Review Article
Sudden Cardiac Death in Hypertrophic Cardiomyopathy
Daria M. Adamczak, MD and Zofia Oko-Sarnowska, MD, DSc
Abstract: Hypertrophic cardiomyopathy (HCM) is a heart disease char-
acterized by hypertrophy of the left ventricular myocardium and is most
often caused by mutations in sarcomere genes. The structural and functional
abnormalities are not explained by flow-limiting coronary artery disease
or loading conditions. The disease affects at least 0.2% of the population
worldwide and is the most common cause of sudden cardiac death in young
people and competitive athletes because of fatal ventricular arrhythmia.
In some patients, however, HCM has a benign course. Therefore, it is of
utmost importance to properly evaluate patients and single out those who
would benefit from an implanted cardioverter defibrillator. In this article,
we review and summarize the sudden cardiac death risk stratification algo-
rithms, methods of preventing death due to HCM, and novel factors that
may improve the existing prediction models.
Key Words: hypertrophic cardiomyopathy, sudden cardiac death, HCM risk-
SCD calculator, risk stratification, new prediction factors
(Cardiology in Review 2018;26: 145–151)
H ypertrophic cardiomyopathy (HCM) is a heart disease char-
acterized by the presence of increased left ventricular (LV)
wall thickness of various morphologies and a wide spectrum of
clinical and hemodynamic manifestations. The structural and
functional abnormalities of the ventricular myocardium are not
explained by inappropriate loading conditions or coronary artery
disease.1
ETIOLOGY OF HCM
Up to 60% of HCM cases in adolescents and adults are
caused by mutations in cardiac sarcomere protein genes with an
autosomal dominant inheritance pattern. At least 15 causative
genes and over 1500 mutations have been reported to date.2 Muta-
tions in the genes encoding beta-myosin heavy chain and myo-
sin-binding protein are the most common. Other affected genes
include those encoding for cardiac troponin I and T, tropomyosin
alpha-1 chain, and myosin light chain 3.3 Approximately 5–10%
of adult cases are caused by other genetic disorders–metabolic
(eg, Anderson-Fabry disease), neuromuscular and mitochon-
drial diseases, and malformation syndromes (eg, Noonan syn-
drome).4 Nongenetic disorders can mimic the genetic forms of
HCM in some patients. Hypertrophy is observed in amyloidosis,
newborns of diabetic mothers, or very rarely, it can be induced
by drugs such as tacrolimus, steroids, or hydroxychloroquine.5,6
Nevertheless, the etiology of the disease is still unknown in up
to 30% of cases.
From the Department of Cardiology, Poznan University of Medical Sciences,
Poznan, Poland.
The authors declare no conflict of interest.
Correspondence: Daria M. Adamczak, MD, Department of Cardiology, Poznan
University of Medical Sciences, Clinical Hospital No. 1, Dluga ½, 61–848,
Poznan, Poland. E-mail: daria.m.adamczak@gmail.com.
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 1061-5377/18/2603-0145
DOI: 10.1097/CRD.0000000000000184
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Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
PREVALENCE OF HCM
According to echocardiographic studies, the worldwide preva-
lence of HCM in the general population is estimated to be 0.2%.7–9
However, newer techniques, such as genetic testing and cardiac mag-
netic resonance (CMR), have improved diagnostic rates.10,11 More-
over, the past epidemiologic studies have not considered that family
members of the identified probands may also be affected because of
the usually autosomal dominant inheritance pattern of the disease.
Therefore, the newest data suggest that the prevalence of HCM is
closer to 0.5%.12
DIAGNOSIS OF HCM
The diagnosis of HCM is usually established by performing
an echocardiographic study. However, HCM is defined by a maxi-
mum ventricular wall thickness ≥15 mm in at least 1 segment of the
LV measured by any imaging technique. In children, the diagnosis
of HCM requires a wall thickness of the LV more than 2 standard
deviations greater than the predicted mean for the child’s age group
(z-score > 2).13
Histologic findings in patients with HCM reveal hypertro-
phied myocytes with bizarre-shaped nuclei and myocardial disarray.
