316

CARDIOVASCULAR MEDICINE

Sudden adult death syndrome and other non-

ischaemic causes of sudden cardiac death
A Fabre, M N Sheppard
...............................................................................................................................
Heart 2006;92:316–320. doi: 10.1136/hrt.2004.045518

Objective: To evaluate non-atherosclerotic cardiac deaths in the UK population aged over 15 years
See end of article for
authors’ affiliations including elderly patients and to highlight the concept of the structurally normal heart in sudden death.
....................... Methods: Pathological data were collected prospectively for sudden adult deaths referred by UK coroners.
Results: 453 cases of sudden death from 1994 to 2003 (278 men (61.4%) and 175 women (38.6%), age
Correspondence to:
Dr Mary N Sheppard, range 15–81 years) were reviewed. Males predominated in both age groups (( 35 years, . 35 years).
Department of Pathology, More than half of the hearts (n = 269, 59.3%) were structurally normal. In the other 40.7%, cardiac
Royal Brompton Hospital, abnormalities were noted, which included: (1) cardiomyopathies (23%) such as idiopathic fibrosis, left
Sydney Street, London
SW3 6NP, UK; ventricular hypertrophy, hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic
m.sheppard@rbh. right ventricular dysplasia; (2) inflammatory disorders (8.6%) including lymphocytic myocarditis and
nthames.nhs.uk cardiac sarcoidosis; (3) non-atheromatous abnormalities of coronary arteries (4.6%); (4) valve diseases;
and (5) miscellaneous and rare causes.
Accepted 13 May 2005
Published Online First Conclusion: The concept of the structurally normal heart in sudden death and the need for histological
27 May 2005 examination to detect underlying disease is highlighted. Relatives need to be referred for cardiological and
....................... genetic screening in cases of normal hearts found at necropsy.

S
udden adult cardiac death is caused by ischaemic heart METHODS
disease in the vast majority of cases.1 In previous UK From January 1994 to April 2003, all cases of sudden adult
series this cause ranged between 59–86% of sudden cardiac deaths referred to us from coroners throughout the
death cases in the community.2 3 Sudden unexpected cardiac UK were entered on a prospective database. All the patients
death in the community in which no cause can be found at a had been well until their sudden death with no history of
coroner’s postmortem examination is increasingly recog- heart disease, apart from the congenital cases and a history of
nised. The proportion of unexplained deaths in one of the hypertension (elicited from the general practitioner’s notes
earliest studies in Wandsworth in 1988 was 3.4%.2 In the first after left ventricular hypertrophy (LVH) was reported
national prospective study of sudden death, funded by the pathologically). Toxicology (reports were provided by the
British Heart Foundation in the early 1990s, a very similar coroner) was negative for all patients. Details concerning
figure of 4.1% of unexplained deaths was reported after other diseases were obtained from the coroners once they had
detailed examination by three cardiac pathologists.3 Both been in contact with patients’ general practitioners. For this
these studies advocated identifying these cases by a name, study, ischaemic heart disease cases were excluded.
the sudden adult death syndrome (SADS), to highlight the Histological sections from the myocardium (51% of cases,
problem and deal with it in a similar fashion to sudden infant between 2–10 sections for each case), a single myocardial
death and to study the aetiology systematically. The concept transverse section of both ventricles (2%), or whole hearts
of the morphologically normal heart in sudden death is of (47%) were referred by pathologists with permission
major importance with the emergence of the molecular obtained from the next of kin. The patient’s age, sex, weight,
channelopathies such as the long QT or Brugada’s syndrome and height (when provided), heart weight, thicknesses of the
giving rise to lethal cardiac arrhythmias in the past 15 years.4 left and right ventricles, and overall description were
Non-ischaemic causes of sudden cardiac death are of major recorded. Where a patient’s height and weight were not
importance because they often include genetic diseases, available, normal cut off parameters for heart weight were
such as hypertrophic cardiomyopathy (HCM), dilated cardio- 500 g for men and 400 g for women and for left ventricular
myopathy, and arrhythmogenic right ventricular dysplasia thickness, 15 mm. Selected sections were stained with elastic
(ARVD).5 After the initial British Heart Foundation study van Gieson to assess fibrosis.
we acted as a referral centre for sudden cardiac death cases Histological criteria were as follows: for HCM, myocyte
from coroners throughout the UK and have now completed disarray, interstitial fibrosis, and vascular changes of small
an analysis of the cases referred for a pathological opinion, arterioles (thickening of the wall); for ARVD, fat and fibrosis
often because the referring pathologist did not find a throughout the wall of the right ventricle with or without
cause of death or was uncertain of the cause of death. The chronic inflammatory infiltrate; for LVH, hypertrophied
patients were all older than 15 years, and atherosclerotic myocytes with no fibrosis; for idiopathic fibrosis, widespread
coronary artery disease (coronary artery stenosis, with or fibrosis in the left ventricle with no evidence of ventricular
without acute or old myocardial infarction or fibrosis) wall thinning; and for dilated cardiomyopathy, a thin walled
was excluded as a cause of death. This is the first path-
ological study to evaluate non-atherosclerotic cardiac deaths Abbreviations: ARVD, arrhythmogenic right ventricular dysplasia;
in the UK population aged over 15 years including elderly HCM, hypertrophic cardiomyopathy; LVH, left ventricular hypertrophy;
patients. SADS, sudden adult death syndrome

