Download Hypertrophic Cardiomyopathy Distance Learning Module

Cardiovascular Screening for Sports Participants
Distance Learning Module
Authors : Dr David Oxborough
Information
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Credits:
Disclosures for Authors: None
Disclosures for Editors: None
Target Audience: Physiologists and medical staff performing
echocardiography wishing to develop or maintain competence in this field.
Learning Objectives: To understand the role echocardiography plays in
screening young athletes (age 14 to 35 years) for inherited cardiovascular
disease.
Term: Launch date March 2015, expiry date 31st March 2017.
Contact: If you have any queries or comments about this module or the
DLM programme, please contact Dawn Appleby
Tel: 020 7345 5185
Fax: 020 7345 5186
Email: [email protected]
Instructions
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This distance learning module should be read in conjunction with the
British Society of Echocardiography / Cardiac Risk in the Young Guideline
for the Practice of Echocardiography in Cardiovascular Screening of Sports
Participants.
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The module features interactive elements intended to highlight important
concepts. Clarification of terms which may be unfamiliar is provided.
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The module concludes with an MCQ designed to check understanding.
Feedback and a score are provided at the end of the test.
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A suggested reading list is also provided.
Introduction
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Cardiac sudden death in a young person, especially those who are seemingly healthy with no previous
symptoms, is a tragic event that generates significant levels of concern from both the medical and wider
communities. Currently in the UK, anecdotal evidence suggests that twelve young people die per week as a
cause of previously undiagnosed inherited cardiac disease. It has been suggested that 81 % of these deaths
occur during or immediately following exercise with 6 % occurring in competitive athletes. This data is
supported by evidence from the US where two large population based studies demonstrate an average of
66 and 55 athletes respectively, dying from cardiac sudden death per year. Furthermore 96 % of all sudden
cardiac deaths appear to be associated with male gender and 75 % between the ages of 18 to 35 years.
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Exercise appears to act as a trigger for individuals who are predisposed to cardiac sudden death and
therefore there is a growing awareness for the need for pre-participation screening. The European Society
of Cardiology advocate the use of a screening health questionnaire and a 12-lead ECG for all competitive
athletes and if any individual presents with potential abnormalities (including T-wave inversion, symptoms,
family history) they are subsequently referred for an echocardiogram. In view of this, echocardiography
plays a pivotal role in the diagnosis or exclusion of inherited cardiac disease with any missed diagnosis
having serious implications for the athlete.
Epidemiology and the Conditions
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There are a number of detectable conditions that cause cardiac sudden death in an athlete. The absolute
figures are debateable with often variable data from different geographical regions. That aside data from the
US provides us with a general understanding of specific epidemiology of cardiac sudden death syndromes
(see figure below).
The ECG should be the first
investigation
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Current European recommendations for cardiac screening of the athlete state that the ECG should be the
primary investigation. The ECG should be interpreted in accordance with specific European Society of
Cardiology (ESC) guidelines and in the context of the individuals’ symptoms, family history and clinical
examination. Transthoracic echocardiography (TTE) is recommended if an athlete presents with Group 2
ECG changes (see below), cardiovascular symptoms, abnormal physical examination findings or a family
history of sudden death under the age of 40.
ESC Classification of ECG Abnormalities in athletes
Group 1 (training-related)
Group 2 (training-unrelated)
Sinus Bradycardia
First degree AV Block
Incomplete RBBB
Early Repolarisation
Isolated QRS voltage criteria for LVH
T-wave inversions
ST-segment depression
Pathological Q-waves
Left Atrial Enlargement
Left axis deviation / left anterior hemiblock
Right axis deviation / left posterior hemiblock
Right Ventricular Hypertrophy
Ventricular pre-excitation
Complete LBBB or RBBB
Long QT or short QT interval
Brugada-like early repolarisation
A full standard Echocardiogram should
be performed
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The athlete’s TTE should be performed according to the BSE Minimum Dataset for a Standard Transthoracic
Echocardiogram in an Adult, and should also take into account recommendations made in the
Supplementary Protocols for Comprehensive Assessment of the Right Heart and the Assessment of
Diastolic Function. Details of where and how to measure these echo parameters are given in these three
protocols. There are however, additional elements that may be considered optional in non-athletes that
become mandatory for accurate interpretation of the athlete’s echocardiogram (see next slide).
