Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias

Nava, Andrea; Thiene, Gaetano; Canciani, B; Martini, Bortolo; Daliento, Luciano; Buja, Gianfranco; Fasoli, G

doi:10.1016/0167-5273(92)90177-5

Cited by 57 publications

(22 citation statements)

References 34 publications

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“…In fact, Case F was the only one in which the experimental protocol image was graded with positive fatty infiltration. If the experimental protocol does not identify fatty infiltration below a certain threshold, and since levels of fatty infiltration that qualify as a positive ARVD/C fatty infiltration diagnosis are significantly higher (Nava et al, 1992) than the levels found in the cadavers of this study, then the experimental protocol would minimize the number of false positive diagnoses of fatty infiltration for ARVD/C diagnosis.…”

Section: Discussionmentioning

confidence: 91%

Correlation of magnetic resonance imaging with histopathology in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)

et al. 2005

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an asymptomatic cardiac disease characterized by fatty infiltration of the right ventricular myocardium and often results in sudden cardiac death. ARVD/C diagnosis includes the assessment of fatty infiltration, which can be achieved noninvasively with cardiovascular magnetic resonance (CMR). The standard CMR protocol places the signal-generating coil directly on the anterior chest wall and produces a nonspecific high intensity signal that obscures the high signal from fatty infiltration. The aim of this study was to determine whether increasing the coil-to-chest distance would improve identification of fatty infiltration. Thoraces from seven embalmed cadavers were imaged on a conventional 1.5 Tesla CMR scanner using the control protocol and an experimental protocol, with a 6 cm coil-to-chest distance. A representative axial MR image and corresponding gross section of the heart were analyzed in each case. Fatty infiltration was graded in a blinded fashion on the MR images with independent histopathologic assessment. In five of the seven cases, the experimental protocol provided a correlation between CMR and histopathology that was as good as or better than the control protocol. The experimental protocol was also better in preventing false positive diagnosis in cases of minimal infiltration. Thus, the experimental protocol showed a stronger correlation with histopathology than did the control protocol. Increasing the distance between the anterior surface coil and chest wall may improve classification of presence or absence of fatty infiltration in the right ventricular myocardium, potentially improving the noninvasive detection of ARVD/C with CMR.

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Section: Discussionmentioning

confidence: 91%

Correlation of magnetic resonance imaging with histopathology in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)

et al. 2005

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“…Because of the variety of symptoms and the complexity of diagnostic criteria, a complete and detailed diagnostic work-up is necessary to make the correct diagnosis. Otherwise, patients with ARVC may be both pathologically and clinically misdiagnosed as suffering from, i.e., idiopathic ventricular tachyarrhythmias (no structural heart disease) or DCM [106,108,169,188].…”

Section: Epidemiologymentioning

confidence: 99%

Molecular Mechanisms of Inherited Ventricular Arrhythmias

Wichter

Schulze‐Bahr

Eckardt

et al. 2002

Herz

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“…A simpler diagnostic tool is the 12-lead ECG and signal-averaged ECG. An abnormal ECG may be seen in up to half of the patients with ARVC [6][7][8] . A relatively high incidence of late potentials may contrast with a low incidence of T wave inversion.…”

Section: Arvc and Clinical Datamentioning

confidence: 99%

“…In particular regional or global dilatation, aneurysms and impaired contractility of right ventricular (RV) [5] , histological and electrical abnormalities [6][7][8] characterize the disease. A morphological ARVC feature is diffuse or segmental intramyocardial fatty tissue infi ltration of the RV free wall [9][10][11][12] while the RV septum is rarely involved [13] .…”

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confidence: 99%

Arrhythmogenic Right Ventricular Cardiomyopathy: A Presentation of Thirty Consecutive Patients

Nielsen

Thomsen²,

Chen

et al. 2005

Heart Drug

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Background: There is a limited number of studies of consecutive patients suspected of having arrhythmogenic right ventricular cardiomyopathy (ARVC) as established by the Task Force report [Br Heart J 1994;71:215–218]. Objective: The aim of this study was to describe a population of ARVC patients who were referred to a university hospital for thorough clinical evaluation of right ventricle arrhythmia using both noninvasive and invasive procedures. Methods: In a prospective design 48 patients suspected of having ARVC underwent cardiac magnetic resonance imaging (MRi), contrast ventriculography, an electrophysiological test and endomyocardial biopsies from the lower septum. Results: A diagnosis of ARVC was established in 30 patients (age 40 ± 2 years, mean ± SEM) and in one third of the patients, intense running, cycling or rowing provoked palpitations and syncope. Arrhythmias were frequent premature contractions of RV origin and/or ventricular tachycardia with a left bundle branch configuration. In 14 ARVC patients the ECG trace showed right bundle branch block or inverted T waves in right precordial leads. Contrast ventriculography demonstrated RV dilatation in 11 ARVC patients and in 18 patients the septal biopsies showed fatty tissue myocardial infiltration. In 25 patients cardiac MRi showed islands of high signal intensity indicative of RV fatty infiltration. Conclusions: ARVC is a heterogeneous syndrome and an abnormal right ventricle and arrhythmias of right ventricle origin must lead to a clinical evaluation. Cardiac MRi appears to be an important diagnostic tool in patients suspected of ARVC and should be used to guide invasive procedures.

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Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias

Cited by 57 publications

References 34 publications

Correlation of magnetic resonance imaging with histopathology in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)

Correlation of magnetic resonance imaging with histopathology in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)

Molecular Mechanisms of Inherited Ventricular Arrhythmias

Arrhythmogenic Right Ventricular Cardiomyopathy: A Presentation of Thirty Consecutive Patients

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