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Long-QT Syndrome
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History Of Long-QT Syndrome (Timeline)
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1856: Friedrich Ludwig Meissner in Germany reports probably the first case of long QT syndrome (LQTS). He describes a deaf girl who collapses and dies while being publicly admonished at school. When the parents are informed, they indicate that two brothers of the girls have already died sudenly after a violent fright or rage.

1901: A large Uruguayan family is reported by Morquio in which several siblings collapse and die in early age. In those days the ECG is of course not yet available.

1937: Latham and Munro describe a family with consanguineous marriage, in which all the five children with congenital deafness suffered from spells defined as epileptic and one died suddenly.

1953: M๖ller reports the case of a congenitally deaf boy with frequent syncopes and describes for the first time the electrocardiographic features of LQTS: bradycardia, abnormal ST and T wave throughout the precordial lead, and a prolonged QT interval.

1957: Anton Jervell and Fred Lange-Nielsen provide the first complete description of LQTS. The disease is called "Jervell, Lange-Nieslen syndrome".

1963/64: Romano and O. Connor Ward report independently patients with a cardiac disorder almost identical to that described by Jervell and Lange-Nielsen but without deafness. It is soon appreciated that the entity that many are already calling "Romano-Ward syndrome" was not much more frequently encountered than the "Jervell-Lange-Nielsen syndrome" but also that it involves a different genetic transmission, presumably autosomal dominant.

1966: Yanowitz reports that the QT interval could be prolonged by either right stellectomy or by left stellate ganglion stimulation.

1971: Moss and McDonald make to first and sucessful attempt of performing a left cardiac sympathetic denervation in a LQTS patient refractory to conventional antiarrhythmic therapy.

1975: Peter J. Schwartz and Malliani indicate T wave alternans as the second electrocardiographic characteristic of patients afflicted by LQTS, and are able experimentally to induce this unusual phenomenon by left stellate ganglion stimulation.

1976: Schwartz demonstrates opposite effects of right and left unilateral stellectomy on ventricular fibrillation threshold.

1979: Crampton, Moss and Schwartz initiate the International Registry for LQTS with the objective of enrolling a large number of patients in a prospective study which is expected to last 25 years.

1980: On theoretical and clinical grounds, Schwartz suggests that the spectrum of LQTS might have been larger than previously thought and might include patients with a normal QT interval on the surface ECG. This unorthodox concept has been definitively confirmed by large clinical studies and carries important pratical and medico-legal implications.

1985: Schwartz suggests that the particularly high arrhythmogenic potential of the left cardiac sympathetic nerves is leaving open the possibility that might decrease electrical stability and render the myocardium more vulnerable to the mostly through the quantitatively dominant left stellate ganglion, would have merely represented the trigger for the life-threatening arrhythmias of LQTS.

1986: Moss and Schwartz, extending earlier suggestions of an undefined alteration in potassium regulation, propose that the "unknown intracardiac abnormality" might have been an alteration in one of the K+ repolarizing currents.

1991: Nador provides the first and unexpected evidence for a mechanical alteration, an unusual ventricular wall motion abnormality, thus showing that LQTS is not a purely "electrical" disease.

Keating demonstrates tight linkage of LQTS to the Harvey ras-1 gene locus on the short arm of chromosome 11 in one large family. This is considered the most significant breakthrough.

1995-1996: The identification of three LQTS genes takes place within 9 months, between March 1995 and January 1996.

In December 1995, Schwartz et al. provided the first evidence of gene-specific differential responses to various interventions.

1996-1998: In 1996 Maryann Anglim and Walter Allan M.D. publish KARA MIA, the story of sudden loss and slow recovery in a teenager with Long QT syndrome. It is the first literary approach to LQTS.

In 1997, the European LQTS Information Center (now QTsyndrome.ch) goes online.

Lisa Machotka founds the LQTS mutual chat group. For the first time, LQTS patients and their relatives from all around the world meet on the Internet.

In 1997 Neyroud et al. showed that the recessive Jervell and Lange-Nielsen syndrome is caused by homozygous or by compound heterozygous mutations.

"Chicago Hope Hospital", a famous american TV show on CBS, dedicates an episode to LQTS which is aired on March 25th 1998 in the USA.

In 1998 Priori et al. provided the first evidence that even the Romano-Ward variant can be transmitted as a recessive trait.

In 1998 Schwartz suspects prolonged QT-interval of being an important cause for Sudden Infant Death Syndrome (SIDS).

1999: In February Priori et al. demonstrate that for each gene carrier showing QT prolongation and symptoms of the disease there may be a substantial number of family members who carry the defect in the presence of a normal QT interval.

The LQT6 gene is identified on Chromosome 21. It is named MiRP1 for Min K related protein 1. It resides next to LQT5 (min K gene).

The Internet search engine Yahoo! admitts the subcategory Long QT Syndrome.

2000: Defects of the SCN5A gene encoding the cardiac sodium channel are associated with both the LQT3 subtype of long QT syndrome and Brugada syndrome.

2001: The LQT7 gene is identified and associated with Andersen syndrome.

2002: The first International Sudden Arrhythmia Death Syndromes (SADS) conference is held in London.

New research by the Mayo Clinic in Rochester suggests that approximately 5% of Sudden Infant Death Syndrome (SIDS) cases are the result of long QT syndrome.

2003: Fatal, electrical chaos known as long QT syndrome Type 4, or LQT4 can occur in the hearts of otherwise healthy people who produce a defective accessory protein called ankyrin-B, reports a team of researchers from the University of Maryland Biotechnology Institute in the February 6 issue of the journal Nature.

A scheme for risk stratification among patients with long-QT syndrome according to sex, genotype and prolongation of the QT-interval is proposed.

2004: What had been suspected for a long time has been confirmed in a new study. According to results published in the September 2004 issue of the Journal of the American Medical Association (Jama), there is a high rate of cardiac events among people with genetic long-QT syndrome treated with Beta blockers, particularly for patients with LQT2 and LQT3 genotypes.

Sources: Peter J. Schwarz; The Long QT Syndrome: Clinical Approaches To Tachyarrhythmias, QTsyndrome.ch


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