In addition, the intramural coronary arterioles are structurally abnor-
mal, and their lumen and vasodilatory capacity are decreased. These
factors lead to small vessel ischemia, and subsequently to replace-
ment fibrosis, which is a substrate for arrhythmias.14
CLINICAL PRESENTATION
Left ventricular hypertrophy (LVH) most commonly develops
during adolescence; however, it can also occur in infancy and child-
hood. The disease is considered to be late onset when it is recognized
between 20 and 50 years of age.15 Male patients with HCM have a
3:2 predominance over female patients; however, female patients are
older, more symptomatic, and more frequently have LV outflow track
(LVOT) obstruction upon diagnosis. Female HCM patients also show
a higher risk of progression to advanced heart failure (HF).16
CLINICAL COURSE
The majority of HCM patients have no or only minor symp-
toms. This group has a significantly better prognosis than those with
more severe symptoms. Patients with mild-to-moderate symptoms
may have a stable clinical course by means of effective treatment
or experience a slow progression with advancing age. In a study of
almost 300 American patients with HCM, 90% were asymptomatic
at presentation. After 8 years of follow-up, 69% of these patients
remained in a good condition, whereas 25% of them had progressive
symptoms or died.17,18
CLINICAL MANIFESTATIONS
The site and extent of LVH have a significant impact on the
range of experienced HCM-related symptoms. Patients can develop
one or more of the following structural and functional abnormali-
ties: LVOT obstruction, diastolic dysfunction, systolic dysfunction
(so called “burned-out” phase of HCM), myocardial ischemia, and
mitral regurgitation. These abnormalities can lead to the wide array
Adamczak and Oko-Sarnowska Cardiology in Review  •  Volume 26, Number 3, May/June 2018
of symptoms experienced by patients and can be divided into 3 major
groups as being related to HF, chest pain, and arrhythmias.19–22
Arrhythmias
Both supraventricular and ventricular arrhythmias are com-
mon in patients with HCM; however, the spectrum of clinical mani-
festations varies significantly. Patients can experience no symptoms
but may also have palpitations, presyncope, syncope, or even sudden
cardiac arrest. The rate of paroxysmal supraventricular arrhythmias
among the HCM patients documented in ambulatory electrocardio-
graphic monitoring was 38%, whereas the rate of premature ven-
tricular beats was as high as 88%, while nonsustained ventricular
tachycardia (VT), according to different studies, ranged from 15% to
31%.23–26 Clinically documented sustained VT is uncommon among
HCM patients. Episodes of nonsustained VT usually occur in older
patients and in times of heightened vagal tone. However, if they
do happen in younger patients, they may presage potentially lethal
arrhythmias such as sustained VT and ventricular fibrillation.26–28
LIFE SPAN AND MORTALITY OF HCM PATIENTS
The majority of patients with HCM are considered to have a
normal life expectancy. In a study of 312 patients, 23% lived at least
to 75 years of age and 14% even to 80. In this group of long-term
survivors, 64% experienced no or only mild symptoms. Most of them
were women.29 In a series of studies published at the end of the 20th
century, the annual mortality rate of HCM patients from the refer-
ral center populations was 4–6% per year30–34; however, more recent
studies of unselected patient populations demonstrated an annual
mortality rate of approximately 1% or less.35–39 The reported annual
morbidity in children with HCM was 2% in the community-based
population and 6% in the tertiary referral cohorts.31,40,41 The 3 most
common causes of death in HCM patients are sudden cardiac death
(SCD) (51%), HF (36%), and stroke (13%).36,42 The annual mortality
rates are, respectively, 0.54–1%, 0.55%, and 0.07%.23,38,42–45 More-
over, 1.8% of HCM patients per year experience life-threatening
events, including appropriate implanted cardioverter defibrillator dis-
charge, resuscitated sudden cardiac arrest, or heart transplant.38 SCD
is most common in the younger patients, whereas the fatal outcome
of HF and stroke concerns mainly the elderly.
