History Of Long-QT Syndrome (Timeline)
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
1901: A large
Uruguayan family is reported by Morquio in which several siblings collapse
and die in early age. In those days the
is of course not yet available.
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.
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.
Jervell and Fred Lange-Nielsen provide the first complete description of
LQTS. The disease is called "Jervell, Lange-Nieslen syndrome".
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.
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
1975: Peter J.
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.
demonstrates opposite effects of right and left unilateral stellectomy on
ventricular fibrillation threshold.
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.
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.
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
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.
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.
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
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
dedicates an episode to LQTS which is aired on March 25th 1998 in the USA.
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).
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
is identified on Chromosome 21. It is named MiRP1 for Min K related protein
1. It resides next to
(min K gene).
The Internet search engine
the subcategory Long QT Syndrome.
encoding the cardiac sodium channel are associated with both the
subtype of long QT syndrome and
gene is identified and associated with
International Sudden Arrhythmia Death Syndromes (SADS)
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.
electrical chaos known as
long QT syndrome Type 4, or
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
scheme for risk stratification
among patients with long-QT syndrome according to sex,
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
Sources: Peter J. Schwarz; The Long QT
Syndrome: Clinical Approaches To Tachyarrhythmias, QTsyndrome.ch
ฉ 1997 - 2007
by QTsyndrome.ch. All rights reserved.