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.     

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.     

The additional value of gadolinium-enhanced MRI to standard assessment for cardiac involvement in patients with pulmonary sarcoidosis

AIM: To determine whether gadolinium-enhanced cardiac MRI (CMR) was of additional diagnostic value to standard assessment in patients with sarcoidosis who underwent evaluation for cardiac involvement. METHODS: We reviewed the findings in patients with pulmonary sarcoidosis who had been assessed with ECG, Doppler echocardiography, 201Tl scintigraphy, and CMR from 2002 to 2004. RESULTS: Of the 55 evaluated patients, standard evaluation diagnosed cardiac involvement in 13 patients while CMR diagnosed myocardial scarring (mean +/- SD, 2.5 +/- 1.9 segments) [all 6 patients] and impaired systolic left ventricular function (1 patient) in an additional 6 patients. The extent of delayed enhancement correlated with disease duration (p < 0.05), ventricular dimensions and function (p < 0.001), severity of mitral regurgitation (p < 0.05), and the presence of ventricular tachycardias (p < 0.001). Patients in whom cardiac involvement was diagnosed only with CMR had less myocardial scarring and functional impairment (p < 0.05) compared to patients with a diagnosis made by standard assessment. CONCLUSION: CMR provides an accurate estimation of the extent of cardiac involvement and may reveal signs of early infiltration that are not detected by standard assessment. The extent of late enhancement with gadolinium relates to the severity of cardiac involvement and may therefore have prognostic implications.     

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。     

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.     

Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia with high defibrillation threshold requiring subcutaneous shocking coil implantation

Cardiac involvement in patients with sarcoidosis has been reported in up to 25-39% of patients and is responsible for up to 85% of deaths attributed to the disease, often due to sudden cardiac death. An established diagnosis of cardiac sarcoidosis (CS) portends an ominous prognosis, with an estimated five year-survival of 44%. We report a case that was initially diagnosed as arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), but extra-cardiac biopsies later on were consistent with sarcoidosis and a final diagnosis of CS was made. The patient received an implantable cardioverter defibrillator (ICD) with a subcutaneous lead array implant for high defibrillation threshold (DFT). Exclusive right ventricular (RV) involvement is atypical for CS. The predominant RV involvement based on echocardiogram, cardiac magnetic resonance imaging (MRI) and right precordial electrocardiogram changes can lead to misdiagnosis as ARVD/C based on the modified task force criteria. Cardiac sarcoidosis is an under-diagnosed disease and the delay in its diagnosis and appropriate therapy can lead to a fatal outcome. High defibrillation thresholds have not been previously reported in patients with CS, but given the natural progression of the disease and the limitations in current pharmacotherapy, implanters who diagnose and treat such patients must be prepared to deal with this issue.     

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.     

The other side of arrhythmogenic right ventricular cardiomyopathy

Knowledge regarding arrhythmogenic right ventricular cardiomyopathy (ARVC) has increased dramatically since the publication of the original International Task Force diagnostic criteria in 1994. Based on symptomatic index cases and sudden cardiac death victims, the Task Force criteria emphasized manifestations observed only at the most severe end of the clinical spectrum of ARVC. It is now known that the phenotypic expression of the disease may vary considerably both in its severity and in the pattern of myocardial involvement. Recent familial studies have emphasized that, despite its name, the abnormalities of ARVC are not limited to the right ventricle. In recognition of the prevalence and clinical significance of left ventricular involvement in ARVC, a proposed modification to the original International Task Force criteria was recently published. We herein describe 3 cases of patients with ARVC who presented with left ventricular involvement as manifested by nonischemic pattern of delayed enhancement in the left ventricle.     

