Critical analysis of cineangiographic criteria for diagnosis of arrhythmogenic right ventricular dysplasia

Biplane 30-degree RAO and 60-degree LAO RV selective cineangiography was performed in 21 patients with significant ventricular arrhythmias (ventricular tachycardia in 14, salvos in three, and complex PVCs in seven) and a high presumption of arrhythmogenic RV dysplasia (ARVD), and in a control group of 10 presumed normal individuals. Comparing the two series revealed the lack of specificity of some angiographic images usually reported as suggestive signs of ARVD, such as slow dye evacuation of RV during the levophase and deep fissuring in the anterior wall with a "pile of plates" image. Inversely, localized morphologic and contraction abnormalities in the RV free wall were more sensitive and specific signs for diagnosis of ARVD; these were localized akinetic or dyskinetic bulges sometimes giving a true image of aneurysm (90%), wide and deep fissuring of the apex or of the inferior wall (33%), and large areas of akinesia. By order of frequency, these abnormalities were found on the apex in 71%, on the inferior wall in 52%, on the anterior wall in 48%, in the subtricuspid area in 38%, and on the pulmonary infundibulum in 33%. These localized lesions can suffice for the diagnosis of RV dysplasia in the absence of associated pathologies, such as ischemic heart disease or congenital defects. Usually a global RV systolic dysfunction is associated in ARVD, as confirmed by greater RV volumes (134 +/- 26 vs 79 +/- 10 ml/m2 for RVEDV, p less than 0.001; 76 +/- 34 vs 32 +/- 6 ml/m2 for RVESV, p less than 0.001), and lower RV ejection fraction (58 +/- 18% vs 47 +/- 8%, p less than 0.001) in the ARVD group compared to controls. Nevertheless, normal RV volumes and ejection fraction can be observed in some localized forms with mono- or bisegmental lesions in which RV systolic dysfunction is absent or moderate, and extensive forms with multiple segmental lesions where RV systolic dysfunction is constant and often severe. Six out of 21 patients in the ARVD group exhibited obvious global or segmental LV dysfunction, indicating the possibility of biventricular forms, as previously reported in other publications.     

Angiographic right and left ventricular function in arrhythmogenic right ventricular dysplasia

We prospectively documented right ventricular (RV) and left ventricular (LV) volumes and ejection fractions in a large series of patients with arrhythmogenic RV dysplasia/cardiomyopathy (ARVD/C). Eighty-five patients with ARVD/C and 11 controls underwent 2 successive orthogonal right and left monoplane x-ray-digitized cineangiographies. Volumes were calculated using the hemielliptical RV and ellipsoidal LV models. All controls and 58 of 85 patients (ARVD/C-I) had a RV ejection fraction > or =35% and 27 patients had a RV ejection fraction <35% (ARVD/C-II). Tricuspid annulus plane systolic excursion (TAPSE) was lower in ARVD/C-II than in ARVD/C-I patients (6 +/- 3 vs 14 +/- 3 mm) and controls (16 +/- 2 mm) (each p <0.001). In patients with ARVD/C, TAPSE was positively related to RV ejection fraction (r = 0.79) and to crista supraventricularis shortening (r = 0.81) (each p <0.001). Sensitivity and specificity of TAPSE <12 mm in identifying patients with RV ejection fraction <35% were 96% and 78%, respectively. LV ejection fraction was > or =50% in 68 patients, 40% to 49% in 10, and <40% in 7. Diffuse RV outflow tract aneurysm was observed in 9 patients, all belonging to ARVD/C-II, and this sign identified patients with LV ejection fraction <40% with 86% sensitivity and 96% specificity. In conclusion, 68% of ARVD/C patients had normal RV ejection fraction and RV volumes, and 80% of ARVD/C patients had normal LV ejection fraction. Decreased TAPSE <12 mm and a diffuse RV outflow tract aneurysm were sensitive and specific indicators of RV ejection fraction <35% and LV ejection fraction <40%, respectively.     

