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Arrhythmogenic right ventricular cardiomyopathy (ARVC)
is a rare disease which may be difficult to diagnose. The aim of the study was to compare
the diagnostic value of different imaging methods for detection of ARVC and to determine
effective drugs to control ventricular arrhythmia in "idiopathic" patients
(pts).
Methods. We studied a group of 33 pts (age 15-60 yrs, mean age 27 yrs, 22 males and 11
females) having "idiopathic" ventricular arrhythmia (VA) and supposed the
absence of any obvious cardiac or pulmonary disease after examination which have included
ECG, chest X-ray, Holter monitoring, exercise stress testing, echocardiography. Spin-echo
and cine-MRI (magnetic resonance imaging), radionuclide ventriculography (RVG) and
cineangiography (CAG) were performed in the next step of examination. These methods were
able to reveal the right ventricular (RV) structural abnormality and allow to recognize
the minor form of ARVC.
Results. Increase of RV size, volume and zones of abnormal RV wall
motion were detected by echo in 25% of pts with ARVC, RVG - in 27% of ARVC cases. MRI was
able to detect different changes in RV morphology and function in all 12 pts with minor
form of ARVC. The following abnormalities of RV were seen on MRI images in the group of
ARVC: increase of RV diameter - 8 pts (67%), dilatation of RV outflow tract - 3 pts (25%),
regional thinning of RV wall - 9 pts (75%), hypo/akinesis of RV wall - 4 pts (33%), the
aneurysmal dilatations and sacculations - 5 pts (42%), focal changes in RV wall signal
intensity - 2 pts (17%). In 2 cases myocardial biopsies were performed and diagnosis of
ARVC were proved. The results of the therapies with propafenon were satisfactory in 67%
pts with ARVC and 81% pts with idiopathic ventricular arrhythmias (IVA). Ethmozin was
successful in 25% cases of ARVC and 55 % cases of IVA. Beta-blocking agent obtained 25%
success in ARV and one success in IVA. Verapamil supressed VA only in group of ARVC (25%).
Besides the antiarrythmic drugs of I, II, IV classes to prevent ventricular arrythmia,
capoten was used in all pts. So, only one pt with idiopathic VA could be controlled by
capoten.
Conclusions. Cardiac MRI may represent a potent and reliable tool in
recognizing of minor form of ARVC. The comparative analysis of antiarrhythmic drugs of I,
II, IV classes and an angiotensinconverting enzyme inhibitor (capoten) to control VA
showed that propafenon was more effective in the group of pts with ARVC and IVA group (pts
with ARVC - 67% and pts with IVA - 81%).
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Arrhythmogenic right ventricular dysplasia
ventricular arrhythmias, magnetic resonance imaging, echocardiography, class I, II, IV
drugs, angiotensin converting enzym inhibitor, propafenone, OA
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