Cardiac magnetic resonance imaging in the evaluation of cardiac sarcoidosis: an Australian single-centre experience

Background:  Cardiac involvement in systemic sarcoidosis is common; however, current diagnostic tools are imprecise. Recognition of cardiac sarcoidosis (CS) is important as it has a relatively poor prognosis. Gadolinium-enhanced cardiac magnetic resonance imaging (Gad-CMR) is emerging as an excellent technique in determining the presence of and extent to which cardiac muscle is affected by sarcoidosis.
Methods:  A retrospective analysis was performed on all patients with biopsy-proven systemic sarcoidosis referred for Gad-CMR scanning to evaluate potential cardiac involvement. All patients also underwent an electrocardiogram, Holter monitor and echocardiography. Gallium-67 radionuclide investigation, positron emission tomography and cardiac biopsy were ordered at the discretion of the treating physician.
Results:  Eleven of the 20 patients had Gad-CMR images supportive of the diagnosis of CS. Eight of these 11 patients met the Japanese Ministry of Health and Welfare (JMHW) criteria for the diagnosis of CS; three abnormal Gad-CME scans consistent with diagnosis of CS were seen in patients who did not meet JMHW criteria. No patients with normal Gad-CMR scan met JMHW criteria for CS.
Conclusion:  These findings suggest that Gad-CMR is potentially superior to the JMHW criteria in the diagnosis of cardiac sarcoidosis.     

Diagnosis of cardiac sarcoidosis and evaluation of the effects of steroid therapy by gadolinium-DTPA-enhanced magnetic resonance imaging

BACKGROUND: Cardiac involvement is an important prognostic factor in patients with sarcoidosis. In this study, we evaluated the usefulness of gadolinium-DTPA (diethylene triamine pentaacetic acid)-enhanced magnetic resonance imaging (Gd-MRI) for diagnosing cardiac sarcoidosis and evaluating the effects of steroid therapy.METHODS: Sixteen patients with sarcoidosis diagnosed by histology or by Japanese Ministry of Health and Welfare criteria for cardiac sarcoidosis underwent Gd-MRI with a 1.5-Tesla superconducting magnet system using a T1-weighted spin-echo sequence.RESULTS: Gd-MRI showed localized enhancement of signal intensity, indicating interstitial edema, in the left ventricle in 8 of the 16 patients. Two patients with enhancement also had thinning of the left ventricular septal wall. After 1 month of prednisolone therapy (60 mg every other day or 30 to 40 mg every day), the localized high-intensity signals were markedly diminished in all 8 patients.CONCLUSIONS: Images of the heart obtained by Gd-MRI may reflect active inflammation with interstitial edema in patients with sarcoidosis. Gd-MRI may be a useful noninvasive method for early detection of cardiac sarcoidosis and for evaluating the effects of steroid therapy.     

Determination of cardiac involvement in sarcoidosis by magnetic resonance imaging and Doppler echocardiography

OBJECTIVES: To elucidate whether cardiac magnetic resonance imaging (MRI) could be useful in disclosing structural changes in the myocardium in sarcoidosis patients and to relate echo-Doppler derived indices of left ventricular function to electrocardiogram (ECG) findings. DESIGN: The MRI was performed in 18 consecutive patients with sarcoidosis. Left ventricular ejection fraction (LVEF), i.e. systolic function, was estimated echocardiographically by Simpson's two-dimensional method (n = 16). Diastolic function was estimated by age-corrected Doppler-derived indices: isovolumetric relaxation time (IVRT), deceleration time (DT) and early filling/atrial contraction ratio (E/A ratio). RESULTS: Eleven patients had conduction defects or dysrhythmias (ECG+) whilst seven patients had a normal ECG (ECG-). In two patients, high signalling, contrast-enhanced, isolated regions, suggestive of deposits, were seen in the left ventricular myocardium on MRI. Both these patients had abnormal ECGs and signs of systolic and/or diastolic dysfunction on echocardiography. LVEF was subnormal in seven of 10 of the ECG+ patients and in two of six of the ECG-. Signs of diastolic dysfunction were found in 59% and 56% of the measurements in the ECG+ and ECG- patients, respectively. CONCLUSION: We conclude (i) that myocardial deposits on MRI in sarcoidosis patients have a high specificity for cardiac involvement but a rather low sensitivity; (ii) that a substantial proportion of sarcoidosis patients with abnormal ECGs have echocardiographic signs of systolic and/or diastolic dysfunction.     