A full standard Echocardiogram should
be performed with additional images
VIEW AND MODALITY
PSAX AV LEVEL (2D)
EXPLANATORY NOTE
Identify Coronary Ostia
PSAX MID TO APICAL LEVEL
(2D)
Excess trabeculation is a
common finding in elite athletes
particularly of African / AfroCaribbean ethnicity20. Left
Ventricular Non-Compaction
(LVNC) Cardiomyopathy needs to
be excluded but making this
diagnosis in an athlete may be
challenging.
IMAGE
A full standard Echocardiogram should
be performed with additional images
PSAX BASAL LV LEVEL (2D)
LV wall thicknesses should be
measured at end diastole from
the basal anterior septum,
inferior septum, posterior wall
and lateral wall.
PSAX MID LV LEVEL (2D)
LV wall thicknesses should be
measured at end diastole from
the mid anterior septum, inferior
septum, posterior wall and
lateral wall.
A full standard Echocardiogram should
be performed
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Prior to the echocardiographic examination, the operator needs to be aware of a number of fundamental
factors that may influence interpretation.These include:
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Age
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Gender
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Ethnicity
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Training type and volume
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Body size
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Symptoms
Sex
Family history
of unexplained
cardiac death
under the age
of 40 years
Type of Sport
and Level
(elite /
recreational)
Age
Ethnicity
Echocardiographic
Focus and
Interpretation
Training
Volume
BSA
Symptoms
Group 2 ECG
changes
Additional Considerations for
Echocardiographic Interpretation
Sport
Parameter
All echocardiographers involved in cardiac screening should have
an understanding of the physiological adaptation in cardiac
structure and function to regular exercise. The main adaptation is
eccentric hypertrophy of all cardiac chambers but this can be
variable depending on the type and volume of exercise training.
The table is adapted from a recent systematic review and metaanalysis and highlights normal ranges for trained athletes. This
includes those that engage in resistance activity and endurance
activity. Resistance activity is defined as anaerobic isometric
exercise at incremental workloads of 40-60% of maximum heart
rate and includes sporting disciplines such as martial arts, windsurfing, weight-lifting. Endurance activity is defined as aerobic
isotonic dynamic exercise at incremental workloads of 70-90% of
maximum heart rate and includes sporting disciplines such as long
and middle distance running, swimming or cycling, soccer and
basketball. It is important to note that many sporting disciplines
involve a combination of resistance and endurance exercise such
as boxing, rugby, rowing and American football and therefore there
is likely to be an overlap in normal ranges.
Endurance-Trained
(ET)
ResistanceTrained (RT)
Sedentary
Controls (CT)
232 (200-260)
[n=64; 1099]
220 (205-234)
[n=25; 510]
166 (145-186)
[n=59; 1239]
11.0 (10.3-11.8)
[n=19; 408]
10.4 (9.8-10.9)
[n=14; 370]
9.2 (8.9-9.5)
[n=63; 1352]
8.8 (8.6-9.1)
[n=53; 1433]
P<0.001
LVPWd
(mm)
11.0 (10.8-11.3)
[n=68; 1802]
10.6 (10.3-10.9)
[n=57; 1928]
LVDd
(mm)
54.8 (54.1-55.6)
[n=61; 1548]
52.4 (51.2-53.6)
[n=17; 384]
50.1 (49.5-50.7)
[n=56; 1174]
P<0.001
LVEDV
(ml)
171 (157-185)
[n=34; 493]