Feasibility and variability of three dimensional echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Arrhythmogenic right ventricular dysplasia (ARVD/C) is a genetic cardiomyopathy characterized by fibrous fatty replacement of the right ventricular (RV) myocardium, leading to progressive RV failure and ventricular arrhythmias in young athletes. This study evaluated whether transthoracic, real-time, 3-dimensional echocardiography (3DE) can adequately assess RV morphology and function in ARVD/C by comparing 3DE with cardiac magnetic resonance (CMR), the current reference standard. Three-dimensional echocardiography was prospectively performed in 58 patients (23 with ARVD/C, 20 first-degree relatives with no ARVD/C, 8 with idiopathic ventricular tachycardia with no ARVD/C, and 7 healthy volunteers). All patients, except 15 patients with ARVD/C with implanted defibrillators, also underwent CMR. Three-dimensional echocardiography and CMR-derived RV volumes and ejection fractions were obtained by offline data analysis by blinded, independent observers. The mean age of the study group was 37 +/- 11 years (30 men). The feasibility of 3DE was high, and analyzable images were obtained in all subjects. Three-dimensional echocardiography revealed a wide variety of RV morphologic abnormalities in ARVD/C. There was a good correlation between 3DE and CMR for RV end-systolic volume (r = 0.72, p = 0.0001), RV end-diastolic volume (r = 0.50, p = 0.0001), and the RV ejection fraction (r = 0.88, p = 0.001). We found high intraobserver and moderate interobserver correlations for 3DE estimations of volumes and ejection fractions. In conclusion, 3DE measurements of RV volumes and ejection fractions closely correlate with CMR values and may be useful in the follow-up of patients with ARVD/C.     

Value and limitations of 2-dimensional echocardiography in the identification of arrhythmogenic right ventricular dysplasia

The aim of this study was to evaluate the value and limitations of Cross-sectional Echocardiography (CSE) in the diagnosis of Arrhythmogenic right ventricular dysplasia (ARVD). Diagnosis was based on accepted clinical, electrocardiographic, electrophysiologic and angiographic criteria. CSE criteria for the diagnosis are segmental right ventricular wall motion abnormalities of unknown cause, usually associated with localized or diffuse dilatation of right ventricular (RV) chamber and with the presence of localized anomalies consisting of sacculation or bulging of RV wall. Comparison of CSE and RV angiographic findings was performed in 8 patients with ARVD (6 men and 2 women, aged 10 to 37 years, mean 28 years). CSE and angiography compared closely when diffuse RV enlargement and wall motion abnormalities were identified by both techniques. Bulging and sacculation of the RV wall at CSE predicted the presence of similar lesions at angiography, but agreement for specific location was poor and, in addition, CSE showed low sensitivity in their detection. The inherent different information provided by the two methods added to the subjectivity of the qualitative analysis probably accounts for the inconsistencies. Therefore in patients with diagnosed ARVD RV enlargement, otherwise unexplained, associated with wall motion abnormalities and localized anomalies at CSE strongly supports the diagnosis and avoids the need for angiography. By other hand, in patients with high clinical suspicion of ARVD a negative CSE study can not exclude the diagnosis and angiography should be indicated.     

M-mode echocardiographic study of right ventricular involvement in the acute phase of inferoposterior myocardial infarction

The aim of this study was to assess the value of a non-invasive technique, echocardiography, in diagnosing RV extension during the acute phase of myocardial infarction. Forty patients with an acute infero-posterior infarct were divided into two groups according to the presence (Group A) or absence (Group B) of RV akinesia on angiography. M mode echocardiography was carried out from two positions: left parasternal, for the study of quantitative parameters: RV and LV diameters, wall thickness and excursion, VCF and fractional shortening, mitral and tricuspid valve morphology, aortic and left atrial dimensions; subxostal: for the study of one parametere: RV inferior wall motion assessed as normal or akinetic. A comparative statistical study including a group of 35 controls was carried out. The results showed at comparable values in both groups, that patients in Group A had lower global LV function, hypokinesia of the LV posterior wall with minor changes in the mitral valve echo and LA dimension; akinesia of the RV inferior wall, a direct and specific sign of RV infarction, was observed in 50 p. 100 of cases; in comparison with the other two groups, despite large individual variations, there was a significant increase in RV diameter (p less than 0,001) and RV/LV diameter (p less than 0,001), and in the amplitude of systolic motion of the RV anterior wall (p less than 0,05 and p less than 0,01). Other qualitative signs were inconstant: paradoxical septal motion (7/20), pericardial separation (3/20), tricuspid B point (5/20). Dilatation of the RV was inconstant (50 p. 100 of cases) but its association with paradoxical septal motion was indicative of significant tricuspid incompetence. Akinesia of the RV inferior wall seemed to be of prognostic value: RVEDP and the extent of angiographic RV akinesia were greater in its presence (p less than 0,05). There was a slight correlation between RV dimensions (RV diameter and RV/LV diameters) and the extent of angiographic RV akinesia (R = 0,50, p less than 0,05) and with cardiac index (R = 0,60, p less than 0,05). This study shows that M mode echo provides positive direct signs of RV infarction in about 50 p. 100 of cases. The sensitivity of the technique is therefore relatively low. However, it does distinguish the more severe forms of biventricular infarction, especially when complicated by tricuspid incompetence.     

Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

AIMS: Incidence and clinical presentation of thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) were analysed. In reports on ARVD/C, thromboembolism is rarely mentioned. The possible risk factors are: right ventricle (RV) dilatation, aneurysms, and wall motion abnormalities. METHODS AND RESULTS: A group of 126 patients (89 male, 37 female, aged 43.6+/-14.3) with ARVD/C was retrospectively analysed for the presence of thromboembolic complications. The mean follow-up period was 99+/-64 months. Thromboembolic complications, i.e. pulmonary embolism (n=2), RV outflow tract thrombosis with severe RV failure (n=1), and cerebrovascular accident associated with atrial fibrillation (n=2) were observed in 4% of the patients. Spontaneous echogenic contrast was observed in seven patients with severe damage to RV. In four of them supraventricular arrhythmias resulting in heart failure were reported. Annual incidence of thromboembolic complications was 0.5/100 patients. CONCLUSIONS: (i) ARVD/C may be complicated by thrombosis. Annual incidence of such complications is significantly lower than reported for left ventricle failure. (ii) Anticoagulation should be used in ARVD/C patients with large, hypokinetic RV and slow blood flow. (iii) Patients with severe forms of ARVD/C, thrombus formation in the RV and/or spontaneous echocardiographic contrast are at higher risk of a poor outcome.     

Arrhythmogenic right ventricular dysplasia: A cause of ventricular tachycardia in children with apparently normal hearts

Arrhythmogenic right ventricular dysplasia (ARVD), a cardiomyopathy with hypokinetic areas limited to the wall of the right ventricle (RV), has been recently described as a cause of recurrent ventricular tachycardia (VT) in young adults with an otherwise normal heart. We reviewed 26 cases of recurrent VT in children and found 10 patients with no clinically recognizable abnormality aside from the dysrhythmia. Three of these 10 patients had ARVD. These three patients were initially seen at 1, 12, and 14 years of age with premature ventricular contractions (PVCs) and/or VT. Sustained VT occurred spontaneously or during stress testing. The PVCs and the VT were of left bundle branch block contour, suggesting RV site of origin. The diagnosis of ARVD was based on wall motion abnormalities of the RV demonstrated angiographically. We suggest that ARVD could be a significantly common cause of VT in children with an apparently normal heart.     

Evolving role of multidetector computed tomography in evaluation of arrhythmogenic right ventricular dysplasia/cardiomyopathy

The purpose of this study was to report 1 center's experience with multidetector computed tomography (MDCT) in the evaluation of patients suspected to have arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C). RV dilatation/dysfunction is 1 of the most important criteria for establishing the diagnosis of ARVD/C. Cardiac magnetic resonance imaging (MRI) is the most preferred imaging modality for the diagnosis of ARVD/C. However, many patients with suspected ARVD/C have implantable cardioverter-defibrillators, prohibiting the use of MRI. Thirty-one patients (19 men; mean age 41 +/- 12 years) referred for evaluation of known or suspected ARVD/C had a complete reevaluation including contrast-enhanced cardiac MDCT at the center. Two patients underwent both cardiac MRI and MDCT. Seventeen of 31 patients met Task Force criteria for ARVD/C and were confirmed to have ARVD/C. Multidetector computed tomographic images were analyzed for qualitative and quantitative characteristic findings of ARVD/C. Increased RV trabeculation (p <0.001), RV intramyocardial fat (p <0.001), and scalloping (p <0.001) were significantly associated with the final diagnosis of ARVD/C. RV volumes, RV inlet dimensions, and RV outflow tract surface area were increased in patients with ARVD/C compared with patients who did not meet the criteria. RV and left ventricular functional analysis was performed in 2 patients. In conclusion, cardiac MDCT has a strong potential to detect many qualitative and quantitative abnormalities of the right ventricle in patients with ARVD/C. Limitations include implantable cardioverter-defibrillators and motion artifacts, along with well-known radiation and contrast-induced reaction.     