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Normal heart in adult sudden death 317

left ventricle with fibrosis throughout the left ventricle with Interestingly, one patient had associated diffuse lymphocytic
no coronary artery disease. The final pathological diagnosis myocarditis.
was noted and results were categorised as follows: (1) normal ARVD was observed in 10 patients, six males and four
heart (with or without associated diseases); (2) cardiomyo- females, and six of 10 cases of ARVD were diagnosed in the
pathies; (3) myocarditis; (4) non-atheromatous abnormality ( 35 age group. Seven were described as having fatty
of the coronary arteries; (5) valvar diseases; and (6) other replacement of the right ventricle macroscopically, one had a
abnormalities including disorders of the conduction system. thin right ventricle wall, and two appeared macroscopically
normal. ARVD was seen in association with diffuse lympho-
RESULTS cytic myocarditis in one patient. Seven additional cases with
We retrieved 453 cases of sudden cardiac death from the fatty infiltration of the right ventricle were referred, but we
database for 278 men (61.4%) (median age 32 years (range had only limited material for all cases (slides only, no whole
15–81)) and 175 women (38.6%) (median age 31 years hearts) and ARVD could not be diagnosed with certainty
(range 15–75)). Two hundred and twenty three (49.2%) were because of this limitation. Dilated cardiomyopathy was
aged ( 35 years (75 women, 148 men) and 230 (50.8%) were diagnosed in nine cases, with a female predominance (seven
aged . 35 years (table 1). of nine). In the . 35 age group, all patients were female. In
retrospect we found five patients had associated heavy
alcohol intake, one died after childbirth, one had diabetes
Normal hearts mellitus, one had acute thyrotoxicosis, and one died after
Hearts were found to have normal macroscopic and micro- chemotherapy. For one patient, a positive family history for
scopic appearances in 269 cases (59.3%; 162 men, 107 dilated cardiomyopathy had been reported.
women), indicating true SADS. Patients 35 or younger
(53.5%) and patients older than 35 (46.5%) (table 1) were
Myocarditis
equally distributed. Males predominate in both age groups. In
Thirty nine patients had myocarditis (two already mentioned
this group of patients with morphologically normal hearts,
in association with HCM and ARVD) accounting for 8.6% of
13% had reported diseases known to predispose to sudden
the studied hearts (table 3). Lymphocytic myocarditis, which
death. These were epilepsy (n = 11), alcoholic fatty liver
was shown by lymphocytes surrounding necrotic myocytes in
(n = 9), anorexia (n = 5), asthma (n = 4), diabetes
at least two foci in each block of tissue examined, was
mellitus (n = 4), and schizophrenia (n = 3).
diagnosed in 24 patients and most of these had macro-
scopically normal hearts in which histological analysis
Cardiomyopathies showed the cause of death. The majority of cases were found
Abnormalities of the myocardium were found with certainty in patients aged ( 35 (16 of 24) and of those, 12 were male.
in 107 patients, accounting for 24% of all cases (table 2). Granulomatous myocarditis, in which there were well formed
Tissue sampling from seven patients (as described below) granulomas without any eosinophilic infiltrate (cardiac
