Circulation, Vol 89, 667-683, Copyright © 1994 by American Heart Association
T Wichter, G Hindricks, H Lerch, P Bartenstein, M Borggrefe, O Schober and G Breithardt
BACKGROUND: In patients with arrhythmogenic right ventricular
cardiomyopathy (ARVC), the frequent provocation of ventricular tachycardia
during exercise, the sensitivity toward catecholamines, and the response
toward antiarrhythmic drug regimen with antiadrenergic properties suggest
an involvement of the sympathetic nervous system in arrhythmogenesis.
METHODS AND RESULTS: To analyze the presence, extent, and location of
impaired myocardial sympathetic innervation in ARVC,
123I-meta-iodobenzylguanidine (123I-MIBG) scintigraphy was performed in 48
patients with ARVC. For comparison, 9 patients with idiopathic ventricular
tachycardia and a control group of 7 patients without heart disease were
investigated. In patients with ARVC, the clinical sustained (n = 25; 52%)
or nonsustained (n = 23; 48%) ventricular tachycardia originated in the
right ventricular outflow tract in 38 patients (79%), whereas in the
remaining 10 patients (21%), the site of origin was the apical (n = 5) or
inferior (n = 5) right ventricle. In 33 patients (69%), nonsustained or
sustained ventricular tachycardia was provocable by exercise (n = 28 of 48;
58%) and/or by isoproterenol infusion (n = 16 of 37; 43%), whereas
programmed ventricular stimulation induced sustained or nonsustained
ventricular tachycardia in 16 patients each (33% each). With 123I-MIBG
scintigraphy, the right ventricle was not visible in any patient. No areas
of intense 123I-MIBG uptake ("hot spots") were observed. All patients of
the control group and 7 of 9 patients (78%) with idiopathic ventricular
tachycardia showed a uniform tracer uptake in the left ventricle. In
contrast, only 8 of 48 ARVC patients (17%) showed a homogeneous
distribution of 123I- MIBG uptake, whereas 40 patients (83%) demonstrated
regional reductions or defects of tracer uptake. In 3 of 48 patients (6%),
the defect area was < 15%; in 21 patients (44%), it was 15% to 30%; and
in 16 patients (33%), it was > 30% of the polar map area of the left
ventricle (mean, 23 +/- 15%; range, 0% to 57%). In 38 of 40 patients (95%)
with an abnormal 123I-MIBG scan, reduced tracer uptake was located in the
basal posteroseptal left ventricle, involving the adjacent lateral wall in
10, the anterior wall in 2, and the apex in 12 patients. Only 2 patients
demonstrated isolated defects of the anterior or lateral wall; one involved
the apex. Perfusion abnormalities in the areas of 123I- MIBG defects were
excluded by stress/redistribution 201T1 single-photon emission computed
tomography scintigraphy and by normal coronary angiograms in all patients.
Abnormalities in 123I-MIBG scintigraphy in patients with ARVC correlated
with the site of origin of ventricular tachycardia, demonstrating a
regionally reduced tracer uptake in 36 of 38 patients (95%) with right
ventricular outflow tract tachycardia compared with only 4 of 10 patients
(40%) with other right ventricular origins of tachycardia. There was no
correlation between the results of 123I-MIBG scintigraphy and the extent of
right ventricular contraction abnormalities, right ventricular ejection
fraction, biopsy results, coronary anatomy, or left ventricular involvement
in ARVC. CONCLUSIONS: In patients with ARVC, regional abnormalities of
sympathetic innervation are frequent and can be demonstrated by 123I-MIBG
scintigraphy. Sympathetic denervation appears to be the underlying
mechanism of reduced 123I-MIBG uptake and may be related to frequent
provocation of ventricular arrhythmias by exercise or catecholamine
exposure in ARVC. Therefore, in patients with ARVC, the noninvasive
detection of localized sympathetic denervation by 123I-MIBG imaging may
have implications for the early diagnosis and for the choice of
antiarrhythmic drugs in the treatment of arrhythmias.
ARTICLES
Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy. An analysis using 123I-meta- iodobenzylguanidine scintigraphy
Hospital of the Westfalische Wilhelms-University, Department of Cardiology and Angiology, Munster, Germany.
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