiography and echocardiography. This paper deals with the first 7 patients examined also by means of Computed Tomography. The CT picture is well defined and rather accurate. The most important elements are: total (6/7) or partial (1/7) enlargement of the right ventricle; thinning of right ventricular myocardium (6/7); marked increase in subepicardial fat limited to the right ventricular wall (7/7); right ventricle hypokinesia (7/7). A good correlation exists between CT, echographic and traditional methodologies findings. Because of its densitometric evaluation, CT is much more precise in the demonstration of fatty degeneration. Moreover, it can be very useful in differentiating intracardiac thrombi from hypertrophic papillary muscles and trabeculae.  相似文献   

4.
OBJECTIVE: The aim of our study was to correlate the findings of three MR imaging sequences with the clinical findings of possible arrhythmogenic right ventricular cardiomyopathy in pediatric patients. MATERIALS AND METHODS: Twenty-six consecutive pediatric patients underwent MR imaging with ECG-gated non-breath-hold spin-echo T1-weighted non-fat-suppressed and fat-suppressed sequences. The MR images were evaluated for thinning or fat signal in the right ventricular wall and for enlargement or increased trabeculation of the right ventricle or right ventricular outflow tract. Cine MR imaging was used to assess wall motion abnormalities. Cardiac biopsy was performed in 17 patients. Biopsy results and other clinical findings suggesting arrhythmogenic right ventricular cardiomyopathy were tabulated. RESULTS: Two MR imaging studies were of poor quality as a result of arrhythmias, and one study was incomplete. In the 23 remaining patients, there were (mean +/- SD) 1.5 +/- 1.0 and 0.8 +/- 1.0 findings of possible arrhythmogenic right ventricular cardiomyopathy in the non-fat-suppressed and the fat-suppressed sequences, respectively. Fat-compatible signal in the myocardium was detected in 16 (70%) of 23 non-fat-suppressed studies and in five (22%) of 23 fat-suppressed studies (p = 0.003). The non-fat-suppressed sequence had a higher sensitivity (75% vs 43%) and a lower specificity (38% vs 75%) for fatty infiltration than did the fat-suppressed sequence when correlated with the biopsies. The linear correlation between all MR findings and all clinical diagnostic criteria, including biopsy, was better for the combination of cine and both T1 sequences (r = 0.58) than for the non-fat-suppressed (r = 0.53) or fat-suppressed (r = 0.46) T1 sequences alone. CONCLUSION: MR imaging showed moderate correlation with the clinical criteria in the diagnosis of arrhythmogenic right ventricular cardiomyopathy.  相似文献   

5.
Arrhythmogenic right ventricular cardiomyopathy or dysplasia, a heart muscle disease of unknown cause, is anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadipose tissue. It is usually considered a selective disorder whereas concomitant left ventricular involvement has been noted in a few cases. Two cases of the disease with evidence of extensive left ventricular involvement at pathologic examination are described. Hearts from two patients who died suddenly showed extensive biventricular infiltration by fibrofatty tissue in the first case and exclusively in the wall of the left ventricle the localization of the fatty and fibrotic lesions. These findings might suggest that the various localizations of the fibroadipose tissue are rather different expressions of the same disease and it is preferable to be termed 'arrhythmogenic cardiomyopathy' as other studies also indicate.  相似文献   

6.
Cine-computed tomography of arrhythmogenic right ventricular dysplasia   总被引:1,自引:0,他引:1  
The CT diagnostic features of arrhythmogenic right ventricular dysplasia (Uhl anomaly) are described in a 72-year-old man. High speed cine-CT was performed using millisecond exposures and multilevel scanning. Ventricular morphology and function were evaluated, and findings were compared with two-dimensional echocardiography and equilibrium gated blood pool imaging. Cine-CT showed a dilated hypokinetic right ventricle with a markedly thin anterior wall and normal left sided chambers. The clinical aspects of Uhl anomaly are reviewed.  相似文献   

7.
Arrhythmogenic left ventricular dysplasia' and sudden death   总被引:1,自引:0,他引:1  
A 32-year-old man who died suddenly and unexpectedly was found at autopsy to have prominent fatty infiltration of his left ventricle with fibrous scarring, in the presence of normal coronary arteries. The right ventricle was minimally involved. A diagnosis of ventricular dysplasia largely limited to the left ventricle was made. Subsequent family screening identified a brother with clinical manifestations of ventricular dysplasia. This case provides further evidence for the association of left ventricular dysplasia with sudden death, and demonstrates that left ventricular involvement may also be inheritable. Whether predominantly left ventricular dysplasia is a manifestation of right ventricular dysplasia, or is a separate entity, is yet to be determined.  相似文献   