Exercise-induced ST elevation in patients with arrhythmogenic right ventricular dysplasia

To test the hypothesis that local or diffuse wall motion abnormalities in the right ventricle in patients with arrhythmogenic right ventricular dysplasia (ARVD) may induce the ST-segment elevation in response to exercise, we examined exercise electrocardiograms in patients with ARVD. In 17 patients with ARVD, who demonstrated right ventricular wall motion abnormalities without organic coronary lesions, we conducted a treadmill exercise test. Significant exercise-induced ST-segment elevation (ESTE) was defined as a 0.1 mV or more ST-segment elevation at J point. ESTE was observed in 11 patients (65%). It manifested most frequently in right-sided precordial leads. Severe right ventricular asynergy was seen in all but one (91%) among 11 with ESTE, whereas it was seen only in two (33%) among six without ESTE (P<.05). The maximal magnitude of ESTE inversely correlated with right ventricular ejection fraction (r = -0.58, P<.05). ESTE was seen in two thirds of ARVD patients, helping us noninvasively diagnose ARVD. The fact that ventricular wall motion abnormalities could cause ESTE in the absence of organic coronary lesions suggested the critical role of mechanical factors in the genesis of ESTE.     

Arrhythmogenic right ventricular dysplasia/ cardiomyopathy

Optional statement Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). The most important aspect in the treatment of ARVD/C is establishing a correct diagnosis based on the International Task Force criteria. In our experience, cardiologists are not aware of these diagnostic criteria for ARVD/C and place too much importance on the results of magnetic resonance imaging of the RV. Patients with ARVD/C generally all have an abnormal 12-lead electrocardiogram, abnormal echocardiogram, and ventricular arrhythmias with a left bundle branch block morphology. If noninvasive testing suggests ARVD, invasive testing with an RV angiogram, RV biopsy, and electrophysiology study are recommended. We encourage patients to participate in the National Institutes of Health-sponsored multicenter clinical trial of ARVD/C (http://www.ARVD.com or http://www.ARVD.org). Once a diagnosis of ARVD/C is established, the main treatment decision involves whether to implant an implantable cardioverter-defibrillator (ICD). ICDs are recommended for patients who have experienced syncope, sudden death, or a sustained ventricular arrhythmia, and also for patients with overt evidence of ARVD, particularly if the electrophysiology study is abnormal or there is a family history of sudden death. We also recommend treatment of patients with ARVD/C with β blockers and angiotensin-converting enzyme inhibitors, and that all patients with ARVD/C be screened for a mutation in the gene for plakophilin-2, because this is present in more than one third of patients with ARVD/C and may be helpful in the management of firstdegree relatives.     

Colour tissue Doppler echocardiographic evaluation of right ventricular function in patients with right ventricular infarction

OBJECTIVE: This study was undertaken to determine right ventricular (RV) function as assessed by colour Doppler tissue imaging (DTI) in patients with RV infarction. METHODS: During the study period, 35 patients were evaluated: 14 patients had an inferior myocardial infarction (MI) with RV infarction and 21 patients had an inferior MI without RV involvement. Twenty age-matched healthy subjects served as controls. The diagnosis of RV infarction was defined by ST segment elevation >0.1 mV in lead V4R. Systolic and early and late diastolic velocities were acquired from the apical four-chamber view at the lateral tricuspid annulus, the septal side of the tricuspid annulus and the RV free mid-wall using colour DTI. RESULTS: Systolic and early diastolic velocities at the lateral tricuspid annulus were significantly reduced in patients with inferior MI with RV infarction compared with those in healthy individuals (7.8 +/- 1 vs. 11 +/- 2 cm/s, p < 0.002) and patients with inferior MI without RV infarction (7.8 +/- 1 vs. 10 +/- 1 cm/s, p < 0.002). The late diastolic lateral annular velocity did not differ between the groups. Systolic and early diastolic RV free wall velocities were also significantly decreased in patients with RV infarction compared with those in healthy individuals (7 +/- 1 vs. 8.7 +/- 1 cm/s, p < 0.01; 6.3 +/- 2 vs. 8.7 +/- 2 cm/s, p < 0.05, respectively) and patients with inferior MI without RV infarction (7 +/- 1 vs. 9 +/- 2 cm/s, p < 0.01; 6.3 +/- 2 vs. 8.3 +/- 2 cm/s, p < 0.05, respectively). CONCLUSION: The evaluation of tricuspid annular and RV free wall velocities using colour DTI provides a rapid and noninvasive tool for assessing RV function in patients with RV infarction.     

