超声心动图对致心律失常性右室心肌病的诊断评价

目的分析超声心动图诊断致心律失常右室心肌病（ARVC）的图像特点。方法对5例ARVC患者的临床资料及超声心动图检查结果进行分析。结果5例ARVC患者中4例以室性心律失常发病,1例为家系检查发现。1例猝死、1例心力衰竭、2例晕厥、1例无症状。所有患者均做了心电图、超声心动图检查,部分患者做了心室核素造影和右心室造影。超声心动图表现为：所有患者均右心室扩大,3例右室流出道扩张,1例左室稍大;3例右室壁运动弥漫性减弱,1例局限性减弱,1例右室壁瘤形成,2例室间隔运动异常。5例患者三尖瓣、肺动脉瓣均有反流,反流压差较低,前向流速减低。结论ARVC超声心动图的表现为右心室扩大、右室流出道扩张、右室壁弥漫性、局限性变薄、运动异常。患者以反复发作性室性心动过速、晕厥、猝死为首发症状,在除外其他可引起右室扩大的病变后可提示本病。     

新型磁共振对典型及早期致心律失常性右室心肌病诊断价值的探讨

目的 用有黑血技术的新型磁共振（MRI）对典型致心律失常性右室心肌病（ARVC）进行检查，以确定新型MRI诊断ARVC的特异性和敏感性，并通过对确诊的ARVC患的一级亲属行MRI检查，以探讨MRI对早期ARVC的诊断价值。方法 10例ARVC患（除1例猝死首诊外）及其7个家系的54名成员全部接受询问病史，体检，心电图，心脏超声等检查；10例临床患均接受MRI检查，分析和确定其影响特征及诊断条件，在此基础上对部分家系成员行MRI检查以发现早期ARVC患。结果 临床患有阵发性室性心动过速（8／8），晕厥（9／10），心力衰竭（3／10）和猝死（3／10）。心电图均有左束支传导阻滞型阵发性室性心动过速，心室晚电位（VLP）均阳性（8／8）。MRI检查显示临床患均有明显右心室（RV）扩大及室壁广泛强信号，经压脂处理后心肌信号呈岛状或连续中断，为特征性纤维脂肪替代影像，患均有RV运动减低或室壁瘤形成，部分伴左心室受累（3／8）。家系筛选发现8例异常，拟诊为早期ARVC，2例有心电图异常，2例VLP阳性。MRI显示，8例心室壁均有局限性纤维脂肪病的影像改变，4例有RV扩大，2例可疑扩大，6例RV心尖部血流淤滞现象。结论 带黑血技术的新型MRI是目前诊断ARVC和早期ARVC的最具特异性和敏感性的检查手段。     

彩色多普勒与其他检查方法诊断心尖肥厚型心肌病31例的比较

目的探讨彩色多普勒（CDFI）与其他检查方法对心尖肥厚型心肌病（AHCM）的诊断价值。方法对31例AHCM患者的CDFI、磁共振检查（MRI、冠脉造影及左心室造影（CAG＋LVG）、心电图（EGG）特点进行分析。结果31例均经CDFI检查,28例证实有心尖部心肌肥厚（厚度〉15mm）,心尖最大室壁厚度／基底部最大室壁厚度≥1．3。9例进行了LVG,其中有4例显示左室舒张期末心尖部心肌肥厚,左心室舒张末期呈“黑桃”（spade—like）样改变。4例行了MRI检查,均可见心尖部不对称性肥厚。全部患者均合并有ECG异常改变（100％）。31例中有1例合并有右室心尖肥厚。结论CDFI为AHCM最理想的检测方法,如结合以上其他检查,则可进一步提高诊断准确性。     

