doi: 10.1161/CIRCULATIONAHA.107.188521
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(Circulation. 2008;117:857-859.)
© 2008 American Heart Association, Inc.
Clinical Summaries
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
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Deficient Zebrafish Ether-à-Go-Go–Related Gene Channel Gating Causes Short-QT Syndrome in Zebrafish Reggae Mutants |
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Various genetic defects, primarily in cardiac ion channels, are known to cause human cardiac repolarization disorders such as long- and short-QT syndrome; however, suitable genetic animal models to study disease mechanisms and evaluate new treatment strategies are largely lacking. In search of suitable arrhythmia models, we isolated the zebrafish mutation reggae (reg), which displays clinical features of the malignant human short-QT syndrome such as accelerated cardiac repolarization accompanied by cardiac fibrillation. By positional cloning, we identified the reg mutation that resides within the voltage sensor of the zebrafish ether-à-go-go–related gene (zERG) potassium channel. The mutation causes premature zERG channel activation and defective inactivation, which results in shortened action potential duration and accelerated cardiac repolarization. Consequently, QT intervals in ECGs from heterozygous and homozygous reg mutant adult zebrafish are considerably shorter than in wild-type zebrafish. Hence, with its molecular and pathophysiological concordance to the human arrhythmia syndrome, zebrafish reg represents the first animal model for human short-QT syndrome and may help to further dissect disease mechanisms and to identify new pharmacological treatment options in high-throughput screens. See p 866.
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Clinical Characteristics and In-Hospital Outcomes of Patients With Cardiogenic Shock Undergoing Coronary Artery Bypass Surgery: Insights From the Society of Thoracic Surgeons National Cardiac Database |
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Because few patients with cardiogenic shock (CS) undergo coronary artery bypass grafting (CABG), the contemporary clinical features and outcomes or risk factors for operative mortality in these patients are not well known. Our study of 708 593 patients with and without CS undergoing CABG enrolled in the Society of Thoracic Surgeons National Cardiac Database (2002–2005) found that patients with preoperative CS constituted 14 956 (2.1%) of patients undergoing
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