Europace (2011) 13, 1411–1418 CLINICAL RESEARCH

doi:10.1093/europace/eur161 Sudden Death and ICDs

Sudden cardiac death in 14- to 35-year olds

in Ireland from 2005 to 2007: a retrospective
registry
Ronan Margey 1,2, Andrew Roy1, Sandra Tobin 3, Conor J. O’Keane 4,
Catherine McGorrian 1, Valerie Morris 5, Siobhan Jennings 6, and Joseph Galvin 1*
1
Sudden Cardiac Death in the Young Registry, Mater Misericordiae University Hospital, Eccles Street, Dublin 7, Ireland; 2Division of Cardiology, Massachusetts General Hospital and
Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA; 3Central Statistics Office, Cork, Ireland; 4Department of Pathology, Mater Misericordiae University Hospital,
Eccles Street, Dublin 7, Ireland; 5Royal College of Surgeons in Ireland, 123 St Stephens Green, Dublin 2, Ireland; and 6Health Services Executive of Ireland

Received 4 January 2011; accepted after revision 4 May 2011; online publish-ahead-of-print 28 July 2011

Introduction Sudden cardiac death (SCD) in young people is a rare but devastating event for families and communities. Ireland has
previously had no measure of the incidence of SCD in young people. We report the incidence and causes of SCD in
persons ,35 years of age.
.....................................................................................................................................................................................

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Methods We undertook a retrospective study of SCD between 2005 and 2007 in persons aged 15 –35 years in the Republic of
and results Ireland. We identified potential cases of out of hospital SCD through the Central Statistics Office (CSO) death cer-
tificate records. Autopsy, toxicology, and inquest reports were then obtained and analysed by an expert panel who
adjudicated on the cause of death. A total of 342 potential SCD cases were identified through the CSO. Fifty were
younger than 15 years of age, and 86 had either incomplete or unavailable post-mortem reports. Of 206 full reports
obtained, 116 were adjudicated as cases of SCD. Cases were predominantly male (75%), with a mean age of 25.8
years (standard deviation 6.3). The incidence of SCD in this age range was 2.85 per 100 000 person-years (4.36
for males and 1.30 for females) and the incidence of sudden arrhythmic death syndrome (SADS) was 0.76 per
100 000 person-years. The commonest causes were SADS, 26.7% (31 of 116), followed by coronary artery
disease, 20.7% (24 of 116), hypertrophic cardiomyopathy (HCM), 14.7% (17 of 116), and idiopathic left ventricular
hypertrophy not fulfilling criteria for HCM, 10.3% (12 of 116).
.....................................................................................................................................................................................
Conclusions The incidence of SCD in the young in Ireland was 4.96 (95% CI 3.06, 6.4) for males and 1.3 (95% CI 0.62,
2.56) for females per 100 000 person-years. Sudden arrhythmic death syndrome was the commonest cause
of SCD in the young, and the incidence of SADS was more than five times that in official reports of
the Irish CSO.
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Keywords Sudden cardiac death † Sudden arrhythmic death syndrome † Hypertrophic cardiomyopathy † Post-mortem †
Autopsy

Introduction
Sudden cardiac death (SCD) is the commonest mode of death in
the developed world, accounting for two-thirds of all cardiovascu-
lar deaths.1 – 9 International guideline groups estimate an incidence
of SCD of 36 –128 deaths per 100 000 person-years with the vast
majority of events due to coronary disease.7 – 9 In the younger age
group, previous studies have shown incidence rates of SCD ranging
from 0.93 per 100 000 person-years (in persons aged 14 –35
years)10 up to 6.2 per 100 000 person-years (in persons aged
20 –40 years).11 A recent national survey from Denmark has
described an incidence rate of SCD in 1 –35-year olds of 1.9 –
2.8 per 100 000 person-years, depending on methodology used.12
The causes of SCD in the young are diverse. While atherosclerotic
coronary artery disease (CAD) remains a significant contributor to
SCD in the young, a significant proportion of deaths are due to