致心律失常性右室心肌病一例

患者男,56岁,主因＂阵发性心悸伴胸闷、气短2年＂收住入院。心悸发作时心电图示室性心动过速（简称室速）。心脏彩超示右房右室内径增大,右室流出道内径增宽。心脏核磁共振示右室室壁信号不均匀,呈高、等、低混杂信号改变,心室游离壁及下壁变薄,右室流出道扩张,右室室壁运动减弱,左室如常,符合致心律失常性右室心肌病。给予埋藏式心脏转复除颤器治疗,术后抗心动过速起搏治疗1次。     

Hoffmayer心电图积分识别ARVC室早／室速

目的探讨应用Hoffmayer心电图积分鉴别致心律失常性右室心肌病（arrhvthmogenic right ventricular cardiomyopathy,ARVC）和特发f生右室流出道室早／室速的临床意义。方法收集2009年9月至2013年5月就诊于北京大学人民医院患者中,心电图表现为右室流出道起源室早／室速患者57例。其中明确诊断为ARVC患者4例,经电生理检查及射频消融治疗成功的特发性右室流出道室早／室速患者53例。由两位电生理医生在不知道确切诊断的情况下,依据Hoffmayer心电图积分对上述患者的心电图进行分析,计算总积分≥5分,各单项积分诊断ARVC伴发的室早／室速的敏感度、特异度、阳性预测值、阴性预测值及诊断符合率。结果Hoffmayer心电图积分≥5分诊断ARVC伴发室早／室速的敏感度75％,特异度96．23％,阳性预测值60％,阴性预测值98％、诊断符合率94．7％。结论Hoffmayer积分≥5可有效鉴别右室流出道室早／室速是ARVC伴发的还是特发性的。应用此项积分具有简单、快速、敏感度及特异度均较高的优点,具较高的临床应用价值。     

磁共振成像在评价心肌病变中的临床应用价值

目的分析心肌病变的磁共振影像学特征,评价磁共振成像在心肌疾病中的应用价值。方法回顾性分析疑似心肌疾病病人21例,所有病人最终均经临床或病理确诊。使用心脏八通道相控阵表面线圈,回顾性心电门控技术与呼吸门控技术,均行磁共振T2平扫、电影扫描、首过灌注、延迟强化扫描。结果 4例肥厚型心肌病病人表现为心肌舒张顺应性减低,首过灌注肥厚心肌区域灌注减低,延迟强化肥厚心肌内可见斑片状云絮状强化,3例心尖肥厚型心肌病肥厚心肌区域未见延迟强化病灶。2例高血压继发代偿性心脏改变的病人出现左室壁对称性肥厚,室壁增厚率正常,左室腔不大,左室壁未见灌注缺损及延迟强化征象。5例扩张型心肌病中3例出现左室增大,室壁变薄,心肌收缩功能减低,延迟强化心肌肌壁间出现线状、环形强化灶,2例病人未出现延迟强化灶。3例心肌梗死病人出现与冠状动脉造影相符合的心室壁节段性运动异常、首过灌注缺损及节段性心内膜下延迟强化或心尖部透壁强化。1例致心律失常性右室型心肌病病人出现右室增大,右室壁变薄及右室壁的弥漫性延迟强化。1例心肌致密化不全病人出现左室心尖及游离壁的心肌局部变薄、运动减弱,致密化不全心肌呈栅栏状,未见出现灌注缺损及延迟强化病灶。1例心肌淀粉样变性病人出现心肌弥漫性增厚积累及心肌全层的弥漫性粉尘样延迟强化,1例心肌炎病人出现室间隔的早期灌注及延迟强化病灶。结论心脏磁共振成像具有无电离辐射、软组织对比度好的特点,尤其是其在不同心肌病变时的特征性延迟强化表现,可以作为心肌疾病诊断的重要影像学检查方法。     