8.
Replacement of myocardium by fat, particularly of the right ventricle, is often diagnosed as arrhythmogenic right ventricular dysplasia. At autopsy, however, 68% of scars associated with chronic ischemic heart disease have shown fatty metaplasia in the scar. Four patients with a past history of previous myocardial infarctions and computed tomography demonstration of fatty change in left ventricular regions of hypokinesis and infarction are presented. It is proposed that these findings represent ischemic fatty metaplasia, an alternative etiology of fatty tissue replacing myocardium.  相似文献   

9.
Left-sided ventricular arrhythmogenic cardiomyopathy is rare and represents a rather different expression of the arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC). Among sudden cardiac deaths, ARVC plays a significant role. ARVC is considered as a cardiomyopathy of unknown aetiology that primarily involves the right ventricle (RV) and is characterized by progressive replacement of myocytes by fibro-fatty tissue, complicating a spectrum of arrhythmias. Predominant ARVC with left ventricular (LV) involvement is also reported. The LV variant of arrhythmogenic cardiomyopathy with minimal or no RV involvement is rare. A 31-year-old previously healthy young man, without a significant family history, was found dead in bed. Autopsy revealed an enlarged heart and asymmetrical LV hypertrophy with widely patent coronary arteries. LV myocardium demonstrated evidence of prominent epicardial fibro-fatty tissue that is predominantly fatty in nature and infiltrates into the myocardium. Microscopy of the LV free wall showed fibro-fatty tissue infiltration into the epicardial aspect of the LV that extends well into the mid-myocardium. A moderate to marked degree of interstitial fibrous tissue deposition was noted about adipocytes and cardiomyocytes. There was no evidence of chronic ischaemic changes or of significant myofibre disarray. The RV showed minimal fibro-fatty infiltration with normal myocytes. This report highlights a rare case which confirms previous observations that the LV variant of arrhythmogenic cardiomyopathy could occur with minimal or no involvement of the RV. Further studies are required in this context to elicit the spectrum and the exact nature of this disease.  相似文献   

10.
Arrhythmogenic right ventricular dysplasia (ARVD) is an inheritable cardiomyopathy that is characterized by fibrofatty infiltration of the RV myocardium, often resulting in arrhythmias and sudden cardiac death. The diagnosis often requires imaging of the right ventricle, typically by magnetic resonance imaging. We describe a case of AVRD in which cardiac computed tomography was used as the imaging method for the right ventricle. The CT images showed changes consistent with ARVD to include RV enlargement, excessive trabeculations, fatty infiltration, and marked RV hypokinesia. We believe these images show a novel use for multislice CT in the diagnostic evaluation of patients with suspected ARVD.  相似文献   

11.
An 18-year-old white male collapsed suddenly in his home and died. At autopsy, the right ventricle of the decedent was noted to be dilated with marked thinning of the wall focally. Microscopically, the myocardium of the right ventricle was noted to be significantly thinned focally, where transmural infiltration with fibroadipose tissue was noted. Depending on the criteria utilized to render such a diagnosis, these findings were consistent with arrhythmogenic right ventricular cardiomyopathy (ARVC). Subsequent microscopic examination of the SA and AV node, however, revealed the presence of a cystic tumor of the AV node (CTAVN), a known cause of sudden death from arrhythmia. The case represents the first reported case of ARVC and CTAVN occurring together in the same individual.  相似文献   

12.
Diagnosis of arrhythmogenic right ventricular dysplasia: a review.   总被引:5,自引:0,他引:5  
Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disorder of primarily the right ventricle, with unknown cause and prevalence and with a frequent familial occurrence. The typical clinical manifestation consists of ventricular arrhythmias with a left bundle branch block (LBBB) pattern that occur predominantly in young adults. ARVD may result in sudden death. Other manifestations are electrocardiographic repolarization and depolarization changes, structural abnormalities that range from subtle wall aneurysms within the so-called "triangle of dysplasia" to biventricular regional or global dysfunction, and localized or widespread fibrofatty infiltration of the right ventricular myocardium. The diagnosis of ARVD is based on the presence of major and minor criteria encompassing genetic, electrocardiographic, pathophysiologic, and histopathologic factors. The imaging modalities used to evaluate right ventricular abnormalities include conventional angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance (MR) imaging. Among these techniques, MR imaging allows the clearest visualization of the heart. Because MR imaging depicts both functional and structural abnormalities, positive MR imaging findings should be used as important additional criteria in the clinical diagnosis of ARVD. MR imaging appears to be the optimal technique for detection and follow-up of clinically suspected ARVD.  相似文献   

