Romano-ward and other congenital long QT syndromes

Cardiovascular Drugs and Therapy

Volumn 16, Issue 1, 2002, Pages 19-23

  Bloise, Raffaella ^a,b   Napolitano, Carlo ^a,b   Priori, Silvia G ^a,b  

^a IRCCS   (Italy)

^b UNIVERSITY OF PAVIA   (Italy)

Author keywords

Genetic bases of arrhythmias; Long QT syndrome; Sudden cardiac death; Ventricular arrhythmias

Indexed keywords

CONFERENCE PAPER; CORRELATION ANALYSIS; GENE MUTATION; GENE THERAPY; GENETIC RISK; GENOTYPE; HEART ARRHYTHMIA; HUMAN; LONG QT SYNDROME; PHENOTYPE; PRIORITY JOURNAL; RISK ASSESSMENT;

DEATH, SUDDEN, CARDIAC; GENOTYPE; HUMANS; LONG QT SYNDROME; MOLECULAR BIOLOGY; PHENOTYPE; ROMANO-WARD SYNDROME;

EID: 0036277134     PISSN: 09203206     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1015359213410     Document Type: Conference Paper

References (43)

1. The idiopathic long QT syndrome: Pathogenetic mechanisms and therapy
2. Autonomic nervous system in sudden death
3. Aritmie cardiache rare in eta' pediatrica
4. New familial cardiac syndrome in children
5. Stress and sudden death. The case of the long QT syndrome
6. Congenital deaf mutism, functional heart disease with prolongation of the QT interval and sudden death
7. The long Q-T syndrome
8. Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene
9. Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity
10. K(V)LQT1 and lsK (minK) proteins associate to form the I(Ks) cardiac potassium current
11. Coassembly of K(V)LQT1 and mink (IsK) proteins to form cardiac I(Ks) potassium channel
12. + channels expressed in Xenopus oocytes
13. A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel
14. Primary structure and functional expression of the human cardiac tetrodotoxin-insensitive voltage-dependent sodium channel
15. MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia
16. Molecular epidemiology of the long QT syndrome in a cohort of 267 probands
17. Homozygous deletion in KVLQT1 associated with Jervell and Lange-Nielsen syndrome
18. Genetic and molecular basis of cardiac arrhythmias: Impact on clinical management parts I and II
19. Functional effects of mutations in KvLQT1 that cause long QT syndrome
20. Cellular dysfunction of LQT5-minK mutants: Abnormalities of IKs, IKr and trafficking in long QT syndrome
21. Dysfunction of delayed rectifier potassium channels in an inherited cardiac arrhythmia
22. + channel-linked long-QT syndrome
23. The loss of function induced by HERG and KVLQT1 mutations does not correlate with the clinical severity of the long QT syndrome
24. KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome
25. Genotype-phenotype correlation in the long-QT syndrome: Gene-specific triggers for life-threatening arrhythmias
26. Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome
27. Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1)
28. Swimming, a gene-specific arrhythmogenic trigger for inherited long QT syndrome
29. Influence of genotype on the clinical course of the long-QT syndrome
30. ECGT-wave patterns in genetically distinct forms of the hereditary long QT syndrome
31. + channel blockade and to increases in heart rate. Implications for gene-specific therapy
32. Sinus node function and ventrieular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects
33. Unexpected phenotypic heterogeneity an prevalence of LQT3
34. The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome
35. Low penetrance in the long-QT syndrome: Clinical impact
36. Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias
37. A genetic basis for quinidine-induced (acquired) long QT syndrome
38. Prevalence of KCNE2 (Mirp1) mutations in acquired long QT syndrome
39. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome
40. + channel blockade, beta-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and HERG defects present in the long-QT syndrome
41. Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium
42. Long QT syndrome associated with syndactyly identified in females
43. A new form of long QT syndrome associated with syndactyly