131 (120-142)
[n=14; 189]
135 (125-145)
[n=34; 539]
P<0.001
106 (97-116)
[n=28; 479]
63 (61-64)
[n=42; 1330]
2.0 (1.9-2.1)
[n=34; 844]
13.6 (12.3-14.9)
[n=7; 204]
91 (63-119)
[n=5; 116]
86 (77-95)
[n=9; 125]
66 (62-70)
[n=7; 85]
1.9 (1.7-2.0)
[n=8; 214]
*
[n=1; 16]
*
83 (77-90)
[n=27; 590]
64 (62-65)
[n=37; 878]
1.8 (1.7-1.9)
[n=34; 868]
11.0 (9.4-12.6)
[n=4; 183]
37 (24-50)
[n=4; 102]
P<0.001
33.5 (21.0-46.0)
[n=4; 140]
222 (216-227)
[n=6; 136]
*
26.1 (16.1-36.1)
[n=4; 95]
156 (153-159)
[n=6; 150]
P=0.347
114 (115-122)
[n=5; 66]
39.2 (35.9-42.5)
[n=10; 206]
*
94 (92-98)
[n=4; 66]
34.9 (31.9-37.9)
[n=11; 243]
P=0.415
LV mass
(g)
IVSd(mm)
LV SV (ml)
LV EF (%)
LV E/A
LV e’
RV mass
(g)
RVD1(mm)
RVEDV
(ml)
RV SV (ml)
LA Size
(mm)
*
31.9 (29.7-34.1)
[n=2; 58]
P-value
(All
groups)
P<0.001
P<0.001
P=0.365
P=0.014
P=0.014
P<0.01
P=0.627
P<0.001
Additional Considerations for
Echocardiographic Interpretation
Sport
The greatest
degree of left
ventricular, right
ventricular and
atrial remodelling
occurs in those
athletes that
engage in sporting
activities that
includes both high
dynamic and high
static
components.
Additional Considerations for
Echocardiographic Interpretation
Sport.
Training Volume
The magnitude of cardiac adaptation is
dependent on the patient’s level of physical
activity, as defined by the training volume. The
total volume of training can be defined as
(volume = intensity x duration) or Metabolic
Equivalent (MET-h/week = METS x duration).
An example of a select range of sporting
disciplines and their specific METS is
highlighted in table 36. In summary, lowintensity exercise is defined as corresponding
to 1.8 to 2.9 METS, moderate-intensity is
defined as corresponding to 3-6 METS and
high-intensity exercise is defined as > 6 METS.
Sporting Discipline
Soccer
Running (6 mph)
Running (7.5 mph)
Running (10.9 mph)
Cycling (>20 mph) racing
Cycling (<10 mph) leisure
Cricket
Rugby
Tennis (singles)
Hockey
Boxing
Golf
Rowing (competitive)
Swimming (leisure)
Swimming (competitive)
Metabolic Equivalent (MET)
10.0
10.0
12.5
18.0
16.0
4.0
5.0
10.0
8.0
8.0
12.0
4.5
12.0
6.0
10.0
Additional Considerations for
Echocardiographic Interpretation
Sport.
Training Volume
Age and Sex
• The athletes age may influence the magnitude of cardiac adaptation. Postpubescent junior athletes between the ages of 14 and 18 demonstrate eccentric
remodelling of the left ventricle compared to non-athletic controls. There are no
studies looking at the right ventricle or atria in this population, however it
would be sensible to expect a similar magnitude of adaptation. It is also apparent
that although junior athletes demonstrate adaptation it appears to be at a lower
level then their elite, senior counterparts.
• Cardiac chamber dimensions in female athletes very rarely fall outside the
normative range.
• Irrespective of body size, the LV cavity dimension rarely exceeds 65mm.
Additional Considerations for
Echocardiographic Interpretation
Training Volume
Sport
Age and Sex
Ethnicity
• Black athletes are likely to present with a higher incidence of abnormal ECG
findings (T-wave inversions) compared to white athletes and therefore they are
more likely to be referred for echocardiography.
• Black athletes demonstrate a significantly larger left ventricular wall thickness
when compared to white athletes and normative cut-offs should reflect this (see
algorithms). The right ventricle does not appear to adapt differently in different
ethnic groups.