致心律失常性右室心肌病的临床研究和家系调查

目的 对7例致心律失常性右室心肌病(ARVC)及其中的3个家系中的34个成员进行调查。方法病史、临床检查、标准12导联心电图、超声心动图和心室晚电位测定。5例进行电生理检查。结果 超声心动图发现5例右室扩大伴搏动减弱，8例右室前壁局限性运动减弱或收缩期膨出，1例下壁局限性变薄和收缩期膨出。心电图显示完全性和不完全右束支阻滞样改变各5例，肢体导联低电压2例，23例右胸(V1-V4)和／或下壁(Ⅱ、Ⅲ、aVF)导联T波倒置，25例V1导联QRS时间明显延长(P<0．01)．13例心室晚电位阳性。电生理检查，5例患者可诱发≥2种的呈左束支阻滞图形室性心动过速(室速)，2例诱发心室颤动(室颤)；右室一处或多处起搏阈值增高或不能有效起搏。1家系2例猝死。1例ARVC合并侏儒症。5例患者反复发作呈左束支阻滞图形的室性心动过速，1例伴有配对间期极短的多形性室速和反复晕厥发作，12例有呈左束支阻滞图形室性早搏。结论 本研究结果提示ARVC是一遗传性疾病．右室结构与功能异常、心电图复极异常和起源于右室室性心律失常是其最常见的表现。     

二维超声室壁运动异常与冠脉病变范围的关联性

目的 :分析心肌梗死患者二维超声室壁运动异常节段分布与冠状动脉损伤间的关系。方法 :回顾性分析 92例心梗患者二维超声室壁运动异常节段分布情况 ,并与冠状动脉病变范围进行比较。结果 :1冠状动脉梗塞后左前降支 （L AD）受累最为多见 （45 % ） ;2 L AD单支病变与前壁、前间隔及心尖部室壁运动异常有关 （P<0 .0 5 ）。 L AD与左回旋支病变 ,主要为前间隔和下壁运动异常 ;而单纯的下壁室壁运动异常多为左旋支与右冠状动脉病变引起。 3支病变造成心肌运动普遍减低 ,且以侧壁及前侧壁的室壁运动异常则更为显著 （P<0 .0 5 ）。结论 :二维超声心动图室壁运动异常与冠脉病变范围有较好的关联性。     

右心室心尖部憩室引起室性心动过速一例

致心律失常性右室心肌病以心肌不同程度地被脂肪或纤维脂肪组织代替为特征，主要累及右室流出道、心尖部受后下壁的心肌，本报道1例致心律失常性右室心肌病由右心室心尖部小憩室引起室性心动过速（VT）的病例,并对诊治过程进行分析。     

应用定量组织速度成像评价原发性高血压患者左室局部心肌功能

目的: 应用定量组织速度成像技术评价原发性高血压（EH）患者心脏的左室局部心肌功能。方法: 将40例EH患者按左室心肌质量指数分为:左室肥厚(LVH)组（14例）和非左室肥厚（NLVH）组（26例）,另30例正常同龄人为正常对照组。获得心尖四腔、两腔及心尖左室长轴切面各室壁的心肌组织速度曲线,测定收缩期、舒张早期、房缩期的平均峰值速度 (VS、VE、VA) 及VE/VA。结果: VS:NLVH组及LVH组各室壁均与对照组无显著性差异;VE :NLVH组后间隔、左室后壁、前间隔、下壁较对照组显著降低 (P<0.05),LVH组除左室侧壁外其余室壁较对照组显著降低(P<0.05) 。VA:NLVH组后间隔与对照组有显著性差异,LVH组各室壁均与对照组有显著性差异(P<0.05)。VE/VA:NLVH组除左室侧壁外其余室壁均较对照组显著降低 (P<0.05)。LVH组各室壁均与对照组有显著性差异(P<0.05)。结论: 定量组织速度成像能够客观、准确的评价EH患者的局部心肌功能。     

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.     