was insufficient for definite diagnosis. Males predominated sarcoidosis), was observed in 10 patients, all aged . 35,
in both age groups. LVH and idiopathic diffuse cardiac with a female predominance (eight of 10). Toxic myocarditis
fibrosis made up 55% of the total cardiomyopathy group. LVH was diagnosed in five patients by an infiltration of
without fibrosis or disarray was the most common abnorm- predominantly macrophages and eosinophils in the inter-
ality, found in 31 patients, with males predominating in both stitium (attributed to drugs in two cases and thyrotoxicosis in
age groups. Associated diseases found in 14 patients were one case).
hypertension (n = 9), including four patients (four males,
three aged less than 35) reported to be of Afro-Caribbean Non-atheromatous abnormality of the coronary
origin, and aortic stenosis (n = 5). Idiopathic diffuse left arteries
ventricular cardiac fibrosis was similarly seen in 29 patients Non-atheromatous coronary artery diseases were observed in
(6.4% of the whole series, 27% of the cardiomyopathy group). 21 patients (4.6%) (table 4). Anomalous coronary arteries
The majority were men (18 of 29 cases) with equal were observed in six patients with both coronary arteries
distribution in both age groups. In 15 of 29 cases, the heart arising from the same coronary ostium in five cases and an
was macroscopically normal with no evidence of thinning or absent right coronary artery in one case. Five of six patients
scarring. In the remaining 14, the heart was hypertrophied. were aged ( 35 years. Coronary spasm was diagnosed in six
No history of hypertension was obtained for these patients. patients, where regional ischaemic damage including con-
HCM was the third most common disease of the myocardium traction band necrosis (three of six) or regional acute
in 28 cases. While 23 patients had the classic hypertrophied transmural or chronic myocardial infarction (three of six)
left ventricle, in five the heart was macroscopically normal. was observed in association with normal coronary arteries.
Males also predominated with this disease (21 of 28 cases). Cases were found in both age groups. Bridging of the left
Cases were equally distributed between both age groups but anterior descending coronary artery by muscle was system-
more women had the diagnosis after the age of 35. atically looked for and observed in four patients, three being
in the ( 35 year age group. The bridge varied from 20–
Table 1 Age and sex distribution of the cohort and 40 mm in length and 2–5 mm in depth. Coronary artery
constitutively normal hearts vasculitis was found in three patients (one associated with an
inflammatory pseudotumour of the kidney with IgG para-
(35 years .35 years Total proteinaemia, one case of eosinophilic vasculitis with possible
Overall Churg-Strauss syndrome, and one case of giant cell arteritis
F 75 100 175 (38.6%) associated with giant cell aortitis). Both cases of spontaneous
M 148 130 278 (61.4%) dissection of the coronary arteries were found in males.
Total 223 230 453
Normal hearts (59.3%)
F 52 55 107 (39.8%) Valvar abnormality
M 92 70 162 (60.2%) Valvar abnormalities as a cause of death were nine floppy
Total 144 125 269 mitral valves, one cleft mitral valve, aortic stenosis with
bicuspid aortic valve in four cases and degenerative trileaflet
F, female; M, male.
aortic valve in one case.

