先天性QT延長症候群に伴う臨床像と心事故に関する検討 : サブタイプによる心事故の誘因と治療効果の違い

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Data about the clinical course of Japanese congenital long-QT syndrome (LQTS) patients are limited. 54 LQTS patients (1 Jervell and Lange-Nielsen, 53 Romano-Ward 53; 14 males, 40 females) seen in our cardiology and pediatric cardiology department were retrospectively evaluated. Direct gene analysis and subtype-typical ST-T-wave ECG patterns were used to identify subtypes (14 LQT1, 25 LQT2, 3 LQT3,1 unidentified). Ten patients experienced aborted cardiac death on initial presentation; no LQTS-related deaths occurred during follow-up. LQT1 patients had the majority of their events (69%) during exercise; only 8% occurred during rest/sleep. Among LQT2 patients, 16% had their events during exercise and 36% during rest/sleep. Swimming and auditory stimuli seem to be specific triggers for LQT1 and LQT2, respectively. LQT1 patients were younger than LQT2 patients at the time of the first cardiac event before therapy was initiated. Most patients (85%) initially received β-blocker therapy. Implantable cardioverter-defibrillators (ICD) were implanted in 11 patients. No difference in the cumulative rate of syncope recurrence-free survival was observed between LQT1 and LQT2 patients receiving β-blockers. However LQT2 patients received more concomitant therapy, such as mexiletine, nicorandil, and ICD. Lethal events occurred among LQT2 and LQT3 patients but not LQT1. In 19 LQT women who experienced pregnancy, there were 36 deliveries and a spontaneous abortion. Four patients had cardiac events during the postpartum period, but not pregnancy. In LQTS patients, triggers of life-threatening arrhythmias and the effectiveness of β-blocker therapy vary by subtype. Therefore, lifestyle measures should be instituted to prevent triggers, and the importance of subtype-specific therapeutic approaches should be recognized.
東京女子医科大学の論文

先天性QT延長症候群に伴う臨床像と心事故に関する検討 : サブタイプによる心事故の誘因と治療効果の違い

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