Carvedilol therapy improved left ventricular function in a patient with arrhythmogenic right ventricular cardiomyopathy

An asymptomatic 35 year-old man was referred to our hospital because of abnormal ECG findings. The ECG showed complete right bundle branch block and left anterior hemiblock. Echocardiography revealed a moderately enlarged right ventricle (RV) and an apical aneurysm. RV wall motion showed diffusely moderate impairment, while the systolic function of the left ventricle (LV) was slightly decreased. The ejection fractions (EF) of the RV and LV were calculated as 28.1% and 41.9% by Simpson's method using multiple cardiac computed tomography (CT) scans. A 24 hour ambulatory ECG showed only 372 single premature ventricular contractions (PVC). Cardiac catheterizaion revealed that the RV was enlarged with prominent trabeculation and decreased motion. In an electrophysiologic study, neither electrical stimulation of the RV nor electrical stimulation plus isoproterenol infusion could induce ventricular tachycardia. Pathological examination of a biopsy from the interventricular septum of the RV revealed fibrofatty change in the myocardium. Based on these results, we made a diagnosis of arrhythmogenic right ventriclular cardiomyopathy (ARVC) and administered 5 mg of carvedilol. Sixty days after the initiation of carvedilol therapy, we performed repeat cardiac CT. The EF of the LV was markedly improved from 41.9% to 62.0%, although the EF of the RV was not changed. The number of PVCs showed no change. This case suggests that carvedilol is not only useful for controlling arrhythmia but also for improving left ventriclular function in some patients with ARVC. Sympathetic overactivity is reported to cause sudden death, so carvedilol may be a first-line drug for some patients with ARVC.     

Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy

Diagnostic sensitivity and specificity of cineangiography were evaluated by multivariate logistic discriminant analysis in 32 patients with arrhythmogenic right ventricular (RV) cardiomyopathy, 27 patients with biventricular dilated cardiomyopathy, 28 patients with atrial septal defect and 18 normal subjects. In patients with arrhythmogenic RV cardiomyopathy and biventricular dilated cardiomyopathy, the diagnosis was confirmed by endomyocardial biopsy. All RV values overlapped for the diagnosis of atrial septal defect and arrhythmogenic RV cardiomyopathy; overlapping extended to dilated cardiomyopathy for end-diastolic volume and infundibular dimensions. RV ejection fraction appeared reduced in all the diseases; in particular, mean values in dilated cardiomyopathy and arrhythmogenic RV cardiomyopathy were 38 and 53%, respectively (p less than 0.05). Left ventricular quantitative studies showed a significant difference between dilated and arrhythmogenic RV cardiomyopathy, both in terms of pumping indexes (mean end-diastolic volumes 180 vs 91 ml/m2 and mean ejection fraction 33 vs 60%), and indexes of contractility (stress/end-diastolic volume 3.7 vs 6.7). Multivariate analysis disclosed that transversally arranged hypertrophic trabeculae, separated by deep fissures, were associated with the highest probability of arrhythmogenic RV cardiomyopathy (p less than 0.001). Posterior subtricuspid and anterior infundibular wall bulgings were the only other independently significant variables. Coexistence of these signs was associated with 96% specificity and 87.5% sensitivity. Thus, arrhythmogenic RV cardiomyopathy presents quantitative volumetric and hemodynamic as well as qualitative features that clearly distinguish it from dilated cardiomyopathy and confirm its nosographic autonomy among the primary diseases of the myocardium.     

Measurement of right ventricular mass in normal and dilated cardiomyopathic ventricles using cine magnetic resonance imaging.