www.heartjnl.com
318 Fabre, Sheppard

Table 2 Abnormality of the myocardium with a diagnosis of certainty by age and sex
(35 years .35 years
Percentage Percentage
No of group of cohort M F M F

LVH 31 28% 6.8% 11 0 15 5

IF 29 27% 6.4% 8 5 10 6
HCM 28 26% 6.2% 13 2 8 5
ARVD 10 9% 2.2% 4 2 2 2
DCM 9 8% 1.9% 2 3 0 4
Total 107 100% 24% 38 12 35 22

ARVD, arrhythmogenic right ventricular dysplasia; DCM, dilated cardiomyopathy; HCM, hypertrophic
cardiomyopathy; IF, idiopathic fibrosis; LVH, left ventricular hypertrophy.

Other abnormalities reports on sudden cardiac deaths due to non-atheromatous

Conduction system or to no discernable cause. Reports of non-atheromatous
The conduction system was examined in seven cases. There causes of sudden cardiac death tend to concentrate on a
was one case of fat and fibrosis in the atrioventricular node of limited age group of patients younger than 35–40, including
a 43 year old woman who had a documented history of athletes.6–9 The current study differs in that it explored
arrhythmias during life (paroxysmal supraventricular tachy- sudden cardiac death in a wider age group (15–81 years) and
cardia, atrial fibrillation, and complete heart block), one case described a large proportion of macroscopically and micro-
of tuberous sclerosis in a 41 year old woman with multiple scopically normal hearts and non-atheromatous causes of
ventricular lipomata and a lipoma in the atrioventricular sudden cardiac death in which pathologists were uncertain of
node, and two cases of lipomatous hypertrophy of the the cause of death or wished for confirmation of the
interatrial septum, one associated with fatty infiltration of diagnosis.
the atrioventricular node. In three cases of cardiac sarcoi- Almost 60% of our patients had a macroscopically and
dosis, epithelioid and giant cell granulomas were found in microscopically normal heart with an equal distribution
the atrioventricular node. between patients 35 and younger and patients older than 35,
emphasising that all age groups and not just the young or
Congenital heart disease athletes can die suddenly. The male predominance is also
There were six cases of surgically corrected complex remarkable. This group is much larger than that of the Italian
congenital heart diseases (two repaired atrial septal defects study of 273 young patients who died suddenly (( 35 years
with right ventricular hypertrophy and LVH, one repaired old), of whom 16 had a normal heart, giving a rate of 6%.8 A
tetralogy of Fallot, one repaired ventricular septal defect with 21% rate of normal hearts in unexplained death was observed
right ventricular hypertrophy, two more complex cases with in a Swedish study9 in the same age group. In a French study
dextrocardia in one patient and corrected atrioventricular in 1996 of 1000 sudden death necropsies of adults under 65
septal defect in another). All these patients had been years of age, 12.3% had normal hearts.10 In an American
operated on many years previously and there was no series of 14–40 year olds, 15.8% had normal hearts.6 We
indication of clinical deterioration before sudden death. At studied all our cases less than 40 years for comparison and
necropsy, apart from the surgical corrections and congenital showed that 60% had a normal heart. The prevalence of
anomalies, there were no specific new findings to explain the hearts with ‘‘no finding’’ decreased with age in the study of
sudden death in each case. The pathologists had referred Virmani et al7 in 2001: 30% in the 14–20 age group, 21% in
them because of their complexity and surgical correction. 21–30 age group, and 9% in the 31–40 age group. In our
series, almost half of the normal hearts were from patients
Miscellaneous older than 35 years, a finding not established in the
Very rare causes of sudden death were idiopathic thrombotic literature, which has in the past emphasised sudden death
thrombocytopenic purpura associated myocardial necrosis in younger age groups. The high proportion of normal hearts
(n = 2), lipoma in the left coronary ostium (n = 1) causing in the present study may be explained by the selective and
obstruction of the left main stem, small benign tumour biased referral pattern by pathologists who found nothing at
(atrioventricular nodal mesothelioma) (n = 1), and meta- necropsy or were uncertain of the cause of death; another
static adenocarcinoma to the myocardium (n = 1). explanation may be the poor sampling in some cases (as seen
for seven cases of suspected ARVD) and the fact that slides
DISCUSSION rather than whole hearts were referred in 51% of cases with
A substantial proportion of people experience sudden death limited sampling of the right ventricle.
as the first and only clinical expression of underlying
coronary artery disease and pathologists are familiar with
this in their coronial practice. However, there are few large
Table 4 Non-atheromatous abnormality of the coronary
arteries by age and sex
Table 3 Inflammatory diseases of the myocardium by
age and sex (35 years .35 years

