Long QT Syndrome
Ilan Goldenberg, MD* and
Arthur J. Moss, MD
Cardiology Division, Department of Medicine, University of Rochester Medical Center, Rochester, New York.
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Figure 1 Distinctive T-Wave Patterns in the 3 Major LQTS Genotypes
T-wave morphology by LQTS genotype: LQT1: typical broad-based T-wave pattern (corrected QT [QTc] 570 ms); LQT2: typical bifid T-wave (QTc 583 ms); and LQT3: typical late-onset peaked/biphasic T-wave (QTc 573 ms). Reprinted, with permission, from Moss et al. (17).
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Figure 2 Probability of LQTS-Related Events by Gender
Kaplan-Meier estimates of the cumulative probability of (A) a first cardiac event (syncope, aborted cardiac arrest [ACA], or sudden cardiac death [SCD]) and (B) a first life-threatening cardiac event (ACA or SCD) from age 1 through 75 years by gender in 3,779 long QT syndrome (LQTS) patients from the International LQTS Registry. Reprinted, with permission, from Goldenberg et al. (30).
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Figure 3 Probability of Cardiac Events in LQT1 Patients
Kaplan-Meier estimates of the cumulative probability of a first cardiac event in KCNQ1 mutation carriers (LQT1 genotype) by (A) location, (B) type, and (C) biophysical function of the mutation. Reprinted, with permission, from Moss et al. (47).
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Figure 4 Suggested Risk-Stratification Scheme for ACA or SCD in LQTS Patients
Risk stratification categories for LQTS patients based on published event rates; more specific risk subsets by age group are detailed in Table 3. Kaplan-Meier (K-M) estimates are based on a series of 869 LQTS patients (52). CPR = cardiopulmonary resuscitation; TdP = torsades de pointes; other abbreviations as in Figure 2.
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