The accurate quantification of right ventricular (RV) mass has eluded conventional imaging modalities. Accordingly, cine magnetic resonance imaging was used for quantification of RV as well as left ventricular (LV) mass in 10 normal subjects and in 10 patients with dilated cardiomyopathy with an LV ejection fraction less than 0.40. Hearts were imaged with 10 mm thick short-axis slices from apex to base with a short echo delay time of 5 ms. Each slice was partitioned into 3 sections: RV free wall, ventricular septum and LV free wall, for calculation of end-diastolic and end-systolic mass and LV:RV free wall ratio. RV end-diastolic mass in normal subjects was 45 +/- 8 g, which was similar to the values determined in previously published postmortem studies, mean 46 g (range 23 to 68). The value determined in patients with dilated cardiomyopathy was higher (50 +/- 11 g), but this difference was not significant. LV:RV free wall ratio in cardiomyopathy (3.6 +/- 1.0) was greater than in normal subjects (2.4 +/- 0.3), because of the greater LV free wall mass in dilated cardiomyopathy, where LV free wall end-diastolic mass was 173 +/- 40 g vs 107.1 +/- 19.9 g in normal subjects (p less than 0.05). RV mass measurements had 6.4 +/- 3.6% interobserver and 7.3 +/- 6.1% intraobserver variability. There were no significant differences between end-diastolic and end-systolic mass measurements. Thus, cine magnetic resonance imaging can reproducibly calculate RV mass. The values in normal subjects correspond to previously reported postmortem values for a population without heart disease.     

Right ventricular scar‐related ventricular tachycardia in nonischemic cardiomyopathy: Electrophysiological characteristics,mapping, and ablation of underlying heart disease

1 Background

Right ventricular (RV)‐scar related ventricular tachycardia (VT) is often due to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) or cardiac sarcoidosis (CS), but some patients whose clinical course has not been described do not fulfill diagnostic criteria for these diseases. We sought to characterize the electrophysiologic substrate and catheter ablation outcomes of such patients, termed RV cardiomyopathy of unknown source (RCUS).

2 Methods and results

Data of 100 consecutive patients who presented with RV cardiomyopathy and/or RV‐related VT for ablation were reviewed (51 ARVC/D, 22 CS; 27 RCUS). Compared to ARVC/D, RCUS patients were older (P = 0.001), less commonly had RV dilatation (P = 0.001) or dysfunction (P = 0.01) and fragmented QRS, parietal block, and T‐wave inversion. Compared to CS, R‐CUS patients had less severe LV dysfunction. Extent and distribution of endocardial/epicardial scar and inducible VTs in RCUS patients were comparable with ARVC/D and CS patients. At a median follow‐up of 23 months, RCUS patients had more favorable VT‐free survival (RCUS 71%, ARVC/D 60%, CS 41%, P = 0.03) and survival free of death or cardiac transplant (RCUS 92%, ARVC/D 92%, CS 62%, P = 0.01). No RCUS patients developed new criteria for ARVC/D or CS in follow‐up.

3 Conclusions

Up to one‐third of patients with RV scar‐related VT are not classifiable as ARVC/D or CS. These patients had a somewhat better prognosis than ARVC/D or sarcoid and did not develop evidence of these diseases during the initial 2 years of follow‐up. The extent to which this population comprises mild ARVC/D, CS, or other diseases is not clear.     

Clinical profile of arrhythmogenic right ventricular cardiomyopathy in Chinese patients.

OBJECTIVE: To study the clinical profile of Chinese patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). PATIENTS: Chinese patients who fulfilled the diagnostic criteria of ARVC proposed by the Task Force of the European Society of Cardiology and of the scientific council on cardiomyopathy of the International Society and Federation of Cardiology were recruited for analysis. METHODS: Clinical data of patients with ARVC including age, sex, family history, presenting symptoms, electrocardiograph (ECG), echocardiography, cardiac catheterization, magnetic resonance imaging (MRI), electrophysiology study (EPS) and therapeutic intervention were analyzed. RESULTS: Eleven patients (seven males) were diagnosed with ARVC. Mean age at clinical presentation was 42.6+/-14.8 years. Two patients (18.1%) had positive family history of ARVC or premature sudden cardiac death. The commonest presenting symptoms were palpitation (73%) and dizziness (46%). Spontaneous ventricular tachycardia (VT) was the presenting arrhythmia in 54% and 1 (9%) with ventricular fibrillation and cardiac arrest. Seven patients (64%) had the ECG abnormality as defined by the Task Force. Echocardiography showed right ventricular (RV) dilatation in five patients (46%) and all patients had normal left ventricular function. Nine patients (90%) had RV wall thinning or fibrofatty replacement on MRI examination. Inducible monomorphic VT was detected in four out of nine patients at EPS. All eight patients had normal coronary arteries and left ventriculogram but RV dilatation and global hypokinesia was seen in three patients. Implantable cardioverter defibrillators were implanted in five patients and two of them had shocks delivered during the follow-up period. CONCLUSION: In this study, familial incidence of premature sudden death in patients with ARVC appears to be low and left ventricular involvement in affected individuals is uncommon. MRI is still the best investigation for ARVC.     