(35 years .35 years M F M F Total

M F M F Total Anomalous coronary arteries 4 1 1 0 6

Spasm 2 2 1 1 6
Lymphocytic myocarditis 12 4 1 7 24 Bridging of the LAD 2 1 1 0 4
Granulomatous myocarditis 0 0 2 8 10 Vasculitis 0 2 1 0 3
Toxic myocarditis 1 0 1 3 5 Spontaneous dissection 1 0 1 0 2

LAD, left anterior descending coronary artery.

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Normal heart in adult sudden death 319

Sudden death with a morphological normal heart is a very Normally we do not check the conduction system in
important negative finding at necropsy.3 Genes have been sudden cardiac death. Abnormalities including abnormal
identified for several disorders responsible for arrhythmias conduction bundles, fibrosis, and fatty infiltration have been
and sudden death. These genes all encode ion channels and described but their role in causing death is controversial.20 In
are referred to as channelopathy genes. The proteins that the current study the conduction system was examined only
regulate electrical activity are not detectable morphologically if clinically indicated, in particular if the patient was know to
at the time of postmortem examination. A diagnosis can only have arrhythmias during life, and this yielded positive
be made by ECG investigation during life.11 The occurrence of findings. Interestingly the Italian study reported 24 cases of
sudden death with a normal heart should therefore prompt conduction system diseases and most of the patients had
referral of close relatives to a specialist cardiologist for genetic clinical evidence of arrhythmias during life.8
screening. In a recently completed study of 147 first degree Many other studies confirm anomalous coronary arteries
relatives of 32 people who died of SADS, 109 (74%) and bridging as a cause of sudden death.21 22 The role of
underwent cardiological assessment; the diagnoses were coronary artery spasm is more controversial but has been
inherited cardiac disease in seven (22%) of the 32 families linked to sudden death and survival after cardiac arrest.23
and the long QT syndrome in four.12 This study emphasises Vasculitis can affect the coronary arteries locally. This
that it is important to screen families for genetic conditions in emphasises that detailed study of all of the coronary arterial
sudden cardiac death cases. However, although we system- system is essential in cases of sudden cardiac death. Lastly,
atically advise genetic screening of relatives in cases of we included congenital heart disease because it has an
sudden adult death with a morphologically normal heart, one established link to fatal arrhythmias in the absence of
limitation of the current study is the lack of genetic results or symptoms and clinical deterioration in which no new
information of family history in this group of patients with findings are seen.24
structurally normal hearts. We need to study these cases in
more detail. Conclusion
The proportion of patients with sudden death with a The non-atheromatous causes of sudden cardiac death
normal heart and associated diseases was 13% in our series. present a diagnostic challenge for coroners and pathologists.
Sudden death has also been reported in epilepsy without A normal heart is an important negative finding in the
clinical evidence of status epilepticus,13 in asthma without investigations, as it warrants referral of living relatives to a
clinical evidence of status asthmaticus,14 in anorexia,15 in specialist cardiologist and genetic screening. As macro-
schizophrenia,16 and in patients with alcoholic fatty liver scopically normal hearts can have microscopic disease,
without an alcoholic cardiomyopathy.17 As arrhythmias have histological analysis is mandatory to make a specific
been documented during life in patients with these condi- diagnosis. Today, with many questioning the role of the
tions, cardiac arrhythmias are thought to be the mode of necropsy in modern medicine,25 this study emphasises its
death. This 13% rate is by no means accurate and is probably importance and central role in helping families come to terms
an underestimate due to lack of detailed clinical data in each with death in a previously healthy relative. Detailed and
case. We need to study the clinical history of all these cases precise cardiac findings will be important for these families in
further. terms of health screening, genetic counselling, health
Normal hearts macroscopically can show microscopic insurance, and treatments such as drugs and implantable
abnormalities as our study also highlights. In the Italian defibrillators.
study8 among the 28% of the hearts that were macroscopi-
cally normal, histological examination of 79% disclosed ACKNOWLEDGEMENTS
concealed pathological substrates, as also described in a We thank all coroners in the UK who participated in this study and
recent French study (of 1930 unexplained sudden deaths, 200 Mrs Anne Marie Campbell for secretarial assistance.
had pathological evidence of ARVD18), emphasising that
histological examination and sampling are essential even in .....................
normal appearing hearts. Authors’ affiliations
A Fabre, M N Sheppard, Pathology Department, Royal Brompton and
All types of cardiomyopathies including HCM, dilated
Harefield NHS Trust, London, UK
cardiomyopathy, and ARVD have an underlying genetic
mutation in many cases. Cardiomyopathies were responsible
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IMAGES IN CARDIOLOGY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
doi: 10.1136/hrt.2005.069518
Left subclavian artery and left anterior descending coronary artery stenoses: obstacles to the left
internal mammary artery

