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Am J Physiol Heart Circ Physiol 285: H1286-H1293, 2003. First published May 29, 2003; doi:10.1152/ajpheart.00276.2003
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Cardiac retention of [11C]HED in genotyped long QT patients: a potential amplifier role for severity of the disease

Alejandro N. Mazzadi,1,2 Xavier André-Fouët,3 Jérôme Duisit,1,2 Véronique Gebuhrer,3 Nicolas Costes,1 Philippe Chevalier,3 Claire Rodriguez,3 Jean-Jacques Schott,4 Hervé Le Marec,4 Pascale Guicheney,5 Didier Le Bars,1 and Marc Janier1,2

1Centre d'Exploration et de Recherche Médicales par Émission de Positons, 69003 Lyon; 2Centre de Recherche et d'Applications en Traitement de l'Image et du Signal, Unité Mixed de Recherche-Centre National de Recherche Scientifique 5515, 69394 Lyon; 3Hôpital Cardiovasculaire et Pneumologique et Université Claude Bernard, 6934 Cedex 03 Lyon; 4Unité 533, Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de Formation et Recherche de Medecine, Hôpital Laënnec, Nantes 44035; and 5Unité 523, INSERM, Institut de Myologie, Groupe Hospitalier Pitié-Salpétrière, Paris 75013, France

Submitted 27 March 2003 ; accepted in final form 22 May 2003

Although mutations in cardiac sodium and potassium channel genes are associated with congenital long QT syndrome (LQTS), a "modifier" role of the sympathetic nervous system was proposed to explain the distinct severity of the disease. We evaluated cardiac sympathetic innervation using [11C]hydroxyephedrine ([11C]HED) and positron emission tomography (PET) in genotyped LQTS patients. H215O and [11C]HED PET studies were performed in 11 patients (5 symptomatic) and 8 controls. Perfusion and [11C]HED images were depicted as 36-sector polar maps. Sectorial values of perfusion (H2O%), absolute (HEDRet) and relative retention (HED%Ret) of [11C]HED, and the ratio of HED%Ret to H2O% (HED%Ret/H2O%) were calculated. Normal databases were obtained from controls. Sectorial values below 2SD database values were defined as "outside sectors." Controls and patients showed similar sectorial perfusion. Sectorial HEDRet did not differ between groups, but means of HED%Ret were lower in three sectors for patients (P < 0.05). Three sectors from 3 controls had HED%Ret below 2SD, whereas 36 sectors in 9 patients were outside sectors (P < 0.01). In patients, average HED%Ret/H2O% was lower in 9 sectors (P < 0.05 vs. controls); 2 outside sectors were found in controls, but 43 outside sectors were found in patients (P < 0.01), 77% of them in the 5 symptomatic patients. Heterogeneous [11C]HED retention was localized in the septal, anterior, and lateral walls. Most LQTS patients showed a localized and decreased pattern of [11C]HED retention. The larger number of heterogeneous sectors in symptomatic patients suggests that sympathetic function could play an amplifier role for severity of the disease.

long QT syndrome; genetics; positron emission tomography; sympathetic nervous system; [11C]hydroxyephedrine



Address for reprint requests and other correspondence: X. André-Fouët, Hôpital Cardiovasculaire et Pneumologique, BP Lyon Montchat, 69394 Lyon Cedex 03, France (E-mail: xavier.andre-fouet{at}chu-lyon.fr).




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