* Corresponding author. Tel: +353 1 8034367; fax: +353 1 8034417, Email: joseph.galvin1@gmail.com
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2011. For permissions please email: journals.permissions@oup.com.
1412
inherited cardiomyopathies or channelopathies.13,14 This has impli-
cations for identification of these disorders in surviving relatives,
who may also be at risk of the same disease, with prior studies
suggesting that these disorders can be identified in 25 –50% of surviv-
ing relatives.15,16 In 4–5% of all SCD, a cause of death cannot be
ascertained, even after comprehensive post-mortem, toxicology
screen, and cardiac pathologic examination. These are classified as
sudden arrhythmic death syndrome (SADS).14 – 16
In the Republic of Ireland, a number of high-profile SCD events
in young persons have focused attention on this condition. A single
retrospective study in 2005 of death certificate-registered SCD in
persons aged 15 –35 years estimated an SCD incidence of 3.18 per
100 000 person-years.17 This unexpectedly high estimate has high-
lighted the need to monitor the incidence of this condition in
Ireland. In this report, we describe the incidence and causes of
out of hospital SCD in persons aged 14 –35 years in the Republic
of Ireland from 2005 to 2007. We also describe the methodologi-
cal considerations when extrapolating incidence rates from the
death registration system.
Methods
This is a retrospective death registration study of SCD in persons
aged 15–34 years inclusively.
Sampling frame
The Irish Sudden Cardiac Death in the Young Registry was set up
in 2007 by the Health Services Executive to identify all sudden
deaths in persons aged 15 –35 years in the Republic of Ireland,
through the national death registration system. The Central Stat-
istics Office of Ireland (CSO) provided a list of all deaths in
persons aged .1 year and ,35 years between 1 January 2005
and 31 December 2007, classified using the International Classifi-
cation of Diseases (ICD) system. Although all deaths should be
registered with the CSO within 3 months of the event, this is
not uniformly achieved in Ireland. Therefore, the list of registered
deaths for the specified time period includes events that occurred
outside the calendar year of 2005– 2007, respectively. Late regis-
tration of 2005, 2006, and 2007 deaths in the 2008 registration
file was not included in this analysis. Deaths in children aged
,15 years are maintained on a paediatric mortality register, and
were not included in this analysis.
Definition
Sudden death was defined as a sudden, unexpected death either
within 1 h of symptom onset (event witnessed) or within 24 h of
having been observed alive and symptom free (unwitnessed
event). Such deaths were deemed to be SCD if there was felt to
be a cardiac cause of death, or if the cause of death were unascer-
tainable after post-mortem evaluation (SADS, a subset of SCD in
this analysis). Persons with sudden death associated with electro-
cution, drowning, poisoning, toxicology suggesting drug overdose
as the cause of death, toxicology indicating presence of drugs, or
substances known to cause cardiac arrhythmias such as sympatho-
mimetics or cocaine were not included as SCD. Persons with
alcohol levels ≥100 mg/dL were not included as SCD. Although
it is recognized that these circumstances of death do not preclude
R. Margey et al.
the possibility of an underlying heart condition that resulted in a
fatal arrhythmia it was considered preferable to exclude such
deaths to avoid inclusion of cases that were possibly non-cardiac.
Case selection
From the CSO-provided list of all deaths in this age group, persons
with the following ICD codes were selected as possible cases of
SCD. International Classification of Diseases-9 codes (used in
2005 and 2006) were (i) disease of the circulatory system 390–
459.7; (ii) other heart disease 420–423; and (iii) sudden death
798–799. International Classification of Diseases-10 codes (used
in 2007) were (i) disease of the circulatory system I00-I99 and
(ii) ill-defined and unknown causes of mortality (including sudden
death) R95–99. Subjects with serious non-cardiac or terminal
illnesses were excluded. The Republic of Ireland has a coronial
system for the investigation of sudden or unexplained deaths.
For all such deaths, post-mortem evaluation is performed as
requested by the coroner, unless he or she determines that
there is sufficient evidence to determine the cause of death
without such an examination. Post-mortem results are not held
centrally in the Republic of Ireland, but rather with the investigating
coroner or pathologist. For each suspected case of SCD identified
through the CSO registration system, the relevant pathologist or
coroner was identified, and full post-mortem and toxicology
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reports were requested. Toxicology screening is a standard com-
ponent of the post-mortem evaluation. During the study period,
a small number of cases (5 of 119) deemed to require an expert
or second opinion were referred to a specialist cardiac pathologist
(Dr Mary Sheppard at the Royal Brompton Hospital, London, UK),
at the discretion of the certifying pathologist.
Confirmation of case status
Each potential case (post-mortem report, including specialist path-
ologist reviews and toxicology reports as relevant) was reviewed
by a panel to determine whether they fulfilled study criteria for
SCD. The panel included a cardiac electrophysiologist, and a cardi-
ologist with an interest in sudden death. Where consensus could
not be reached, independent review was sought from two consult-
ant pathologists with special interest in SCD, who gave a final
decision. If post-mortem details were not available on a potential
case, that case was not included in the SCD count or further
analyses.
Once SCD was confirmed on post-mortem review, the under-
lying cause was determined. Cases with no macroscopic or
microscopic evidence of cardiac disease and no potentially causative
findings on toxicological screen were classified as SADS. Hyper-
trophic cardiomyopathy (HCM) cases were classified in the
presence of left ventricular hypertrophy (LVH), with either myofi-
brillary disarray or interstitial fibrosis on histological examination.
Cases with LVH (wall thickness .15 mm or a cardiac weight
.500 g considered excessive for the subject’s size by the examining
pathologist) without myofibrillary disarray or fibrosis were
described as idiopathic LVH.
Incidence rate calculation
The CSO provided data on the population aged between 15 and
35 years from the 2006 national population census (Table 1).18
Sudden cardiac death in 14- to 35-year olds in Ireland from 2005 to 2007 1413