SUDDEN CARDIAC DEATH
SCD refers to the unexpected cessation of cardiac activity,
with hemodynamic collapse and death within 1 hour from the onset
of symptoms in a person without any prior condition that would seem
fatal. The direct cause of death is usually ventricular tachyarrhyth-
mia. It is estimated that the incidence of SCD worldwide may be as
high as 1/1000 per year. SCD usually occurs in those with previously
known or latent structural heart disease, and the most common cause
of SCD in the general population is coronary artery disease. Nev-
ertheless, HCM is the most frequent cause of SCD in young people
and competitive athletes.46–48 Therefore, for medical, social, and eco-
nomic reasons, it is important to identify people who are at increased
risk of sudden death.
Risk Stratification
Clinical Evaluation
Risk stratification should be performed in all patients with HCM
regardless of their symptoms, hemodynamic status, or the severity of
the hypertrophy. Because the disease can evolve over time, all patients
with known or suspected HCM should undergo serial assessments
every 12–24 months, especially those potentially eligible for implanted
cardioverter defibrillator (ICD) use. The evaluation should include his-
tory and physical examination, family history (with emphasis on cases
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of HCM, SCD, ICD, or syncope among relatives), 2D echocardiog-
raphy (to determine the maximal wall thickness of the LV, possible
LVOT obstruction, and systolic anterior motion), 24–48–hour ambu-
latory electrocardiographic monitoring (to identify arrhythmias), and
cardiac stress test (to assess blood pressure response). CMR may be
considered to precisely determine the maximal wall thickness of the
LV and to assess for the presence and extent of myocardial fibrosis (ie,
late gadolinium enhancement). The evaluation of SCD risk is not usu-
ally repeated in patients who already have an ICD.20
Major (Conventional) Risk Factors
The joint American College of Cardiology/European Society
of Cardiology consensus document from 2003 consolidated a num-
ber of outcome studies that identified prognostic indicators with-
out regard for different effect sizes. The 2011 American College
of Cardiology Foundation/American Heart Association guidelines
upgraded family history of SCD, maximal LV wall thickness, and
unexplained syncope to reasonable (class IIa) indications for ICD
therapy, still recognizing the others as risk-modifying. Although
the major risk factors have limited sensitivity and moderate-to-high
specificity for predicting SCD, they help identify patients who should
receive an ICD for primary prevention. Despite their relatively low
positive predictive value, their negative predicative value is high. The
most important information regarding those factors is presented in
Table 1.15,20,23,24,26,27,43,49–58
Impact of Multiple Major Risk Factors
Identification of ICD-eligible patients may be improved
by summation of conventional risk factors.25,59,60 A cohort of 368
HCM patients demonstrated that multiple risk factors significantly
increase the probability of SCD. Patients without any of the known
risk factors had an estimated 6-year survival rate of 95%. For 1, 2,
and 3 factors, the survival rates were 93%, 82%, and 36%, respec-
tively.25 On the other hand, data from a multicenter registry of ICDs
in HCM patients in primary prevention revealed that 35% of appro-
priate discharges occurred in those who had only a single risk factor
of SCD.61
HCM Risk-SCD Calculator
In 2013, O’Mahony et al62 proposed the HCM Risk-SCD
Calculator, a novel clinical risk prediction model of SCD in HCM
derived from a retrospective, multicenter longitudinal cohort study.
The cohort consisted of 3675 patients from 6 centers in the European
Union. The aim of the study was to derive and validate a new SCD
model assessing an individualized 5-year risk of SCD and to improve
ICD therapy in patients with HCM. The abnormal blood pressure
response to exercise was excluded from the model, whereas maximal
LVOT gradient at rest or during the Valsalva maneuver (formerly an
ancillary risk factor) was upgraded. There were also 2 additions: age
and left atrial diameter. The 5-year risk of SCD < 4% was classified
as low risk without indication for an ICD. An ICD may be considered
if the risk is intermediate, namely 4 to < 6%. Finally, a risk ≥ 6% was
considered high and a clear indication for ICD. Patients who did not
reach the SCD endpoint had a mean calculated 5-year risk of 3.7%.
