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      Chou's Electrocardiography in Clinical Practice 

      Cardiac Arrhythmias in the Fetus, Infants, Children, and Adolescents with Congenital Heart Disease

      edited_book
      Elsevier

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          Most cited references177

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          Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia.

          Mutations in the cardiac ryanodine receptor gene (RyR2) underlie catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited arrhythmogenic disease occurring in the structurally intact heart. The proportion of patients with CPVT carrying RyR2 mutations is unknown, and the clinical features of RyR2-CPVT as compared with nongenotyped CPVT are undefined. Patients with documented polymorphic ventricular arrhythmias occurring during physical or emotional stress with a normal heart entered the study. The clinical phenotype of the 30 probands and of 118 family members was evaluated, and mutation screening on the RyR2 gene was performed. Arrhythmias documented in probands were: 14 of 30 bidirectional ventricular tachycardia, 12 of 30 polymorphic ventricular tachycardia, and 4 of 30 catecholaminergic idiopathic ventricular fibrillation; RyR2 mutations were identified in 14 of 30 probands (36% bidirectional ventricular tachycardia, 58% polymorphic ventricular tachycardia, 50% catecholaminergic idiopathic ventricular fibrillation) and in 9 family members (4 silent gene carriers). Genotype-phenotype analysis showed that patients with RyR2 CPVT have events at a younger age than do patients with nongenotyped CPVT and that male sex is a risk factor for syncope in RyR2-CPVT (relative risk=4.2). CPVT is a clinically and genetically heterogeneous disease manifesting beyond pediatric age with a spectrum of polymorphic arrhythmias. beta-Blockers reduce arrhythmias, but in 30% of patients an implantable defibrillator may be required. Genetic analysis identifies two groups of patients: Patients with nongenotyped CPVT are predominantly women and become symptomatic later in life; patients with RyR2 CPVT become symptomatic earlier, and men are at higher risk of cardiac events. These data provide a rationale for prompt evaluation and treatment of young men with RyR2 mutations.
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            P-wave morphology in focal atrial tachycardia: development of an algorithm to predict the anatomic site of origin.

            The purpose of this study was to perform a detailed analysis of the P-wave morphology (PWM) in focal atrial tachycardia (AT) and construct and prospectively evaluate an algorithm for identification of the anatomic site of origin. Although smaller studies have described the PWM from particular anatomic locations, a detailed algorithm characterizing the likely location of a tachycardia associated with a P-wave of unknown origin has been lacking. The PWMs for 126 consecutive patients undergoing successful radiofrequency ablation of 130 ATs are reported. P waves were included only when the onset was preceded by a discernible isoelectric segment. P waves were classified as positive (+), negative (-), isoelectric, or biphasic. Sensitivity, specificity, and predictive values were calculated. On the basis of these results, an algorithm was constructed and prospectively evaluated in 30 new consecutive ATs. The distribution of ATs was right atrial (RA) in 82 of 130 (63%) and left atrial (LA) in 48 of 130 (37%). Right atrial sites included crista (n = 28), tricuspid annulus (n = 29), coronary sinus (CS) ostium (n = 14), perinodal (n = 7), right septum (n = 1), and RA appendage (n= 3). Left atrial sites included pulmonary veins (n = 32), mitral annulus (n = 8), CS body (n= 3), left septum (n = 3), and LA appendage (n = 2). In electrocardiographic lead V1, a negative or +/- P-wave demonstrated a specificity of 100% for a RA focus, and a + or -/+ P-wave demonstrated a sensitivity of 100% for a LA focus. A characteristic PWM was associated with high sensitivity and specificity at common atrial sites for tachycardia foci. A P-wave algorithm correctly identified the focus in 93%. Characteristic PWMs corresponding to known anatomic sites for focal AT are associated with high specificity and sensitivity. A P-wave algorithm correctly identified the site of tachycardia origin in 93%.
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              Genetics of acquired long QT syndrome.

              The QT interval is the electrocardiographic manifestation of ventricular repolarization, is variable under physiologic conditions, and is measurably prolonged by many drugs. Rarely, however, individuals with normal base-line intervals may display exaggerated QT interval prolongation, and the potentially fatal polymorphic ventricular tachycardia torsade de pointes, with drugs or other environmental stressors such as heart block or heart failure. This review summarizes the molecular and cellular mechanisms underlying this acquired or drug-induced form of long QT syndrome, describes approaches to the analysis of a role for DNA variants in the mediation of individual susceptibility, and proposes that these concepts may be generalizable to common acquired arrhythmias.
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                Author and book information

                Book Chapter
                2008
                : 694-720
                10.1016/B978-141603774-3.10031-0
                b62d68d7-a4c1-4859-ab8b-0d7e1d806729
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