From this, the overall incidence of SCD and the incidence of modelling. The statistical package used was Intercooled Stata 9
specific cardiac diseases per 100 000 person-years were estimated. (StataCorp, TX, USA).
Confidence intervals were estimated using Poisson distribution
Ethical approval
Ethical approval was obtained from the Department of Health and
Table 1 Population census numbers divided by gender Children and the CSO to obtain the dataset of registered deaths
and age group adapted from the Central Statistics and the post-mortem and inquest results from the coroners and
Office of Ireland national population census 2006 pathologists throughout the country.
Age group (years) Males Females Total
................................................................................
0 –4 154556 147696 302252
Results
5 –9 147984 140341 288325
Cases identified to the Sudden Cardiac
10– 14 140504 133368 273872
15– 19 148241 142016 290257
Death in the Young Registry
20– 24 172766 169709 342475 Figure 1 outlines the case selection process of the potential cardiac
25– 29 189252 183826 373078 cases from the cases identified. For the period 2005–2007 inclus-
30– 34 177487 171874 349361 ive, there were a total of 342 cases selected from the death regis-
Total 1130790 1088830 2219620 tration files of the CSO fulfilling the ICD-9 or ICD-10 cardiac or
sudden death criteria. Fifty cases (14.6%) were in persons aged
,15 years and were omitted from the final rate calculations. Of

342 possible SCD cases identified by

CSO using death certificates

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50 omitted due to age<14yrs
(14.6%)

292 post-mortem requests of

possible SCD cases

39 post-mortems not
located (39/292, 13.4%), 90 non-cardiac cases
7 with insufficient data excluded by panel SUDEP* 24.4% (22/90)
(7/292, 2.4%), (90/292, 30.8%)
40 no post-mortem
performed (40/292, 13.7%)
Drug or Alcohol related
21.1% (19/90)

Pulmonary embolism
13.3% (12/90)

Cancer 11.1% (10/90)

Suicide 14.4% (13/90)

116 CARDIAC CASES (116/292,

Other†12.2% (11/90)
39.7%)

Figure 1 Flow diagram of case selection processes performed by the Sudden Cardiac Death in the Young Registry of potential sudden unex-
plained or cardiac deaths for 2005 – 2007 inclusive. *SUDEP, sudden unexplained death in epilepsy; †‘Other’ denotes deaths as a result of
trauma/accidents, infections, organ failure, neurologic causes, aortic dissection and deaths not otherwise categorized.
1414 R. Margey et al.