13.
RATIONALE AND OBJECTIVES: The purpose of this study was to develop and implement MR sequences for chemical shift-selective breath-hold cine imaging of the heart. Fibroadipose conversion of myocardium in cases suspected of right ventricular dysplasia should be revealed in fat- and water-selective MR images of high quality. METHODS: Frequency-selective saturation of one chemical shift component was applied in modified k-space-segmented, electrocardiography-gated sequences, allowing high-quality cine imaging of the human heart in a single breath-hold. Phantom studies and human examinations in eight normal subjects (aged 24-62 years) and in seven patients (aged 31-47 years) with suspected right ventricular dysplasia were performed. The patients showed suspicious findings, such as a dyskinetic and dilated right ventricle combined with ventricular arrhythmia, and underwent MR imaging after exclusion of other possible reasons (eg, coronary artery disease or pulmonary hypertension). RESULTS: High selectivity to the desired chemical shift component was confirmed by test measurements in a phantom containing water and lipids. In the human subjects, minor problems with magnetic field inhomogeneities appeared in the thoracic walls only. Four patients with suspected right ventricular dysplasia showed clearly abnormal signal behavior of the right myocardial wall in both fat- and water-selective cine images. Bright transmural structures were exhibited in fat-selective images, but the origin of the fat (epicardium or infiltrated myocardium) was often difficult to assess. CONCLUSIONS: Right ventricular areas with fibrosis and fatty degeneration often show normal signal intensity in standard T1-weighted images but can be differentiated from normal tissue by the new chemical shift-selective breath-hold cine techniques.  相似文献   

14.
PURPOSE: Black blood single shot FSE sequences (Nffse) employ 180 degrees RF refocalisation pulses preceded by an inversion RF double pulse associated to presaturation pulses. The latter produce signal void of the external volume, and possible reduction of the field of view without wrap-around artifacts along the phase coding direction. The aim of our study was to compare the diagnostic possibilities of the Nffse sequences with those of conventional SE study of cardiac morphology. MATERIAL AND METHODS: Twenty-five patients (19 males and 9 females with age ranging from 20 to 54 years) presented findings suggesting right ventricular arrhythmogenic dysplasia. MR examinations were performed with a 1,5 T unit (GE Signa Horizon Echospeed 8.3, Milwaukee, USA) and Torso Phased Array coil positioned at thoracic level. The morphologic study was performed with SE multiphase-multislice ECG-gated sequences (TR: R-R, TE: 30 ms, FOV 320X250, matrix 160X256, slice thickness 10 mm, acquisition time about 5 minutes) and Single-Shot FSE Half Fourier sequences (TR: R-R, TE: 30 ms, flip angle 120 degrees, ETL 30-40, FOV 360X180, Phase FOV 0,5, VBW 64 MHz, slice tickness 10 mm, acquisition time about 10-12 seconds), by imaging along the long and short axis. The study was completed with Fast Gradient Echo sequences (TR: 9ms, TE: 8,2ms, flip angle 25 degrees, VBW 15,63 MHz, FOV 320X250, 10 mm slice thickness, matrix 128X256), subsequently assessed by cine-MR. In order to compare both sequences, two experienced radiologists performed an analysis of quantitative parameters (signal intensity ratio between fat and muscular interventricular septum) and qualitative parameters (double blind evaluation for the presence of cardiac and respiratory artifacts). RESULTS: The signal intensity ratio for the Nffse sequence images was 4.63 +/- 1.56 on the long axis and 7.69 +/- 2.46 on the short axis, whereas it was 3.17 +/- 0.64 on the long axis and 3,50 +/- 0,75 on the axis one for SE images, with a statistically significant difference (p<0,001 and p<0.002 for the long and short axis, respectively). The two radiologists evaluation of the magnitude of artifacts on the SE and Nffse images was similar only as regards the images with significant artefacts alone. Nffse images consistently afforded a detailed evaluation of the right ventricular wall, although blurring artifacts were more common than with good quality SE images. Presence of fatty infiltration of the right ventricle wall was observed in 5 out of 25 patients. In the remaining 20 patients no fatty substitution of the muscular wall of the right ventricle was observed. DISCUSSION AND CONCLUSIONS: The Nffse sequences provide a number of gated multiphase-multislice images, similar to that obtained by conventional SE sequences, in one breath-hold time interval. Due to high intrinsic contrast and reduction of motion artifacts, the Nffse sequences allow a good evaluation of the ventricular morphology and subepicardial and paracardiac adipose tissue. Image quality can be suboptimal due to blurring artifacts. Therefore Nffse sequences can be advantageously employed to image patients with suspected right ventricular arrhythmogenic dysplasia, whenever conventional SE images exhibit substandard quality.  相似文献   

