Significant proportion of unexplained sudden cardiac deaths may represent inherited heart disease
A significant proportion of sudden cardiac
deaths that remain unexplained after autopsy and blood tests may
be due to inherited heart disease, according to findings reported
in the November 1st issue of the Lancet. The British researchers
suggest that family members of people who have an unexplained sudden
cardiac death should be evaluated themselves.
Roughly 1 in 10,000 people in England die
of sudden cardiac death. Previous research had indicated that around
4 percent of these deaths are unexplained, with postmortem results
indicating normal heart function before death. The researchers tested
the hypothesis that at least some of these deaths represent inherited
heart disease by evaluating the first-degree relatives (siblings,
children, parents) of 32 people who had such a death (average age
at death, 30 years). Of the 32 families evaluated, 7 (22 percent)
were found to have inherited electrophysiological or myocardial
disease.
William McKenna, MD, senior study author,
commented "In those families diagnosed with inherited heart
disease, there had been significantly more sudden cardiac deaths
among relatives than in the other families. If these sudden deaths
had prompted family screening, particularly of relatives with symptoms,
some of those deaths may have been prevented."
In an accompanying Commentary, Adriana Lee,
MD, and Michael J Ackerman, MD, concluded that "It now seems
reasonable to suggest a screening 12-lead electrocardiogram for
the assessment of first-degree relatives of a confirmed sudden unexplained
death. However, it will often take more than just an electrocardiogram
to unveil the cause of death. Further studies are needed to establish
the most cost-effective standard of care for the post-death evaluation
of sudden unexplained death in those left behind...With the cause
in hand, physicians can allay the greatest fear, the unknown, for
those whose loved one died suddenly, unexpectedly, and without explanation,
and be better equipped to guide those that are left behind with
informed counseling, thorough and appropriate evaluation, proper
treatment, and hopefully prevention of another loss."
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