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J Am Coll Cardiol, 2007; 50:1808-1809, doi:10.1016/j.jacc.2007.07.037 (Published online 12 October 2007).
© 2007 by the American College of Cardiology Foundation
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Anti-KCNH2 Antibody-Induced Long QT Syndrome

Novel Acquired Form of Long QT Syndrome

Kazufumi Nakamura, MD, PhD*, Yusuke Katayama, MD, PhD, Kengo F. Kusano, MD, PhD, Kayo Haraoka, MD, Yoshinori Tani, MD, Satoshi Nagase, MD, PhD, Hiroshi Morita, MD, PhD, Daiji Miura, PhD, Yoshihisa Fujimoto, MD, PhD, Tetsushi Furukawa, MD, PhD, Kazuo Ueda, MD, Yoshiyasu Aizawa, MD, Akinori Kimura, MD, PhD, Yoshihisa Kurachi, MD, PhD and Tohru Ohe, MD, PhD, FACC


Figure 1
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  		Figure 1 Electrocardiogram Traces of a Patient With Acquired QT Prolongation

(A) Marked QT prolongation and Torsades de pointes shown in 24-h electrocardiogram traces. (B) Prolonged QT interval was persistently observed after the initial episodes (corrected QT interval [QTc] 0.70 s0.5). (C) Twelve-lead electrocardiogram 1 year before is normal (QTc 0.43 s0.5).

 

Figure 2
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  		Figure 2 KCNH2 (HERG) Channel Currents and Western Blot Analysis of Autoantibodies Produced Against KCNH2

(A) Representative KCNH2 (HERG) channel currents recorded from HEK 293 cells cultured in control medium (left) and medium supplemented with 2% serum from the patient (right). (B) Representative KCNH2 channel currents recorded from HEK 293 cells cultured in control medium (left) and medium supplemented with 75 µg/ml immunoglobulin (Ig)G from the patient (right). (C) Current (I)–voltage relationships of Istep (left) and Itail (right) recorded from HEK 293 cells cultured in control medium (open circles; n = 4) or a medium supplemented with either 7.5 µg/ml (solid circles; n = 7) or 75 µg/ml (solid squares; n = 7) IgG. The lines fitted to Itail data (right) represent Boltzmann functions. (D) A comparison of the dose-dependent effects of serum (0.2% and 2%) and IgG (7.5 and 75 µg/ml) added to the culture medium (control) of HEK 293 cells expressing KCNH2 on maximal Itail amplitude. (E) Western blot analysis of autoantibodies produced against KCNH2. Lane 1: IgG from a healthy control subject; lane 2: IgG from the patient; lane 3: pre-incubation of the HEK 293 lysate expressing KCNH2 protein with IgG from the patient; lane 4: anti-KCNH2 polyclonal antibodies (positive control).

 




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