Cardiac Sarcoidosis: A Clinical Overview

Cardiac sarcoidosis (CS) with clinical manifestation occurs in about 5-8% of patients with sarcoidosis. CS may be clinically suspected by the presence of ventricular arrhythmia, conduction abnormalities, and heart failure (HF). However, 20%-25% of patients may present with silent CS, having asymptomatic cardiac involvement. The diagnosis of CS is based on findings from nuclear studies, cardiac magnetic resonance, and extra-cardiac tissue biopsy. Due to the inflammatory nature of the disease, immunosuppressive medications are a cornerstone of therapy. The treatment also includes recommended HF medical therapies. Since CS patients are at risk of sudden cardiac death resulting from progression of cardiac dysfunction or the presence of scar originating from fatal arrhythmias, implantable cardioverter-defibrillators should be considered, with special indication beyond accepted recommendations in HF. In CS, the extent of left ventricular dysfunction is the most important mortality predictor. Heart transplant or mechanical circulatory support may represent life saving strategies in selective CS patients.     

Assessment of cardiac involvement in sarcoidosis by echocardiography

The main objective of this study is to determine the prevalence of left ventricular systolic and diastolic dysfunction in patients with chronic sarcoidosis without clinical evidence of heart disease. The study includes 69 chronic sarcoidosis patients, 30 diagnosed by organ biopsy and 39 by clinical history, chest X-ray, high resolution computerized tomography (HRCT) and bronchoalveolar lavage (BAL), without suspected cardiac involvement. The control group consisted of 26 subjects selected from a population of hospital workers. The examination includes 12-lead ECG and echocardiographic examination. The results show that there were no differences in atrial size, left ventricular diameters, wall thickness, left ventricular ejection fraction or endocardial fractional shortening between the sarcoid group and controls. Signs of diastolic dysfunction were found in 33 (55%) patients, however, this group was significantly older than the others and had marginally higher blood pressure. Sarcoid patients had lower midwall fractional shortening (mFS) than controls; patients with diastolic dysfunction also had lower mFS but the difference was not significant. In conclusion, the results demonstrated an absence of left ventricular systolic dysfunction, evaluated by traditional echocardiographic methods, in our chronic sarcoidosis patients and an apparent absence of any relation between left ventricular diastolic dysfunction and sarcoidosis. Lower mFS was found among patients, particularly those with a long history of sarcoidosis. Further analysis is required to evaluate the significance of this index as a potential marker of heart involvement in chronic sarcoidosis.     

Value of MRI for the diagnosis of cardiac involvement in sarcoidosis

PURPOSE: The aim of the study was to assess the place of cardiac Magnetic Resonance Imaging (MRI) in patients with sarcoidosis with or without cardiac involvement. MATERIALS AND METHODS: Fifty patients with histologically-proven sarcoidosis underwent initial cardiac evaluation including MRI, ECG, holter ECG, echocardiography. Seven of them had cardiac involvement (cardiac insufficiency, auriculo-ventricular block, bundle-branch block). Fiveteen patients had a second evaluation at 10-month follow-up. MRI was classified in three stages, on the base of literature data (stage 1 "granulomatous", stage 2 "exsudative", stage 3 "fibrotic"). RESULTS: A good correlation between the type of the sarcoidosis and MRI was observed: patients with cardiac involvement had all stage 2 MRI; patients with quiescent sarcoidosis had normal or stage 3 MRI; patients without cardiac involvement had all stages on MRI. A good correlation was observed between cardiac MRI abnormalities and evolution of sarcoidosis. Patients under corticoid, with or without cardiac involvement all had regression of MRI lesions and sarcoidosis. In 2 cases, MRI was predictive of clinical cardiac involvement. CONCLUSION: Cardiac MRI is a useful non-invasive method for the early diagnosis and follow-up of cardiac sarcoidosis.     

Diagnostic Utility of Signal‐Averaged Electrocardiography for Detection of Cardiac Sarcoidosis

Introduction: Cardiac sarcoidosis (CS) occurs in up to 25% of patients with pulmonary involvement. Early diagnosis is critical because sudden death from ventricular arrhythmias can be the initial presentation. We sought to evaluate the diagnostic utility of signal‐averaged ECG (SAECG) for detection of cardiac involvement of sarcoidosis. Methods: Subjects with biopsy proven sarcoidosis and symptoms suggestive of possible cardiac involvement were included in the cohort. Standard criteria for SAECG were used. Subjects were considered to have CS if they met criteria established by the Japanese Ministry of Health and Welfare modified to include cardiac MRI. Results: Of the 88 patients in the cohort 27 had evidence of CS independent of the SAECG results. The SAECG was abnormal in 14 of these 27 patients and 11 of the 61 of the subjects without cardiac involvement (P < 0.01). The sensitivity of SAECG detection of CS was 52% with a specificity of 82%. For the entire cohort, SAECG had a positive predictive value (PPV) of 0.56 and a negative predictive value (NPV) of 0.79. Within a subgroup of 67 patients with an unfiltered QRS duration of <100 ms, the specificity for diagnosing cardiac sarcoidosis improves to 100% with a reduced sensitivity of 36.8. Of the SAECG parameters, LAS40 was significantly associated with the diagnosis of cardiac sarcoidosis for the entire cohort (P < 0.01) and among the subgroup of patients with an unfiltered QRS duration of <100 ms (P < 0.01). Conclusions: SAECG is a useful screening tool in the evaluation of sarcoidosis for detection of cardiac involvement. Ann Noninvasive Electrocardiol 2011;16(1):70–76     

Prospective evaluation of relatives for familial arrhythmogenic right ventricular cardiomyopathy/dysplasia reveals a need to broaden diagnostic criteria

OBJECTIVES: We sought to ascertain the prevalence and mode of expression of familial disease in a consecutive series of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). BACKGROUND: Autosomal-dominant inheritance is recognized in ARVC. The prevalence and mode of expression of familial disease in consecutive, unselected families is uncertain. METHODS: First- and second-degree relatives of 67 ARVC index patients underwent cardiac evaluation with history and examination, 12-lead and signal-averaged electrocardiogram (ECG), two-dimensional and Doppler echocardiography, metabolic exercise testing and Holter monitoring. Diagnoses were made in accordance with published criteria. RESULTS: Of 298 relatives, 29 (10%; mean age 37.4 +/- 16.4 years) had ARVC. These were from 19 of the 67 families, representing familial involvement in 28%. Of these affected relatives, 72% were asymptomatic, 17% had ventricular tachycardia (sustained VT 10%, nonsustained VT 7%) and 21% had left ventricular involvement. A further 32 relatives (11%; 37.7 +/- 12.4 years) exhibited nondiagnostic ECG, echocardiographic or Holter abnormalities. Fifteen of these relatives were from families with only the proband affected, and inclusion of this subset of relatives would have resulted in familial ARVC in 48% of index cases. Four additional relatives (1% to 3%) fulfilled diagnostic criteria for dilated cardiomyopathy without any features of right ventricular disease. CONCLUSIONS: By using current diagnostic criteria, familial disease was present in 28% of index patients. A further 11% of their relatives had minor cardiac abnormalities, which, in the context of a disease whose mode of inheritance is autosomal dominant, are likely to represent early or mild disease expression. We advocate that the current ARVC diagnostic criteria are modified to reflect the broader spectrum of disease that is observed in family members.     

Diagnosing isolated cardiac sarcoidosis

Abstract. Kandolin R, Lehtonen J, Graner M, Schildt J, Salmenkivi K, Kivistö SM, Kupari M (Helsinki University Central Hospital, Helsinki, Finland). Diagnosing isolated cardiac sarcoidosis. J Intern Med 2011; 270 : 461–468. Objectives. Cardiac sarcoidosis (CS) without clinically apparent extracardiac disease may escape detection because of the poor sensitivity of endomyocardial biopsy (EMB). We set out to analyse our experience of repeated and imaging‐guided biopsies in clinically isolated CS. Methods. We retrospectively reviewed the medical records, laboratory test results, imaging studies and pathological analyses of 74 patients with either histologically proven or clinically probable CS at our institution between January 2000 and December 2010. Results. Fifty‐two patients had histologically proven CS, of whom 33 (26 women) had disease that was clinically isolated to the heart. Sarcoidosis was detected in the first EMB in 10 of the 31 patients who underwent biopsy. CS was found by repeated EMBs, targeted by cardiac imaging, in seven additional patients, and 11 patients were diagnosed by sampling 18‐F‐fluorodeoxyglucose position emission tomography‐positive mediastinal lymph nodes at mediastinoscopy. Together, the first biopsy (cardiac or mediastinal lymph node) provided the diagnosis in 34%, the second biopsy in 31% and the third in 22% of biopsied patients with isolated CS. Four (13%) of the remaining diagnosis were made after cardiac transplantation and one in a patient who did not undergo biopsy) at autopsy after sudden cardiac death. Conclusions. Cardiac sarcoidosis may present without clinically apparent disease in other organs. At least two‐thirds of patients remain undiagnosed after a single EMB session. The detection rate can be improved by repeated and imaging‐guided cardiac or mediastinal lymph‐node biopsies. Nevertheless, false‐negative biopsy results remain a problem in CS patients with no apparent extracardiac disease.     