The model predicts that for every 16 ICDs, 1 patient will be saved
from SCD at 5 years (number needed to treat = 16 over 5 years).62
The calculator was implemented into the 2014 ESC Guidelines on the
diagnosis and management of HCM. Independent validation studies
revealed mixed results, but most of them suggest that the HCM SCD-
Risk Calculator outperforms algorithms from 2003 and 2011. In a ter-
tiary center cohort study of 706 HCM patients, the C-statistics for the
2003, 2011, and 2014 algorithms were 0.55, 0.60, and 0.69, respec-
tively.63–65 The achievement of the new model lies in the shift from
relative to absolute risk estimation.66 Nevertheless, it also has some
Cardiology in Review  •  Volume 26, Number 3, May/June 2018 Sudden Cardiac Death in HCM

TABLE 1.  Major Risk Factors of Sudden Cardiac Death in Hypertrophic Cardiomyopathy
Risk Factor
Family history of SCD
Unexplained syncope
Maximal LV wall thickness > 30 mm
Abnormal BP response to exercise (failure to increase systolic
BP by at least 20 mm Hg from rest to peak exercise or a fall of
>20 mm Hg from the peak exercise BP during ongoing exercise)
Nonsustained ventricular tachycardia (VT; ≥ 3 consecutive
ventricular beats at 120 beats per minute lasting < 30 sec)
SCD indicates sudden cardiac death; LV, left ventricular; BP, blood pressure.
Comment
An increased risk if the deceased close relative was < 40 years old or had confirmed
hypertrophic cardiomyopathy.
An increased risk of SCD if the syncope appears to be due to arrhythmia, particularly
in younger patients, and if it is relatively close (< 6 mo) to evaluation.
An inverted U-shaped relation between maximal LV wall thickness and the estimated
5-year risk of SCD. The incidence of SCD almost doubles for each 5-mm increase
in wall thickness. Patients with wall thickness 30–34 mm have the greatest risk of
SCD. The extreme hypertrophy ≥ 35 mm is very rare, and there is a lacking body of
evidence regarding the risk of SCD in such patients.
An increased risk of SCD in patients < 40 years old or with family history of SCD.
Conflicting data. It is reasonable to assume an increased risk if nonsustained VT occurs
in the young and if the episodes are prolonged, fast, repetitive, or associated with
impaired consciousness.

limitations. It should not be used in pediatric patients < 16 years old,
elite or competitive athletes, HCM associated with metabolic diseases
and genetic syndromes, and in patients for whom ICD is an option for
secondary prevention. The most important information regarding the
risk factors used in the HCM SCD-Risk Calculator are presented in
Table 2.44,62,65,67–72
Low-Risk Patient Profile
Limited data suggest that a patient with the features listed in
Table 3 may have an incidence of SCD 0.2–0.4% per year.15,20,73
Other Potential Prognostic Factors
Although the SCD risk stratification algorithms are getting
more accurate, there are still a number of factors worth considering.
The potentially prognostic features collected in Table 4 may be use-
ful in resolving often difficult and uncertain decisions concerning
implementation of an ICD.2,3,22,25,36,74–95
THE PREVENTION OF SUDDEN CARDIAC DEATH
Cardioverter Defibrillator Implantation
The ICD is the best available therapy for HCM patients who
have survived sudden cardiac arrest (secondary prevention) or who
are at high risk of potentially lethal ventricular arrhythmias (primary
prevention). However, there are many complications after device
therapy, for example, inappropriate ICD discharges (25%), lead
dysfunction (6–13%), infection (4–5%), bleeding or thrombosis
(2–3%).96–98 Therefore, it is of utmost importance to carefully choose
those patients in whom the benefits of ICD therapy outweigh the
risks. It is recommended that patients who have ≥ 4% risk in the
HCM Risk-SCD Calculator, at least 2 major risk factors, LV api-
cal aneurysm, or end-stage HCM undergo ICD. Additionally, pri-
mary prevention may be reasonable in patients with moderate risk
and massive LVH ≥ 30 mm, family history of SCD due to HCM,
or recent unexplained syncope. The presence of extensive late gad-
olinium enhancement on CMR may be useful to make a decision
with patients having 1 major factor and otherwise uncertain risk
stratification.25,49,50,61,62,76
Pharmacologic Treatment
There is no evidence that pharmacologic therapy provides
any protection against SCD due to malignant ventricular arrhyth-
mias in patients with HCM.99 Therefore, there are no recommen-
dations to suppress arrhythmia in the asymptomatic patient with
premature ventricular beats or nonsustained VT.50,100 Neverthe-
less, symptomatic patients are usually treated with amiodarone
and a beta blocker (eg, a nonvasodilating one, or if a patient has
an ICD, use sotalol).