15.
PURPOSE: To assess electrocardiographically gated spin-echo (SE) and double inversion-recovery fast SE magnetic resonance (MR) imaging in the depiction of intramyocardial fat in cadaveric heart specimens and patients with arrhythmogenic right ventricular dysplasia (ARVD). MATERIALS AND METHODS: A phantom was used to determine the effective in-plane spatial resolution of SE and fast SE MR imaging protocols. Two cadavers with proved ARVD were imaged with identical sequences with spectrally selected fat suppression. Contrast-to-noise ratios (CNRs) of intramyocardial fat in the right ventricle (RV) were compared by using analysis of variance and Student t test with Bonferroni correction. Eleven patients with ARVD and 10 control subjects underwent fast SE MR imaging. Two blinded readers semiquantitatively evaluated images for fat conspicuity and image quality. RESULTS: Fast SE MR imaging achieved better spatial resolution but lower CNR than that of gated SE imaging. CNRs in cadaveric specimens were higher for double R-R than for single R-R fast SE sequences for all section thicknesses (P <.0001). Absolute CNR values were higher for fat-suppressed fast SE sequences than for those without fat suppression. Cadaveric specimens demonstrated fatty infiltration from epicardium toward endocardium of the RV free wall. Intramyocardial fat was detected in eight of 11 (73%) patients with ARVD and in no control subjects (P <.001). CONCLUSION: Intramyocardial fat detection in ARVD was better with fast SE MR imaging alone and combined with fat suppression than was gated SE MR imaging. When fast SE imaging is applied in vivo, however, breath-holding constraints limit the spatial resolution for RV fat detection.  相似文献   

16.
A histomorphometry study was carried out to assess the degree of right ventricular lipomatosis in control autopsy cases and to evaluate if this was correlated with parameters such as sex, age, body mass index (BMI) and heart weight. A total of 70 adult cases were selected from cases of violent death between 1991 and 1999 and where autopsies were carried out in the Department of Pathology and Forensic Medicine in Garches. All cases with heart pathology, abnormal BMI or putrefaction were excluded. Cases with lung or liver pathology were also excluded. Furthermore, 10 adult autopsy cases who died suddenly of arrhythmogenic right ventricular cardiomyopathy (ARVC) were compared with 10 age and sex-matched control cases. Details on sex, age, BMI and heart weight were obtained from the post-mortem records. For each case one sample of the right front ventricular wall was fixed in 10% neutral saline-buffered formalin and one 5-μm-section was stained with haematoxylin and eosin. The Leica Quantimet 500 analysis system was used for the histomorphometrical study. The mean degree of lipomatosis was measured under blind conditions in the ventricular wall and epicardial fat was excluded. Covariance analysis and the Wilcoxon test were used for statistics. The mean age of the control population was 37.5 years, the sex ratio was 1.9:1 (male:female). The mean degree of lipomatosis was 17.03% and the degree of lipomatosis was significantly correlated with age (p = 0.0029) but not with sex, BMI and heart weight. There was a statistically significant increase in fat in ARVC cases compared with age and sex-matched controls (p < 0.001). Fat infiltration of the right ventricle could be an adipose involution due to an ageing process and heavy fat infiltration can be difficult to distinguish from ARVC. Our study suggests that fat infiltration is not essential for the post-mortem diagnosis of ARVC which also requires fibrosis and degenerating myocytes trapped within areas of fibrosis. Received: 6 October 2000 / Accepted: 20 March 2001  相似文献   