超声心动图评价致心律失常性右心室心肌病病变程度的临床研究

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与超声心动图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏磁共振(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组(A组)、右心室弥漫病变组(B组)、累及左心室的双心室病变组(C组),分析比较3组间的超声心动图.结果 心脏MRI结果示,A组患者19例(31%),B组28例(46%),C组14例(23%).共15例(25%)患者超声心动图结果正常(A组13例,B组2例).A组患者中均无右心房增大、右心室室壁变薄、右心室室壁运动减弱.超声心动图示:右心房、心室增大,右心室流出道增宽、右心室室壁变薄及室壁运动减弱均与病变程度呈正相关,而左心参数差异均无统计学意义.结论 早期无症状或局部轻度病变时应用超声心动图检查容易漏诊,应当结合临床表现、心电图并进行心脏MRI检查.依据超声心动图结果中右心参数可以判断病变进展程度,指导治疗方案的选择.     

超声心动图评价致心律失常性右心室心肌病病变程度的临床研究

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与超声心动图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏磁共振(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组(A组)、右心室弥漫病变组(B组)、累及左心室的双心室病变组(C组),分析比较3组间的超声心动图.结果 心脏MRI结果示,...     

Diagnosis of cardiac sarcoidosis and follow-up of 24 consecutive patients

PURPOSE: Cardiac sarcoidosis is responsible for 50% of deaths which mainly occur by ventricular arrhythmia or conduction disorders. The aim of this study is to determine the value of cardiac explorations for an early diagnosis of these localizations, which are often underestimated and can cause sudden death. PATIENTS AND METHODS: We prospectively studied 24 consecutive patients, aged 33 +/-10 years, presenting with a sarcoidosis. Nine (38%) were asymptomatic and had no treatment. Fifteen (62%) were symptomatic: two (8%) had only pulmonary lesions and 13 (54%) had a polyvisceral disease. Seven (30%) were treated. Thirteen (54%) had an elevation of the disease activity markers. The patients had a 12-lead ECG, an echocardiography (TTE), a Holter ECG and a Magnetic Resonance Imaging (MRI) at inclusion. RESULTS: Realization rate was: 100% ECG (24), 83% TTE (20), 75% Holter ECG (18) and 62% MRI (15). Only two patients (8%) had a cardiac involvement. The first one had a polyvisceral sarcoidosis presenting with a hypokinetic cardiomyopathy and a complete AV block and the second one presented with a complete AV block which revealed sarcoidosis. Both patients had a MRI septal hypersignal and disease activity markers. They were treated with cardiac stimulation and corticotherapy: the first patient died suddenly, the second one remains asymptomatic after a 14 months follow-up. The 22 patients (92%) with normal explorations did not present any cardiac involvement during the follow-up (3.7 +/-1.6 years). CONCLUSIONS: This study confirms the rarity of cardiac involvement in sarcoidosis. An exhaustive cardiac check-up does not seem very productive even for patients presenting with polyvisceral disease or an elevation of disease markers. A systematic 12-lead ECG seems to be the most useful and simple tool for the early diagnosis of cardiac sarcoidosis. The other explorations will be realized according to clinical data. The absence of abnormal findings seems to have a good negative predictive value allowing to rule out a cardiac problem.