TABLE 2.  Prognostic Factors in HCM SCD-Risk Calculator

Risk Factor
Age at evaluation
Left atrial diameter
Maximal LVOT gradients
Maximal left ventricular
wall thickness
Family history of SCD
Unexplained syncope
Nonsustained VT
LVOT indicates left ventricular outflow track; LV, ventricular tachycardia.
Coding Comment
Continuous An increase in SCD is greatest in young patients < 30 years old, and this decreases through mid-life. Patients >
60 years old have a very low risk of SCD (< 1%), even if they have one or more conventional risk factors.
Continuous Determined by M-Mode or 2D echocardiography at the time of evaluation. Among patients without atrial
fibrillation, an enlarged left atrium was an independent risk factor of SCD.
Continuous Determined at rest and with Valsalva maneuver using pulsed and continuous wave Doppler from the apical 3-
and 5-chamber views. Most of the studies failed to show a correlation between LVOT gradient and adverse
prognosis. Two large studies revealed a slightly increased risk of SCD in patients with resting gradient ≥
30 mm Hg with a significant trend toward lower sudden cardiac arrest survival in patients with increasing
LVOT obstruction.
Continuous The greatest thickness in the anterior and posterior septum, lateral and posterior wall, measured at the level of
mitral valve, papillary muscles, and apex using parasternal short-axis plane in 2D echocardiography at the
time of evaluation; see Table 1
Binary (yes/no) See Table 1
Binary (yes/no) See Table 1
Binary (yes/no) See Table 1

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Adamczak and Oko-Sarnowska Cardiology in Review  •  Volume 26, Number 3, May/June 2018

TABLE 3.  Profile for Low Risk of Sudden Cardiac Death in

the Hypertrophic Cardiomyopathic Patient
Low-risk Features
None of the 5 conventional risk factors
At most, mild symptoms of heart failure
Left atrial diameter < 45 mm
Maximal left ventricular wall thickness < 20 mm
Left ventricular outflow gradient < 50 mm Hg
Septal Reduction Therapies
According to the 2011 American College of Cardiology
Foundation/American Heart Association guidelines, surgical myec-
tomy (SM) remains the treatment of choice for patients with LVOT
obstruction and symptoms of HF. Surgical myectomy has a class IIa
recommendation; alcohol septal ablation (ASA) is class IIb.20 How-
ever, a large meta-analysis of 1019 patients who underwent SM and
805 patients who underwent ASA revealed no difference in symptom
relief, and with regard to SCD, no difference in short-term and long-
term mortality.101 Moreover, the mortality rate of both procedures
mirrors the age- and gender-matched non-HCM survival rates.102–105
Although some older papers claim an increased risk of ventricular
arrhythmia after ASA,106,107 most case series contradict that state-
ment, including no increased risk of SCD and appropriate ICD ther-
apy.105,108–111 Some alternative methods of nonsurgical septal reduction
have been explored, such as injection of glue, microspheres, and coil
embolization, but they have not been adopted because of limited
long-term impact on gradients in LVOT. However, endocardial radio-
frequency ablation of septal hypertrophy seems to be an interesting
option for patients with inappropriate coronary anatomy, although its
impact on the risk of SCD requires further research.112–114
Catheter Ablation
In case reports of HCM patients with persistent VT, radiofre-
quency ablation has been successful in terminating the arrhythmia,
especially in those with apical aneurysm, but further studies on large
cohorts would be beneficial.
Restriction of Physical Activity
During the 36th Bethesda Conference on Eligibility Recom-
mendations for Competitive Athletes with Cardiovascular Abnor-
malities in 2005, experts came to a consensus that athletes with a
probable or diagnosed HCM should not participate in most competi-
tive sports unless they are low intensity.115 The same recommenda-
tions concern the physical activity of other HCM patients.