• Athletes of Middle Eastern and South and East Asian ethnicity do not have different
adaptation compared to white athletes
Additional Considerations for
Echocardiographic Interpretation
Sport
Training Volume
There is a higher
incidence of excess
trabeculations in
athletes and these
appear to be more
associated with
T-wave inversion
and black ethnicity
Age and Sex
Ethnicity
The Algorithms
Sport
Training Volume
Age and Sex
Ethnicity
Algorithms
The Algorithms
Sport
Training Volume
Age and Sex
Ethnicity
Algorithms
CASE 1
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A 25 year old male elite footballer of Afro-Caribbean
ethnicity is referred for pre-participation screening. The
athlete engages in predominantly dynamic (endurance)
exercise training for approximately 30 hours per week
and has no symptoms or family history of explained
sudden death.
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On examination his blood pressure is 120/70 mmHg. His
height is 184 cm with a body mass of 75 kg.
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HYPERTRABECULATION
The ECG
Prior to Echocardiography
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Ethnicity = Black (ethnicity and gender suggests wall thicknesses
up to 14mm, high prevalence of hypertrabeculation in black
athletes)
Training Type = Football / High Dynamic (eccentric hypertrophy
more likely)
Training Volume = High (training volume suggests increased cavity
size of both LV, Atria and RV).
BSA = 1.94cm2 (normal anthropometry)
ECG = Early repolarisation and T wave inversion (not lateral)
(this is a typical ECG from an elite black athlete, expect
hypertrabeculation and/or increased eccentric / concentric
hypertrophy)
The Echocardiogram
The Echocardiogram
The Echocardiogram
Echocardiography Key Findings
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Normal LV size when indexed for BSA with normal
geometry.
Maximum LV wall thickness = 12mm
Good systolic and diastolic function evidenced with an EF
of 60%, normal transmitral E/A and Septal E’ = 12cm/s,
Lateral E’ = 17cm/s
Marked mid to apical hyper-trabeculation
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To Exclude Isolated Left Ventricular Non-Compaction
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These findings are consistent with
athletic adaptation
Hypertrabeculation Vs. ILVNC
ATHLETE
ILVNC
• Hypertrabeculation is common in elite athletes with a higher prevalence in those
of black ethnicity and may be associated with T-wave inversion on ECG
• ILVNC patients usually have LV systolic or diastolic dysfunction
• Additional imaging may be beneficial in cases with marked hypetrabeculation
CASE 2
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A 22 year old male elite footballer of Caucasian ethnicity
is referred for pre-participation screening. The athlete
engages in predominantly dynamic (endurance) exercise
training for approximately 25 hours per week. He has no
symptoms but a family history of an explained death in a
sibling at the age of 22 years old.
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On examination his blood pressure is 120/60 mmHg. His
height is 170 cm with a body mass of 70 kg.
The ECG
Prior to Echocardiography
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Ethnicity = Caucasian (ethnicity and gender suggests wall
thicknesses up to 12mm)
Training Type = Football / High Dynamic (eccentric hypertrophy
more likely)
Training Volume = High (training volume suggests increased cavity
size of both LV, Atria and RV).
BSA = 1.82cm2 (normal anthropometry)
ECG = Lateral T wave inversion (is not consistent with athletic
adaptation and should consider pathology)
The Echocardiogram
The Echocardiogram
Echocardiography Key Findings
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Normal LV size (45mm) when indexed for BSA with
normal geometry – would be considered small for
training volume
Maximum LV wall thickness = 13mm but no evidence of
asymmetric hypertrophy or outflow obstruction
Good systolic and global diastolic function however
myocardial diastolic velocities are low (Septal E’ = 7cm/s)
Normal RV structure and function
This athlete was referred for further investigations
and diagnosed with a mild phenotype HCM
CASE 3
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A 27 year old male, caucasian elite cyclist is referred for
pre-participation screening. The athlete engages in a
mixed type training involving both high static (resistance)
and high dynamic (endurance) activity for 45 hours per
week. He has no symptoms or family history of
unexplained sudden death.
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On examination his blood pressure is 110/65 mmHg. His
height is 190 cm with a body mass of 83 kg.
The ECG
Prior to Echocardiography
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Ethnicity = Caucasian (ethnicity and gender suggests wall
thicknesses up to 12mm)
Training Type = Cycling / High Dynamic and High Static
(eccentric hypertrophy very likely)
Training Volume = High (training volume suggests increased cavity
size of both LV, Atria and RV).