17.
OBJECTIVE: 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. METHODS: This prospective study included 996 subjects who underwent ECG-gated 16-slice CT for determination of coronary calcium scores. The CT findings were analyzed in terms of location, pattern, and degree of RV fat infiltration, along with the shape and dimension of RV. The RV fat was regarded present when a region showed less than or equal to -30 Hounsfield units as revealed by CT. RESULTS: Computed tomography features suggestive of RV fat were found in 169 subjects (17%; 136 men and 33 women; mean age, 56.3 years). The most frequent location of fat was the basal superior wall (93%); next was the middle superior wall (72%); and then, the RV outflow tract (44%). Subjects with moderate to severe involvement were older than those with mild involvement (P = 0.012). In 3 subjects, the RV wall thickening with fat was more than 5 mm. Angular deformity and undulating appearance of the RV also occurred in 25 (15%) and 20 subjects (12%), respectively. However, these were not patients with ECG findings positive for arrhythmogenic RV dysplasia. CONCLUSIONS: Asymptomatic subjects may have fat in RV on CT. However, these subjects show no RV dysfunction or significant ECG-abnormalities consistent with the diagnosis of arrhythmogenic RV dysplasia.  相似文献   

18.
One type of arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disorder resulting in fatty replacement of the right ventricular wall and consequent fatal arrhythmias. It is a major cause of sudden death in young people. Mutations in the genes encoding desmosomal proteins have been identified as a cause of ARVC. We report a case of sudden death during exercise in a juvenile. This case showed fatty replacement of the right ventricular wall, which suggests that ARVC may have been associated with the cause of death. Further genetic analysis showed a novel homozygous R292C substitution of the desmoglein-2 gene (DSG2), which encodes a desmosomal protein. In addition to morphological examination, genetic analysis may be useful for diagnosis of ARVC-suspected autopsy cases.  相似文献   

19.
致心律不齐性右室心肌病的MRI诊断   总被引:4,自引:1,他引:4  
目的:用心脏MR新技术评价致心律不齐性右室心肌病(ARVC)的MRI征象,探讨MR扫描技术。方法:对15例临床、超声诊断或疑为ARVC的病人进行RM检查,使用GE Signa1.5TCV/iMR扫描系统,扫描序列包括黑血技术:双反转恢复快速自旋回波(double-IR FSE)和三反转自旋回波(triple-IR FSE)序列;白血技术:快速电影成像(fastcine)序列。扫描平面有短轴面、四腔面和长轴面。结果:10例诊断为ARVC,ARVC的主要MRI表现有:右室壁脂肪信号3例,右室壁变薄9例,右心室扩大6例,室壁瘤形成2例,右心腔内慢血流信号9例,右室射血分数降低6例,右房扩大3例。右室乳状肌和左室心尖部、室间隔前部累及2例。黑血技术可显示心脏解剖、形态及组织特性,白血技术主要了解心脏功能及心肌壁的运动,短轴面和四腔面显示病变较满意。结论:ARVC的RMI表现具有一定的特征,多序列、多平面成像的MR新技术对该病的诊断更准确、更可靠。  相似文献   

20.
MRI is seeing an increasing role in the evaluation of suspected arrhythmogenic right ventricular dysplasia (ARVD). Our aims were to establish wall thickness criteria for diagnosis of ARVD based on MR imaging measurements. A cross-sectional case-control retrospective study of 21 cardiac MR exams over a 3 year period identified five patients (two men, three women) of average age 43 years (range 36-48) who were diagnosed with ARVD and 16 patients (nine men, seven women) of average age 52 years (range 25-78) who were diagnosed with normal right ventricular wall motion. Patient demographic characteristics (age, sex) and right ventricular free wall thickness (RVFWT) were evaluated for predictive ability. Calculated RVFWT was (mean+/-SD, in mm); (4.4+/-1.4) for ARVD, and (7.8+/-2.9), controls with P<0.001. Logistic regression analysis indicated that sex and age were not significant independent predictors (P>0.05). RVFWT allowed for a prediction rule with Area under the receiver operator curve of 0.94 to be generated. In our study, measurement of the thickness of the right ventricular free wall using cardiac-gated MRI proved to be a statistically significant predictor of ARVD.  相似文献   

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