SUMMARY
Although the SCD risk stratification models have become
more accurate in recent years, even the newest one requires improve-
ment. The HCM Risk-SCD Calculator from 2014 predicts that for
every 16 ICDs in HCM patients, one will be saved from SCD at 5
years. The number needed to treat is very favorable, but we have to
keep in mind that 15 patients may experience serious and unneces-
sary device therapy complications such as inappropriate discharges or
lead malfunction. Therefore, reliable identification of those patients
who would most benefit from ICD therapy is an important medical,
social, and economic issue. The achievement of the newest model is
the absolute, rather than relative, risk estimation; however, it cannot
be used in children and phenotypic mimics of genetic HCM forms.
The role of therapeutic methods, such as invasive septal reduction or
pharmacotherapy, in diminishing the SCD risk was also not consid-
ered. Therefore, the development of and refinement of existing risk
prediction models are crucial for the HCM population and require
further research.

TABLE 4.  Potential Prognostic Factors of SCD in HCM

Risk Factor
The presence of LGE on CMR
Genotype
Left ventricle apical aneurysm
Myocardial ischemia
Intensive physical activity
Extreme RVH
“Burned-out” phase of
HCM/end-stage HCM
SCD indicates sudden cardiac death; HCM, hypertrophic cardiomyopathy; LGE, late gadolinium enhancement; CMR, cardiac magnetic resonance imaging; RVH, right ventricle
hypertrophy.
Comment
The presence of LGE on CMR seems to reflect fibrosis—a potential pathogenic factor for arrhythmia that is associated with
a significantly greater risk of total mortality, cardiovascular mortality, and SCD (OR: 3.4; 95% CI: 2.0–5.9). Each 10%
increase in LGE correlates with a 40% increase in relative SCD risk. Patients with LGE confined to the right ventricle
insertion sites (anterior and inferior septa) do not have an increased risk of mortality or SCD. The extent of LGE also
predicts the development of the “burned-out” phase of HCM with systolic dysfunction.
Hypertrophy in patients with a sarcomere protein mutation develops earlier. They also have a higher prevalence of family
history of HCM and SCD than those without mutation. There seem to be high-risk mutations for SCD—related to the
troponin T gene and several of the beta-myosin heavy chain. However, available data come from rather small studies and
most of the mutations are novel—a certain genotype may be associated with a higher risk in a specific family. Therefore,
the clinical decisions about ICD implementation in primary prevention should not be made on the basis of genetic testing.
On the other hand, multiple gene mutations (double or compound) increase the risk of SCD.
HCM patient with left ventricle apical aneurysm have an increased risk of ventricular arrhythmias, especially VT. The
arrhythmic event rate is 4.7% per year compared with 0.9% per year in patients without this feature. The “pure” apical
HCM is more benign, whereas the “mixed” morphologic variant of midventricular obstruction with cavity obliteration
is associated with an unfavorable prognosis. The size of the aneurysm does not affect the risk of SCD. This is the only
subgroup of patients in whom radiofrequency ablation of recurrent VT seems to be successful.
There are conflicting data if myocardial ischemia due to coronary stenosis or myocardial bridging is a risk factor of SCD.
The relationship between SCD in HCM and ischemia is probably dependent on its etiology and a patient’s age. Typical
exertional chest pain occurs in 25–30% of patients with HCM—usually the coronary arteriogram is normal.
HCM was reported in 36% cases of SCD in American competitive athletes, especially African Americans. Physical exertion
may induce potentially lethal arrhythmias. However, among HCM patients who were not athletes, SCD occurred most
frequently during sedentary or mild physical activities.
Although extreme RVH is uncommon in HCM, it is associated with a worse prognosis and higher cardiovascular mortality.
Further investigations are needed to conclude if there is also a correlation with SCD.
Patients with end-stage HCM (approximately 5%) characterized by systolic dysfunction with a left ventricular ejection
fraction ≤ 50% and often associated with wall thinning and chamber dilation are at high risk of SCD.

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Cardiology in Review  •  Volume 26, Number 3, May/June 2018 Sudden Cardiac Death in HCM
ACKNOWLEDGMENTS
The authors thank Dr. Margarita Lianeri for her editorial assistance.
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