BSA = 2.09cm2 (increased BSA)
ECG = Partial RBBB, Inferior T-wave inversion, early
repolarisation, isolated voltage criteria for LVH (with exception
of theT wave inversion the rest of the ECG demonstrates training
related adaptation)
The Echocardiogram
The Echocardiogram
The Echocardiogram
Echocardiography Key Findings
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LV size is enlarged (72mm) with increased indexed LV mass and a RWT of
0.31. This is eccentric hypertrophy.
LV systolic function is at the lower end of normal (EF of 52% and septal S’
= 7cm/s, however lateral wall is normal 12cm/s) Transmitral E/A is normal
but borderline reduced septal E’ = 9cm/s and normal lateral E’ = 15cm/s.
Enlarged RV inflow and outflow but functions well and both atria are
enlarged.
Need to exclude dilated cardiomyopathy.
The athlete underwent a short
exercise stimulus and all myocardial
velocities and EF improved
demonstrating RESERVE
cMRI demonstrated
athletic adaptation of all
cardiac chambers
CASE 4
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A 27 year old male, caucasian recreational long-distance
runner is referred for echocardiography following
syncope during a marathon race. His training volume
involves 6 hours per week of dynamic (endurance)
training only. He has no family history of sudden cardiac
death.
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On examination is blood pressure is 115/70 mmHg. His
height is 177 cm with a body mass of 68 kg.
Additional finding of a Pectus Excavatum.
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The ECG
Prior to Echocardiography
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Ethnicity = Caucasian (ethnicity and gender suggests wall
thicknesses up to 12mm)
Training Type = Long Distance Running / High Dynamic and
Moderate Static (eccentric hypertrophy likely)
Training Volume = Low (training volume suggests adaptation will
be at a lower degree).
BSA = 1.83cm2 (normal anthropometry)
ECG = T-wave inversion V1-V3 (Need to exclude ARVC – this is
unlikely to be training related without early repolarisation)
Pectus Excavatum (May impact on the anterior/lateral RV free
wall)
The Echocardiogram
The Echocardiogram
Echocardiography Key Findings
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LV size and wall thicknesses are within normal limits for body size.
The RV is not dilated but appears compressed at the base giving an
abnormal geometry. RV function is normal (RVFAC= 40% and normal
annular velocities (S’ = 16m/s). The RV outflow was normal size.
Although there is an abnormal RV geometry, function is normal and no
criteria for ARVC is met.
The recreational athlete underwent
cardiac MRI and was diagnosed with
some compression secondary to
pectus excavatum
Key Learning
POINT:
Look for pectus
excavatum during
pre-participation
screening
Key References

Maron B, Thompson P, Ackerman M et al. 2007 Recommendations and Considerations Related to
Preparticipation Screening for Cardiovascular Abnormalities in Competitive Athletes: 2007 Update: A
Scientific Statement From the American Heart Association Council on Nutrition, Physical Activity, and
Metabolism: Endorsed by the American College of Cardiology Foundation. Circulation 115:1643-1655
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Utomi V, Oxborough D, Whyte G, Somauroo J, Sharma S, Shave R et al. 2013 Systematic Review and Meta
Analysis of Training Mode, Imaging Modality and Body Size Influences on the Morphology and Function of
the Male Athlete’s Heart. Heart – in press
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de Noronha SV, Sharma S, Papadakis M, Desai S, Whyte G, Sheppard MN. Aetiology of sudden cardiac death
in athletes in the United Kingdom: a pathological study. Heart. 2009 Sep;95(17):1409-14.

Oxborough D, Sharma S, Shave R, Whyte G, Birch K, Artis N et al. 2011 The right ventricle of the endurance
athlete: the relationship between morphology and deformation. J Am Soc Echocardiogr 25(3):263-271

Riding N, Salah O, Sharma S, Carre F, O’Hanlon R, George K et al. 2012 Do big athletes have big hearts?
Impact of extreme anthropometry upon cardiac hypertrophy in professional male athletes. Br J Sports Med
46: i90-i97
MCQ’s