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Epinephrine unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome

Wataru Shimizu, MD, PhD^*,*, Takashi Noda, MD^*, Hiroshi Takaki, MD, Takashi Kurita, MD, PhD^*, Noritoshi Nagaya, MD, PhD^*, Kazuhiro Satomi, MD^*, Kazuhiro Suyama, MD, PhD^*, Naohiko Aihara, MD^*, Shiro Kamakura, MD, PhD^*, Kenji Sunagawa, MD, PhD, Shigeyuki Echigo, MD, Kazufumi Nakamura, MD, PhD, Tohru Ohe, MD, PhD, FACC, Jeffrey A. Towbin, MD^||, Carlo Napolitano, MD, PhD^¶ and Silvia G. Priori, MD, PhD^¶

^* Division of Cardiology, Department of Internal Medicine, Suita, Japan
Department of Cardiovascular DynamicsSuita, Japan
Department of Pediatrics, National Cardiovascular Center, Suita, Japan
Department of Cardiovascular Medicine, Okayama University Graduate School of Medical and Dentistry, Okayama, Japan
^|| Department of Pediatrics (Cardiology), Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA
^¶ Molecular Cardiology, Salvatore Maugeri Foundation, IRCCS, Pavia, Italy

View larger version (92K): [in a new window]   Figure 1 Twelve-lead electrocardiograms under baseline condition (A) and precordial electrocardiograms during epinephrine infusion (2 min after start of epinephrine) (B) in an LQT1 mutation carrier. The mean corrected Q-Tend interval was dramatically prolonged by epinephrine, leading to spontaneously terminating torsade de pointes.

View larger version (71K): [in a new window]   Figure 2 Twelve-lead electrocardiograms under baseline conditions and during epinephrine infusion in Group I (A and B) and Group II (C and D) patients. Epinephrine markedly prolonged the mean corrected Q-Tend in both Group I (516586 ms) and Group II (435516 ms) patients.

View larger version (70K): [in a new window]   Figure 3 Twelve-lead electrocardiograms under baseline conditions and during epinephrine infusion in Group III (A and B) and Group IV (C and D) patients. No significant changes were produced by epinephrine in both Group III (402394 ms) and Group IV (381392 ms) patients.

View larger version (31K): [in a new window]   Figure 4 Composite data of the electrocardiographic parameters (A to E) and heart rate (F) before and after epinephrine in Groups I (closed circle), II (open circle), III (open square), and IV (open triangle). *p < 0.005 vs. Groups II, III, and IV; p < 0.005 vs. Groups III and IV; p < 0.05 vs. Groups III and IV; ¶p < 0.005 vs. before; ¶¶p < 0.05 vs. before.

View larger version (29K): [in a new window]   Figure 5 Composite data of the changes () of the electrocardiographic parameters (A to E) and heart rate (F) with epinephrine in Groups I, II, III, and IV. *p < 0.005 vs. Groups III and IV; **p < 0.05 vs. Group III.

View larger version (27K): [in a new window]   Figure 6 Composite data of the changes () of the electrocardiographic parameters (A to E) and heart rate (F) with epinephrine in 15 symptomatic patients (Sym) and 19 asymptomatic mutation carriers (Asym) in Groups I and II. *p < 0.05 vs. Asym.