Cardiac sarcoidosis mimicking right ventricular dysplasia.

A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.     

Cardiac sarcoidosis masquerading as arrhythmogenic right ventricular cardiomyopathy

We present a case of ventricular tachycardia with clinical features suggestive of arrhythmogenic right ventricular cardiomyopathy. However, endomyocardial biopsy revealed non-caseating granulomas diagnostic of cardiac sarcoidosis.     

Chronic (or healed) myocarditis mimicking arrhythmogenic right ventricular dysplasia

The aetiology of arrhythmogenic right ventricular dysplasiais still unknown, and there are few reports on familial coincidencein the literature. A case of a previously healthy man with anepisode of acute myocarditis is described. After recovery fromacute myocarditis, the patient was resuscitated from abortedsudden cardiac death 16 months later. Angiographic and electrophysiologicalevaluation suggested the pattern of arrhythmogenic right ventriculardysplasia. The case seems to suggest that arrhythmogenic rightand/or left ventricular dysplasia could be mimicked by chronic(or healed) myocarditis.     

Etiopathogenesis of arrhythmogenic right ventricular dysplasia

Arhythmogenic right ventricular dysplasia (ARVD) is a genetically determined cardiomyopathy with a dominant transmission mode and variable penetrance. Transdifferenciation of cardiomyocytes into adipocytes is likely to explain massive replacement of right ventricular and to a lesser extent left ventricular myocardium by adipose tissue. This phenomenon starts in the mediomural layers and extends into the epicardium. It can occur in the fetus, however youth and young adults are more frequently involved. Apoptosis defined as a programmed cell death, is likely to enhance adipogenesis and tiny fibrosis production. Inflammation can be superimposed on the genetically determined substrate and usually involves both ventricles. Myocarditis can be acute or chronic with interstitial or scar fibrosis, or active chronic. In some cases, left ventricular involvement can be as important as right ventricle, characterizing biventricular dysplasia. In Naxos disease, ARVD is associated with an ectodermic dysplasia. The transmission mode is recessive.     

A case of arrhythmogenic right ventricular dysplasia with ventricular fibrillation

A case of repeated attacks of ventricular fibrillation is described. The patient suffered from an arrhythmogenic right ventricular dysplasia (ARVD) documented by right and left ventriculograms and myocardial biopsies obtained during surgical treatment of the arrhythmia. The histological changes were interpreted as being signs of fresh myocardial damage of unknown origin in addition to a replacement of the normal myocardium by adipose and fibrotic tissue. The repeated attacks of ventricular fibrillation in this patient contrast to the arrhythmia spectrum noted in the available literature on ARVD, mostly stable chronic ventricular tachycardias.     

Late potentials and arrhythmogenic right ventricular dysplasia

The authors report on their experience in investigating late potentials (LPs) in arrhythmogenic right ventricular dysplasia (ARVD), by comparing 35 patients with ARVD with 40 control subjects. The values of QRS and RMS40 and the duration of the LP showed a marked difference between the control group and the ARVDs, particularly if sustained VT was present. In this case, 68 per cent of patients were found to have LPs (with at least 2 out of 3 parameters abnormal) versus 50 per cent in the ARVDs without sustained VT and 5 per cent in the controls. There was a difference between the diffuse forms (10 per cent) and the localized forms (25 per cent) of ARVD: the LPs were markedly more obvious in the diffuse forms. Likewise, there was a clear relationship between age and the criteria for LPs in ARVD with sustained VT. This is an argument in favour of the evolutive character of the disease, which could be observed directly in a few cases in which the examinations were repeated over the course of time.     

Electroanatomic mapping of arrhythmogenic right ventricular dysplasia

OBJECTIVES: We tested the hypothesis that spatial association of low-amplitude intracardiac electrograms can identify the presence, location and extent of dysplastic regions in arrhythmogenic right ventricular dysplasia (ARVD). BACKGROUND: Arrhythmogenic right ventricular dysplasia is a right ventricular (RV) cardiomyopathy characterized pathologically by fibrofatty infiltration and clinically by a spectrum of arrhythmias, sudden cardiac death and RV failure. Diagnosis of ARVD still remains a clinical challenge. METHODS: A three-dimensional electroanatomic mapping technique was used to map the RV of two groups of patients: 1) those with ARVD presenting with typical clinical, electrocardiographic and echocardiographic or magnetic resonance imaging (MRI) findings; and 2) those with structurally normal ventricles. RESULTS: The dysfunctional RV area could be identified only in the first group and was characterized by the presence of discrete areas of abnormally low-amplitude electrograms. Hence, the normal voltage values observed in the control group (unipolar: 11.9 +/- 0.3 mV; bipolar: 4.6 +/- 0.2 mV [mean +/- SEM]) and in the nonaffected zones in the ARVD group (unipolar: 10.4 +/- 0.2 mV; bipolar: 4.6 +/- 0.2 mV) were reduced significantly (p < 0.05) in the dysplastic areas (unipolar: 3.3 +/- 0.1 mV; bipolar: 0.5 +/- 0.1 mV). The pathologic process mainly involved the RV anterolateral free wall, apex and inflow and outflow tracts and ranged from patchy areas to uniform and extensive involvement. Concordance between electroanatomic findings and MRI or echocardiographic findings was noted in all patients. CONCLUSIONS: The pathologic substrate in ARVD can be identified by spatial association of low-amplitude endocardial electrograms, reflecting replaced myocardial tissue. The ability to accurately identify the presence, location and extent of the pathologic substrate may have important diagnostic, prognostic and therapeutic implications.     

Cardioangiographic findings in patients with arrhythmogenic right ventricular dysplasia

The dimension, contractility, and regional wall motion of the right and left ventricles were scored on the angiograms of 13 patients with arrhythmogenic right ventricular dysplasia. In 10 patients the right ventricle was enlarged, in eight the contractility of the right ventricle was reduced, and in all but one patient there were regional wall motion abnormalities of the right ventricle. The most common abnormality of regional wall motion was mild hypokinesia. There were bulging or dyskinetic areas in seven patients. Regional wall motion abnormalities of the left ventricle were found in five patients, two of whom also had bulging or dyskinetic areas. The reproducibility of right ventricular dimension, contractility, and regional wall motion scores was generally fair but varied unexpectedly both within and between two observers (Kendall's Tau 0.38-0.92). The score values of regional wall motion for some of the segments differed considerably within and between observers. One of the observers consistently gave higher scores than the other. These data suggest that a more objective approach is needed for evaluating angiographic changes in arrhythmogenic right ventricular dysplasia.