these 50 cases, 39 (66.1%) were deemed to be due to cardiac Incidence of sudden cardiac death and
aetiology on review of their death registration and post-mortem incidence of the various identified causes
data. Eleven cases were referred for adjudication by the expert
Over the period 2005– 2007, the incidence of SCD in the
panel to the pathology collaborators. Tables 2 and 3 show how
15 –35-year-old age group was 2.85 per 100 000 person-years
cases identified to the registry were classified.
(95% CI 2.05, 3.93). This equated to an incidence of SCD in males
Case status was not ascertainable for 29.5% (86 of 292) of the
of 4.36 per 100 000 person-years (95% CI 3.06, 6.4) and an incidence
possible events identified. This was because no post-mortem
of SCD in females of 1.3 per 100 000 person-years (95% CI 0.62,
evaluation was undertaken in 40 of 86 (46.5%), insufficient or unsa-
2.56). The incidence of SADS in the 15–35-year-old age group
tisfactory post-mortem details in 7 of 86 (8.1%), an inability to
was 0.76 per 100 000 person-years (95% CI 0.35, 1.40) with an inci-
ascertain the correct coroner for that event (inaccurate coroner’s
dence in males of 0.96 per 100 000 person-years (95% CI 0.41, 2.10)
jurisdiction listed by the CSO) in 18 of 86 (20.9%), or because the
and an incidence in females of 0.54 per 100 000 person-years (95%
coroner or pathologist did not forward the post-mortem details to
CI 0.16, 1.5). The incidence of sudden death due to coronary disease
the registry despite multiple requests in 21 of 86 (24.4%). These
cases have a mean age of 24.9 + 6.4 years and are 65%:35% mal-
e:female gender distribution, similar to the population included in Table 3 Demographic characteristics and cause of
our study below. Of the 79 CSO registered sudden deaths on sudden cardiac death cases
which post-mortem reports were either unavailable or not per-
formed, 20 (25.3%) were non-cardiac (as described on death cer- Variable 2005 2006 2007 Total
................................................................................
tification) and the rest were felt to be cardiac in aetiology, with 33
Number 42 35 42 116
(41.8%) being attributed to congenital heart disease (ConHD) and
Male gender 37 (88.9%) 21 (60%) 32 (76.1%) 90 (77.5%)
an additional five cases listed as SADS deaths (Table 4).
Age mean, (SD) 24.9 (6.8) 26.6 (5.7) 26 (6.4) 25.8 (6.3)
A total of 116 deaths (39.7% of the total of 292 cases available
Cause of sudden death
for examination) were considered by the panel to represent true
SADS 8 10 13 31 (26.7%)
SCDs. Most deaths occurred in males, with an average age of
HCM 10 4 3 17 (14.7%)

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25.8 years (range 15 –34) (Table 3).
CAD 7 6 11 24 (20.7%)
LVH 7 2 3 12 (10.3%)
Congenital HD 3 4 3 10 (8.6%)
Causes of sudden cardiac death DCM 0 2 1 3 (2.6%)
ARVC 1 1 0 2 (1.7%)
Causes of SCD in the population examined are shown in Figure 2
Sarcoidosis 0 3 0 3 (2.6%)
and Table 3. Figure 3 demonstrates the causes of SCD by gender
Myocarditis 3 0 4 7 (6.0%)
over the 3-year period and Figure 4 demonstrates the cause of
Anom coronary 1 1 0 2 (1.7%)
death by year of registration. The commonest finding at post-
Inf Endo 1 0 0 1 (0.8%)
mortem examination in this selected population of registered
Ao aneurysm 0 1 0 1 (0.8%)
SCDs was SADS, in 31 of 116 cases (26.7%). Other findings
MVP 1 0 0 1 (0.8%)
were CAD with evidence of acute or prior myocardial infarction
Acute rejection 0 0 1 1 (0.8%)
in 24 of 116 (20.7%), HCM in 17 of 116 (14.6%), idiopathic LVH
Duchenne MD 0 0 1 1 (0.8%)
in 12 of 116 (10.3%), ConHD in 10 of 116 (8.6%), and myocarditis
in 7 of 116(6.0%).

Table 2 Case selection and categorization by the Table 4 Death certificate causes of death in the cases in
Sudden Cardiac Death in the Young Registry which post-mortem results were either unobtainable or
not performed (total n 5 79)
Variable 2005 2006 2007 Total (%)
................................................................................ Cause of death Year of death Total
CSO identified 122 92 128 342 registration
cases ...............................
2005 2006 2007
Omitted due to 27 7 16 50 (14.6%) ................................................................................
age ,15 years
Cardiac-related causes
Potential cardiac 95 85 112 292
Congenital heart disease 22 4 7 33
cases adjudicated
Cardiomyopathy 2 2 1 5
Cardiac cases as 42 35 42 119/292 (40.7%)
adjudicated by SCDYR Ischaemia 0 3 0 3
Non-cardiac cases as 14 27 49 90/292 (30.8%) SADS/primary arrhythmia 1 1 2 4
adjudicated by SCDYR Othera 2 1 3
Incomplete PM/missing cases 39 24 23 86/292 (29.7%)
a
Other, cardiac sarcoidosis (1) and myocarditis (2).
Sudden cardiac death in 14- to 35-year olds in Ireland from 2005 to 2007 1415

Sudden Cardiac Death Ireland 2005-2007 Ages 15-34yrs incl.

Anom coronary
2% Duchenne MD Ao Aneurysm
1% 1%
Myocarditis SADS
MVP
6%
1% Inf Endo
Acute rejection SADS HCM
1%
Sarcoidosis 1% 26%
3% CAD

ARVC LVH
2%
DCM
Congenital
3%
HD
DCM

Congenital HD ARVC
9%
HCM Sarcoidosis
14%
LVH Myocarditis
10%

CAD
20%

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Figure 2 Pie-chart of causes of sudden cardiac death 2005 – 2007. SADS, sudden arrhythmic death syndrome; CAD, coronary artery disease;
HCM, hypertrophic cardiomyopathy; LVH, left ventricular hypertrophy; Congen, congenital heart disease; Myocard, myocarditis; DCM, dilated
cardiomyopathy; Sarcoid, sarcoidosis; ARVC, arhythmogenic right ventricular cardiomyopathy; Anom Cor, anomalous coronary arteries; Other,
infective endocarditis, mitral valve prolapse, acute cardiac transplant rejection, and Duchenne’s muscular dystrophy.

was 0.59 (95% CI 0.26, 1.20) per 100 000 person-years [1.01 (95% CI
0.60, 2.50) per 100 000 person-years in males and 0.14 (95% CI 0.04,
Cause of death by gender 2005-2007 0.84) per 100 000 person-years in females]. The incidence of sudden
35
Female Male
death due to HCM was 0.41 (95% CI 0.16, 0.96) per 100 000 person-
30 years [0.67 (95% CI 0.24, 1.70) per 100 000 person-years in males
25 and 0.14 (95% CI 0.04, 0.84) per 100 000 person-years in females).
20
Non-cardiac sudden death cases
15
Figure 1 outlines the selection of potential cardiac cases from the
10 overall number of deaths registered. From the cohort of potential
5 cardiac cases, on expert panel consensus, a total of 87 cases were
0 attributed to non-cardiac causes. The commonest cause of death
SADS

CAD

HCM

LVH

CONGEN

Myocard

Ao Diss

DCM

Sarcoid

ARVC

Anom Cor

Other

in these cases was sudden unexpected death from epilepsy, account-

Figure 3 Cause of sudden death by gender over the 3 years of
the study. X-axis, cause of death; Y-axis, number of cases; SADS,
sudden adult death syndrome; CAD, coronary artery disease;
HCM, hypertrophic cardiomyopathy; LVH, left ventricular hyper-
trophy; Congen, congenital heart disease; Myocard, myocarditis;
Ao Diss, aortic dissection; DCM, dilated cardiomyopathy;
Sarcoid, sarcoidosis; ARVC, arrhythmogenic right ventricular car-
diomyopathy; Anom Cor, anomalous coronaries; Other, infec-
tious endocarditis, Duchenne muscular dystrophy, aortic
aneurysm, mitral valve prolapse, acute rejection.
ing for 22 of 87 (25.2%). Drug- or alcohol- related deaths accounted
for 19 of 81 cases (23.5%). Other non-cardiac causes included pul-
monary embolism, 12 of 87 (13%), acute respiratory distress, bac-
terial endocarditis, and intracerebral haemorrhage. There were
three cases of aortic dissection, which, although included in other
reports of sudden death, we excluded from our analysis.
Circumstances and activity surrounding
sudden death
In 43% of cases (50 of 116), no details of the circumstances of
death were known. In 7.7% of cases (9 of 116), death was specified
as occurring during exertion. Five out of 17 deaths (29%) due to
1416
Sudden Cardiac Death by year and frequency
35
30
25
20
No.
15
10
EN
rd
ss
DS
M
D
ca
CA
HC
LV
Di
NG
SA
yo
Ao
CO
Cause of death
Figure 4 Cause of death by year and number. X-axis, cause of death; Y-axis, number of cases; SADS, sudden adult death syndrome; CAD,
coronary artery disease; HCM, hypertrophic cardiomyopathy; LVH, left ventricular hypertrophy; Congen, congenital heart disease; Myocard,
myocarditis; Ao Diss, aortic dissection; DCM, dilated cardiomyopathy; Sarcoid, sarcoidosis; ARVC, arrhythmogenic right ventricular cardiomyo-
pathy; Anom Cor, anomalous coronaries; Other, infectious endocarditis, Duchenne muscular dystrophy, aortic aneurysm, mitral valve prolapse,
acute rejection.
HCM occurred during exertion, with one death each occurring
while playing football, rugby, cycling, skipping, and herding cattle.
Two deaths due to SADS occurred during soccer. One death
due to an anomalous coronary artery occurred during football
and one death due to CAD occurred during basketball. The
remaining 45% (50 of 109) occurred during non-exertion or sleep.
Discussion
The incidence of SCD of 2.85 per 100 000 persons per year for
persons aged 15 –35 years and the incidence of SADS of 0.76
per 100 000 persons per year for this age group were derived
using structured data collection methodology and strict case defi-
nition. National post-mortem-based studies have previously exam-
ined the incidence and causes of SCD in the young in Sweden,
Iceland, and Denmark and the incidence of SADS in the UK. In
Sweden, Wisten et al. 10 found the incidence of SCD to be 0.93
cases per 100 000 persons per year among 15 –35 years old
between 1992 and 1999 and the commonest finding was a struc-
turally normal heart. In Iceland, Einarsson et al. 19 documented an
incidence of SCD of 1.38 per 100 000 persons per year in the
age range 12–35 years old and the commonest cause of death
was premature CAD. Winkel et al. 12 noted an incidence of SCD
of 1.9 per 100 000 cases per year in Denmark with SADS account-
ing for almost half of the deaths. A prospective study from the
Veneto region in Italy, 1979–1996, showed an incidence of
sudden death of 0.8 per 100 000 per year in persons under 35
years of age, of which nearly 75% could be attributed to cardiovas-
cular deaths, a large proportion due to arrhythmogenic right ven-
tricular cardiomyopathy.13 In the USA, a population-based study in
Olmstead County reported an incidence of 6.2 sudden deaths per
100 000 in persons aged between 20 and 40 years, with the
R. Margey et al.
2007 total
2006 total
2005 total
r
d
VC
Co
er
M
oi
DC
th
rc
AR
om
O
Sa
An
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commonest cause of death being premature CAD, although
unlike our study, it included drug-, alcohol-, and cocaine-related
deaths.11
Possible explanations for differences in rates and causes of SCD
between studies include differences in case definition, methods of
case ascertainment, post-mortem utilization rates, methodology
reporting and report availability, cardiac arrest response systems,
regional and ethnic variations in the frequency or pathogenicity
of individual gene mutations responsible for cardiac ion channelo-
pathies or cardiomyopathies as well as environmental factors such
as diet or pollution. However, international comparisons of SCD
rates are only meaningful if they compare studies with similar
methodologies and autopsy rates. In China where autopsies are
rarely performed, Hua et al. 20 documented a significantly lower
incidence of SCD in the general population through extensive
questioning of family members and collection of medical infor-
mation through the local household administrative system.
Variations in relative rates of the underlying causes of SCD are
also seen between international studies. The post-mortem diagno-
sis of HCM and its distinction from LVH for other reasons (such as
competitive sports, aortic stenosis, or hypertension) is a challenge
in retrospective studies. It is possible that cases of secondary LVH
were classified as HCM, if the LVH was associated with myocardial
fibrosis or myofibrillary disarray, and this distinction may bias the
true prevalence of HCM in our study. Population variations in
the allele frequencies of genes associated with cardiomyopathy
and ion channelopathy may also account for some of the differ-
ences in the rates and causes of SCD between countries.21 Prior
work from Finland22 and South Africa23 has identified the
concept of ‘founder gene mutation’ effect as a potential expla-
nation for the higher proportion of individuals in those geographic
areas who test positive for known long QT syndrome gene
mutations. The Irish population is genetically homogenous and is
Sudden cardiac death in 14- to 35-year olds in Ireland from 2005 to 2007
well known to have high frequencies of gene carriage for inherited
conditions such as haemochromotosis and cystic fibrosis.24,25 One
putative explanation for this apparent international variation in
disease incidences may be a similar ‘founder gene mutation’
effect. Comparison of SADS rates and specific gene mutation
rates between different countries would be a major undertaking
but would be helpful in giving a more complete picture of the con-
tribution of genetic disease not just to SCD in young people but
also potentially to determining vulnerability to lethal ventricular
arrhythmias in later life in the setting of ischaemic heart disease.
This study highlights the many challenges in estimating SCD and
SADS events from routine national data collection. Similar to the
Behr study, the incidence of SADS is higher than that available
from the official national statistics service. Behr et al. 14 estimated
the incidence of SADS mortality at 0.16 per 100 000 persons
per annum compared with 0.10 per 100 000 persons per annum
from the National Office for Statistics. Using our study method-
ology, we estimated a SADS incidence rate for 2006 which was
five times higher than that estimated using national CSO coding
alone (0.14 deaths per 100 000 persons per year). Indeed, SADS
cases were varyingly coded as ‘unascertained’, conduction disorder
(426), cardiac dysrhythmia (427), heart failure (428), ill-defined
descriptions and complications of heart disease (429),
Wolff-Parkinson-White, and ‘special symptoms of syndromes not
classified elsewhere’. Again in 2007, 11 out of 13 SADS cases
(84%) were assigned incorrectly, with at least four different
codes listed. Currently, the physician, coroner or pathologist cer-
tifying the cause of death issues an official certificate which is
then electronically transcribed to an online database by a non-
medical administrative staff member. From the electronic free
text version of the written cause of death, a dedicated team of
ICD-coding staff apply an appropriate ICD code. However, due
to the natural variation in the terms used to describe the cause
of death, differing codes can be applied to same diagnosis. This
suggests the need for the implementation of immediate coding of
the cause of death by the physician/coroner/pathologist attesting
to the cause of death rather than remote coding by non-medical
personnel.
We propose that prospective data collection for cases of pre-
sumed SCD in young people, with timely acquisition of demo-
graphic, clinical, and pathologic information, represents the best
possibility of increased precision in estimates of SCD and SADS.
Furthermore, this would also facilitate appropriate cardiac screen-
ing of first-degree family relatives without undue delay. The use of
clear case definitions, a standard SCD post-mortem procedure,
with molecular and genetic tissue analysis, and accurate ICD
coding at source when death occurs will result in prompt detec-
tion of first-degree family members with a 14 –20% , as well as pro-
viding more accurate epidemiological data. In other jurisdictions
such as Denmark, national medical registries allow relatively easy
access to a patient’s pre-mortem medical records and post-
mortem reports, compared with Irish access, which depends on
the co-operation of some 70 coroners and pathologists, some
with very limited support staff and resources. Most countries
have their own local difficulties in terms of data collection in
cases of SCD requiring investigators to create innovative local sol-
utions. In possible SADS cases where doubt or difficulty exists,
1417
then expert cardiac pathological and electrophysiological opinion
should be sought. Previously, Irish pathologists would consult
with an international expert on cardiac pathology, and this
occurred in 5 of 204 of the post-mortems reviewed. Through
experts in the Faculty of Pathology, and the national SCD in the
Young Taskforce, specialized guidelines for post-mortem evalu-
ations in cases of SCD are now available for Irish pathologists.
Study limitations
The authors acknowledge that a major limitation of this study is the
lack of adequate or available autopsy reports in 86 of 292 cases of
death among the target population. Indeed, 40 of 292 of these
missing cases did not actually undergo autopsy examination.
Despite multiple requests for the other 39 reports, these missing
cases were ultimately not available for inclusion in the analysis.
The likely bias resulting from the missing data is an underestimation
of the true incidence of SCD in our population. This has been
addressed for future surveys by planned prospective collection
of data and by raising awareness among coroners and pathologists
through the national Faculty of Pathology.
We used the CSO classification of causes of death for the initial
identification of potential cases. This caused inclusion of inap-
propriate (non-cardiac) cases, and therefore possible exclusion
of some relevant cases because of mis-coding. We have minimized
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the former by detailed analysis of all cases identified and the latter
by using as broad a ‘net’ of ICD codes as possible to capture cases.
Post-mortem performance guidelines are available through the
Faculty of Pathology, Royal College of Physicians in Ireland.
However, there is no audit system in place to ensure that such
guidelines are followed. Post-mortem reports in our registry
were issued by over 30 different pathologists, and report quality
was variable. We therefore retained only those post-mortem
reports which had both gross macroscopic and microscopic
descriptions of the heart. Again this would likely lead to an under-
estimation of disease burden of SCD and SADS in the young. We
excluded all cases of sudden death that occurred in the setting of
alcohol excess, the presence of positive blood levels of drugs of
abuse, a history of epilepsy, drowning, or road traffic accidents.
Detailed analysis of such cases to identify underlying heart
disease which may have led to a fatal arrhythmia was beyond the
scope or resources of this registry; however, we acknowledge
that some of these cases may have had an underlying arrhythmic
aetiology.
Due to concerns over the right to privacy, both the CSO and the
Data Protection Commissioner precluded the collection of infor-
mation on pre-mortem electrocardiograms or hospital charts or
access to family history unless the pathologist made reference to it
in his report. Furthermore, in providing the ICD code data, the
CSO expressly stipulated that the data could not be used to make
contact with the families of victims. Therefore, we did not have
access to any subsequent family screening outcomes.
Our registry is restricted to cases of SCD in 15–35-year olds,
making comparative analyses with younger age groups impossible.
Data on survivors of out of hospital cardiac arrest in the age group
were not included. At present, there is no central registry or linked
database to allow reliable collection of such data nationwide. Mol-
ecular autopsy was not used in any of the cases of SADS in this
1418
registry, and we did not have ethical permission to contact families
for further clinical information on either the victim or other family
members. As a result, no further clarification of underlying channe-
lopathy diagnoses can be included in the SADS cases.
Conclusion
The incidence of SCD in Ireland was 4.36 per 100 000 person-
years (95% CI 3.06, 6.4) for males and 1.3 per 100 000 person-
years (95% CI 0.62, 2.56) for females. The commonest finding at
autopsy was a structurally normal heart followed by CAD
and HCM.
The incidence of SADS after careful examination of postmortem
and toxicology reports was more than five times the incidence
reported by the Irish CSO.
Acknowledgements
We thank the Faculty of Pathologists and the Coroner’s Society of
Ireland and their respective members for their co-operation and
provision of post-mortem materials to the registry and we also
thank the Central Statistics Office, Sudden Infant Death Register,
and Out-of-Hospital Cardiac Arrest Registry for their assistance.
Funding
Funding for the National Sudden Cardiac Death in the Young Registry
has been provided by the Mater Foundation and the Health Service
Executive of Ireland.
Conflict of interest: None of the authors report any conflicts of
interest.
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