A
77 year old man, having previously
undergone triple vessel coronary artery
bypass (CABG) surgery with a left
internal mammary graft (LIMAG) to the left
anterior descending (LAD), saphenous vein
grafts (SVG) to the obtuse marginal (OM)
and circumflex (Cx) branches, presented
with an anterior acute coronary syndrome.
Coronary angiography revealed an occluded
Cx-SVG graft and patent OM-SVG. There
was considerable difficulty in selectively
intubating the LIMAG; suboptimal images
suggested complete occlusion of a relatively
small calibre LAD beyond the insertion of the
LIMAG (panel A). A subsequent pullback
pressure gradient (60 mm Hg) across the left
subclavian artery (LSCA) and arch aorto-
gram revealed a significant LSCA stenosis
(panel B). At this stage the LSCA stenosis
was dilated with an 8 mm diameter balloon
by a vascular radiologist, enabling selective
catheterisation of the LIMAG and enhanced
visualisation of the LIMAG-distal LAD (A) Initial appearance of the left internal mammary graft-left anterior descending (LIMAG-LAD)
(panel C). It was then apparent that there system with a non-selective injection suggesting an occluded LAD beyond the graft anastomosis
was subtotal occlusion of the LAD with (arrow). (B) Subsequent arch aortogram demonstrating a significant flow limiting left subclavian
minimal antegrade flow. In light of the artery (LSCA) stenosis (arrow).
clinical presentation, the LAD was recana-
lised by percutaneous transluminal coronary
angioplasty (PTCA) and stenting via the
LIMAG (panel D). The patient has remained
asymptomatic since. This case highlights the
importance of pursuing the acquisition of
selective images of all vessels at the time of
coronary angiography particularly in CABG
patients since, in this instance, discovery and
then treatment of the LSCA stenosis
undoubtedly improved flow and hence
visualisation of the LIMAG-LAD system.
This certainly facilitated successful PTCA of
the culprit LAD lesion which led to symptom
resolution.

S Patel
J Coltart
T Sabharwal
[email protected]
We, the authors, wish to state that there are no
competing interests/no conflicts of interest
(C) Successful balloon dilatation of the LSCA stenosis. (D) Final injection of the LIMAG following
coronary angioplasty and stenting of the LAD.

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