Do patients with right ventricular outflow tract ventricular arrhythmias have a normal right ventricular wall motion? A quantitative analysis compared to normal subjects

BACKGROUND/AIM: Patients with ventricular ectopy from the right ventricular (RV) outflow tract (RVOT) are often referred for RV angiography to exclude disorders such as arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). This is usually based on a qualitative assessment of the wall motion. We present a method to quantify the wall motion and to apply this method to compare patients with RVOT ectopy to normal subjects. METHODS: RV angiograms were analyzed from 19 normal subjects and 11 subjects with RVOT ventricular arrhythmias (RVOT arrhythmia subjects) who had no other clinical or other evidence for ARVC/D. By a newly developed computer-based method, RV contours were first traced from multiple frames spanning the entire cardiac cycle. The fractional change in area between contours was then calculated as a serial function of time and location to determine both total contour area change and timing of contour movement. Contour area strain, defined as the differential change in area between nearby regions, was also computed. RESULTS: The contour area change was greatest in the tricuspid valve region and least in the RVOT and midanterior regions. The onset of contraction was earliest in the RVOT region and latest in the apical, inferior, inferoapical, and subtricuspid valve regions. The contour strain was largest in superior tricuspid valve and inferior wall and near zero within the lateral tricuspid valve region. There were significant pairwise differences in contraction area, timing, and strain in the various regions. There were no significant differences between normal subjects and RVOT arrhythmia subjects. CONCLUSIONS: The RV wall motion is nonuniform in contour area change, strain, and timing of motion. Patients with RVOT ventricular ectopy demonstrate wall motion parameters similar to those of normal subjects. This technique should be applicable in analyzing RV wall motion in patients suspected of having ARVC/D.     

单纯急性下壁心肌梗死及其合并右心室心肌梗死对右心长轴功能的影响

目的应用心肌组织多普勒技术结合M型超声心动图,评价单纯急性下壁心肌梗死及其合并右心室心肌梗死对右心室长轴功能的影响。方法选择首次急性下壁心肌梗死患者28例,分为单纯急性下壁心肌梗死18例(Ⅰ组),急性下壁心肌梗死伴右心室心肌梗死10例(Ⅱ组),另选健康体检者20例(Ⅲ组)。在标准心尖四腔心切面二维图像指引下,应用M型超声记录右心房室环右心室游离壁及中心纤维支架处运动曲线,测量收缩期、舒张早期、舒张晚期最大运动幅度(SD,DED,DAD)及收缩期、舒张早期、舒张晚期平均运动速度(SMV,DEMV,DAMV),计算DED/DAD比值。心肌组织多普勒记录该处运动速度曲线,测量上述各期最大运动速度(Sm、Em、Am)及Em/Am比值。结果与Ⅲ组比较,Ⅰ组和Ⅱ组右心房室环右心室游离壁处SD、DED、DED/DAD、SMV、DEMV、Sm及Em均明显下降。DAD、DAMV、Am虽有下降,但差异无统计学意义。结论急性下壁心肌梗死无论是否合并右心室心肌梗死均可影响右心室长轴功能,导致右心室整体功能降低。     

Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia: sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle.

