title: Evaluation of regional cardiac sympathetic innervation in congenital long QT syndrome using 123I-MIBG scintigraphy.
abstract: The aim of this study was to determine whether sympathetic imbalance in congenital long QT syndrome (LQTS) can be identified by cardiac sympathetic neuronal dysinnervation. 123I-metaiodobenzylguanidine (MIBG) is a tracer of the norepinephrine analogue which reflects the regional innervation and norepinephrine kinetics in cardiac sympathetic nerves. Sixteen patients with LQTS, who were members of 12 families, and 7 normal controls underwent MIBG scintigraphy. Myocardial SPET and planar images were obtained 15 min and 4 h after the injection of MIBG in each patient. The relative regional uptake (RRU) and regional washout rate (rWR) of MIBG at 4 h in each of nine regions were compared with those in the control group. The heat-to-mediastinum ratio (H/M) and global washout rate (GWR) were also calculated. The RRU, rWR, H/M and GWR showed no significant difference between LQTS patients and normal controls. Furthermore, the RRU in 96% of all regions in LQTS was within the mean +/- 2 standard deviations of that in the control group. Patients with LQTS have normal cardiac sympathetic innervation, as assessed by MIBG. The sympathetic imbalance hypothesis is unlikely to be attributed to an abnormal distribution and different regional norepinephrine kinetics of cardiac sympathetic nerves.
authors.affiliation: Department of Radiology, Tokyo Women's Medical College, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Momose
authors.forename: H
authors.initials: H
authors.lastname: Kobayashi
authors.forename: H
authors.initials: H
authors.lastname: Kasanuki
authors.forename: K
authors.initials: K
authors.lastname: Kusakabe
authors.forename: A
authors.initials: A
authors.lastname: Tamaki
authors.forename: S
authors.initials: S
authors.lastname: Onishi
authors.forename: T
authors.initials: T
authors.lastname: Okawa
mesh_terms: D019797:3-Iodobenzylguanidine / Q000493:pharmacokinetics; D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D002648:Child; D005260:Female; D006321:Heart / Q000000981:diagnostic imaging* / Q000294:innervation*; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D009474:Neurons / Q000000981:diagnostic imaging / Q000502:physiology; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D013564:Sympathetic Nervous System / Q000000981:diagnostic imaging / Q000503:physiopathology
chemical_list: D000319:Adrenergic beta-Antagonists; D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine