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a Service
de Cardiologie Pédiatrique, CHU Necker-Enfants Malades, 149 rue de
Sèvres, Paris, France, b Service
Hospitalier Frédéric Joliot, Département de Recherche Médicale,
DSV-CEA, 4 place du Général Leclerc, 91406 Orsay, France
Correspondence to: Dr Merlet pmerlet{at}europost.org
Accepted 21 December
2000
OBJECTIVE
To evaluate the prognostic
value of metaiodobenzylguanidine (MIBG) imaging in childhood cardiomyopathy.
DESIGN
Prospective cohort study.
SETTING
Tertiary referral centre.
PATIENTS
40 children (21 boys, 19 girls; mean (SD) age, 7.0 (5.6) years) with heart failure resulting
from idiopathic dilated cardiomyopathy (n = 23) or various other
disorders (n = 17).
METHODS
At the initial examination,
cardiac 123I-MIBG uptake and release, circulating
noradrenaline (norepinephrine) concentration,
x ray cardiothoracic ratio, and
echocardiographic variables were recorded. Cardiac MIBG uptake was
obtained by measuring the heart to mediastinum activity ratio on the
planar image obtained four hours after MIBG injection. MIBG washout
rate was evaluated using relative decrease in cardiac activity measured
at 20 minutes and four hours. Patients were treated with angiotensin
converting enzyme inhibitors, diuretics, and digitalis, and were
followed up for 12 (10) months. Fifteen patients did not respond to
medical treatment (12 heart transplants; three deaths), and 25 did
respond (improved or stable).
RESULTS
Cardiac MIBG uptake was
positively correlated with x ray
cardiothoracic index (r = 0.55,
p = 0.0008) and echocardiographic left ventricular fractional
shortening (r = 0.68, p < 0.0001). Among
all the clinical and laboratory variables tested, multivariate discriminant analysis showed that the only independent predictor of an
unfavourable outcome was a low MIBG uptake (p < 0.001). Survival
curves had a mean threshold value of 1.54 for MIBG uptake.
CONCLUSIONS
Impaired cardiac
adrenergic innervation is strongly related to adverse outcome in
children with dilated cardiomyopathy, independently of the aetiology.
MIBG imaging may help to stratify risk in such patients.
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