OBJECTIVES: The purpose of this study was to determine interobserver agreement for interpretation of magnetic resonance imaging (MRI) examinations of arrhythmogenic right ventricular dysplasia (ARVD) and to determine sensitivity and specificity of fat detection versus functional parameters measured by MRI. BACKGROUND: The interobserver variability of MRI and the relative importance of different MRI parameters (fat detection, regional and global right ventricular [RV] function) for ARVD diagnosis is unknown. METHODS: Two experienced observers blinded to the clinical history independently analyzed MRI datasets obtained from 40 patients evaluated for ARVD. Twenty normal subjects underwent MRI and served as control subjects. The MRI scans were performed according to a standard protocol on a 1.5-T scanner. The observers reported on fat infiltration, global and regional RV function, myocardial thinning, and chamber dilatation qualitatively. The RV volumes were measured on the cine sequences. RESULTS: Interobserver kappa scores for fat infiltration, global and regional RV function, wall thinning, and RV outflow dilatation were 0.74, 0.94, 0.89, 0.93, and 0.93, respectively. Correlation coefficients between observers for RV end-diastolic volume, end-systolic volume, and ejection fraction were 0.93, 0.94, and 0.95, respectively (p < 0.001). Fifteen patients were diagnosed with ARVD using Task Force criteria. Sensitivity of fat infiltration, RV enlargement, and regional RV dysfunction for diagnosing ARVD was 84%, 68%, and 78%, and specificity was 79%, 96%, and 94%, respectively. CONCLUSIONS: Qualitative assessment of RV structure and function is highly reproducible for experienced observers. Among the qualitative parameters, fat infiltration is less reproducible and lacks specificity compared with RV kinetic abnormalities.     

Histologic findings in patients with clinical and instrumental diagnosis of sporadic arrhythmogenic right ventricular dysplasia

OBJECTIVES: We sought to analyze the histologic findings of 30 patients with a diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) based on established clinical and instrumental criteria, who did not have a family history of ARVD. BACKGROUND: The diagnostic role of endomyocardial biopsy (EMB) in patients with a clinical profile of ARVD is still debated. METHODS: Thirty patients (19 male, 11 female, mean age 27 +/- 10 years) with left bundle branch block morphology ventricular tachyarrhythmias and echocardiographic, angiographic, and magnetic resonance imaging (MRI) findings diagnostic of ARVD were studied. All patients, besides diagnostic, noninvasive, and invasive cardiac studies, underwent EMB in the apex, anterior free wall, inferior wall of the right ventricle (RV) and in the septal-apical region of the left ventricle. RESULTS: Diagnostic histologic features of ARVD were found only in 9 (30%) patients and a myocarditis, according to the Dallas criteria, in the remaining 21 (70%) patients. Morphometric evaluation of RV samples showed significant differences in fatty tissue and myocyte percent area between ARVD and myocarditis (p < 0.001). Conversely, no difference was found between the two groups in arrhythmic patterns and structural and functional echocardiographic, angiographic, and MRI RV alterations. Magnetic resonance imaging showed hyperintense signals in 67% of ARVD and in 62% of myocarditis group (p = NS). During follow-up (mean, 23 +/- 14 months), all patients with myocarditis remained stable on antiarrhythmic therapy while five patients with ARVD required implantation of an implantable cardioverter defibrillator. CONCLUSIONS: A myocarditis involving the RV can mimic ARVD. An EMB appears the most reliable diagnostic technique, with significant prognostic and therapeutic implications.     

Automatic backscatter analysis of regional right ventricular systolic function using colour kinesis in patients with inferior wall acute myocardial infarction with or without right ventricular involvement

BACKGROUND: In patients with inferior acute myocardial infarction (AMI), right ventricular (RV) function is an important determinant of global cardiac performance, prognosis, and exercise capacity. Several echocardiographic methods for quantifying RV function have been developed over the years but the usefulness of colour kinesis (CK) and acoustic quantification (AQ) have not yet been investigated. AIM: To test whether AQ and CK may provide quantitative assessment of global and regional RV function in patients with inferior AMI. METHODS: Thirty two consecutive patients with recent inferior AMI with or without RV involvement (n=17 and n=15, respectively), and 15 age- and gender-matched controls were studied. The graphs of RV fractional area change were displayed along with ECG and the concurrent cross sectional image. CK digitised end-systolic images of RV and were evaluated by reviewing the stored loops obtained from normal subjects and patients. To evaluate the entire RV systolic endocardial excursion, further quantitative CK analysis was performed by measuring the systolic segmental endocardial motion (SEM). RESULTS: In comparison with the control group, patients with inferior AMI with or without RV involvement had reduced RV fractional area change (30+/-7%, 36+/-6%,45+/-6%, p<0.05, p<0.01 respectively), reduced mean free wall SEM (3.9+/-1.1 mm, 5.2+/-1.3 mm, 6.3+/-1.4 mm, p<0.05, p<0.01 respectively) and mean septal wall SEM (4.9+/-1.2 mm, 6.4+/-1.5 mm, 7.2+/-1.4 mm, p<0.05, p<0.05, respectively). CONCLUSIONS: Our results confirmed that RV systolic functions are significantly more altered in patients with inferior AMI than in controls, and that RV abnormalities are more pronounced in patients with rather than without RV involvement. AQ and CK are able to detect wall motion disturbances in patients with inferior AMI with RV involvement.     

Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia

Introduction: Case studies indicate that cardiac sarcoid may mimic the clinical presentation of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, the incidence and clinical predictors to diagnose cardiac sarcoid in patients who meet International Task Force criteria for ARVD/C are unknown.
Methods and Results: Patients referred for evaluation of left bundle branch block (LBBB)-type ventricular arrhythmia and suspected ARVD/C were prospectively evaluated by a standardized protocol including right ventricle (RV) cineangiography-guided myocardial biopsy. Sixteen patients had definite ARVD/C and four had probable ARVD/C. Three patients were found to have noncaseating granulomas on biopsy consistent with sarcoid. Age, systemic symptoms, findings on chest X-ray or magnetic resonance imaging (MRI), type of ventricular arrhythmia, RV function, ECG abnormalities, and the presence or duration of late potentials did not discriminate between sarcoid and ARVD/C. Left ventricular dysfunction (ejection fraction <50%) was present in 3/3 patients with cardiac sarcoid, but only 2/17 remaining patients with definite or probable ARVD/C (P = 0.01).
Conclusions: In this prospective study of consecutive patients with suspected ARVD/C evaluated by a standard protocol including biopsy, the incidence of cardiac sarcoid was surprisingly high (15%). Clinical features, with the exception of left ventricular dysfunction and histological findings, did not discriminate between the two entities.     

Right ventricular function in patients with first inferior myocardial infarction: assessment by tricuspid annular motion and tricuspid annular velocity

BACKGROUND: Unlike left ventricular function, right ventricular (RV) function has not been widely studied after a myocardial infarction (MI). The current study describes RV function determined by tricuspid annular motion and tricuspid annular velocity after MI. METHODS AND RESULTS: Thirty-eight patients with a first acute inferior MI were prospectively compared with 33 patients with a first anterior MI and 24 age-matched healthy individuals. Association of RV infarction in inferior MI was defined as the presence of >/=1-mm ST-segment elevation at the right precordial lead, V(4)R, of the electrocardiograms. From the echocardiographic apical 4-chamber views, the systolic motion of the tricuspid annulus was recorded at the RV free wall with the use of 2-dimensional guided M-mode recordings. Peak systolic and peak early and late diastolic velocities of the tricuspid annulus at the RV free wall also were recorded with the use of pulsed-wave Doppler tissue imaging. The tricuspid annular motion was reduced in inferior MI compared with that in healthy individuals (20.5 and 25 mm, P <.001). The peak systolic velocity of the tricuspid annulus was significantly reduced in inferior MI compared with that in healthy individuals (12 vs 14.5 cm/s, P <.001) and patients with anterior MI (12 and 14.5 cm/s, P <.001). Patients with inferior MI were divided into 2 subgroups: those with and those without electrocardiographic signs of RV infarction. The tricuspid annular motion was significantly lower in patients with RV infarction than in patients without RV infarction (17 and 22.7 mm, P <.001). In addition, compared with patients without electrocardiographic signs of RV infarction, patients with RV infarction also had a significantly decreased peak systolic tricuspid annular velocity (13.3 and 10.3 cm/s, P <.001) and peak early diastolic velocity (13 and 8.2 cm/s, P <.001). CONCLUSIONS: These results suggest that tricuspid annular motion and tricuspid annular velocity can be used to assess RV function in association with inferior MI.     

Structural and functional assessment of arrhythmogenic right ventricular dysplasia/cardiomyopathy by multi-slice computed tomography: comparison with cardiovascular magnetic resonance

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an uncommon inheritable cardiomyopathy involving predominant right ventricle with progressive fibrofatty tissue replacement. An integrated assessment of electrical, functional and anatomic abnormalities, in addition to personal and family history would be used to diagnose this disease entity. We present the case of a 69-year-old man with a history of sustained ventricular tachycardia. Fatty infiltration and regional wall motion abnormalities over biventricular myocardium were clearly demonstrated by cardiac 64-slice computed tomography (CT), as consistent with magnetic resonance imaging. Thus, multi-slice CT may have a significant role in the assessment and follow-up of patients with ARVD/C by providing excellent structural, functional assessment and tissue characterization.