Relative Utility of Magnetic Resonance Imaging and Right Ventricular Angiography to Diagnose Arrhythmogenic Right Ventricular Cardiomyopathy

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of the RV myocardium. Two imaging techniques used to assess patients suspected of having ARVC are magnetic resonance imaging (MRI) and right ventricular angiography (RVA). Traditionally, RVA has played a central role in the diagnosis of ARVC, but the non-invasive nature of MRI and its unique ability to detect fatty tissue infiltration has increased its popularity as a diagnostic tool. The objective of this study was to assess the relative diagnostic accuracy of MRI and RVA for ARVC. METHODS AND RESULTS: Seventeen patients (9 men, 8 women; ages 42 +/- 17 [range 16-78] years) with documented ventricular arrhythmias were investigated for ARVC. A positive diagnosis of ARVC was based on criteria set forth by the ISFC Working Group on Cardiomyopathies and Dysplasia. ECG-gated spin-echo and gradient-echo MR images in multiple planes and RAO/LAO RV angiograms were compared for diagnostic concordance. Based on working group criteria, 7 patients were diagnosed with ARVC. In ten patients, MRI suggested ARVC. The remaining 7 patients had no MRI findings suggestive of the disease. Four patients with MRI findings of ARVC were incorrectly diagnosed based on Task Force criteria. Conversely, 1 patient with a normal MRI met Task Force criteria for the diagnosis of ARVC. Based on RV angiograms, 7 patients had findings suggestive of ARVC. The 10 patients without AVRD (based on RVA) also did not meet the necessary criteria for diagnosis of ARVC using Task Force standards. RVA was 100% specific and 100% sensitive compared to MRI that was only 86% sensitive and 60% specific. MRI proved to be most reliable when the images demonstrated gross, lipomatous infiltration, evidenced by a large area of hyperintensity. When the results of MRI and RVA were congruent, the diagnosis was always accurate. CONCLUSION: RVA is more sensitive and specific to diagnose ARVC diagnosis than MRI, at least until MRI protocols are better developed. MRI results are most robust when indicators of ARVC are grossly apparent. False-positive diagnosis by MRI was primarily related to perceived motion abnormalities that were not seen by RVA. One of its greatest potential assets (fat detection) did not enhance diagnostic specificity.     

Prognostic Value of Magnetic Resonance Phenotype in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

BackgroundCardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered.ObjectivesThe authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations.MethodsA total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient’s characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered.ResultsCMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement.ConclusionsDifferent CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.     

Right atrial abnormalities in a patient with arrhythmogenic right ventricular cardiomyopathy without ventricular tachycardia

We describe a 59-year-old woman with sick sinus syndrome (SSS) and arrhythmogenic right ventricular cardiomyopathy (ARVC). Diagnosis of SSS was made because she had frequent episodes of sinus arrest with prolonged ventricular asystole. Cardiac images showed a dilated right atrium (RA) and a right ventricle (RV). Electroanatomical mapping of the RA showed extensive scarring with no recordable electrical potentials. Although she had frequent premature ventricular contractions, neither spontaneous ventricular tachycardia (VT) nor induced VT was observed. Microscopic examination of the RV indicated fibrofatty myocardium. Atrial arrhythmias associated with SSS may be the cause of symptoms in some cases of ARVC.