Hypertrophic Cardiomyopathy Distance Learning Module

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Transcript Hypertrophic Cardiomyopathy Distance Learning Module

Cardiovascular Screening for Sports Participants Distance Learning Module

Authors : Dr David Oxborough

Information

      

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 31 st 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

 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.

 The module features interactive elements intended to highlight important concepts. Clarification of terms which may be unfamiliar is provided.  The module concludes with an MCQ designed to check understanding.

Feedback and a score are provided at the end of the test.

 A suggested reading list is also provided.

Introduction

 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.

 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

 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

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) Sinus Bradycardia First degree AV Block Incomplete RBBB Early Repolarisation Isolated QRS voltage criteria for LVH

Group 2 (training-unrelated)

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

 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

IMAGE PSAX MID TO APICAL LEVEL (2D)

Excess trabeculation is a common finding in elite athletes particularly of African / Afro Caribbean ethnicity 20 . Left Ventricular Non-Compaction (LVNC) Cardiomyopathy needs to be excluded but making this diagnosis in an athlete may be challenging.

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

Prior to the echocardiographic examination, the operator needs to be aware of a number of fundamental factors that may influence interpretation.These include:       Age Gender Ethnicity Training type and volume Body size Symptoms Family history of unexplained cardiac death under the age of 40 years Sex Age Type of Sport and Level (elite / recreational) Echocardiographic Focus and Interpretation Ethnicity Training Volume BSA Group 2 ECG changes Symptoms

Additional Considerations for Echocardiographic Interpretation

Sport 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 meta analysis 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, wind surfing, 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. Parameter

Endurance-Trained

(ET)

LV mass

(g) IVSd(mm)

LVPWd

(mm)

LVDd

(mm)

LVEDV

(ml) LV SV (ml) LV EF (%) LV E/A LV e’

RV mass

(g) RVD1(mm)

RVEDV

(ml) RV SV (ml)

LA Size

(mm) 232 (200-260) [n=64; 1099] 11.0 (10.8-11.3) [n=68; 1802] 10.6 (10.3-10.9) [n=57; 1928] 54.8 (54.1-55.6) [n=61; 1548] 171 (157-185) [n=34; 493] 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] 33.5 (21.0-46.0) [n=4; 140] 222 (216-227) [n=6; 136] 114 (115-122) [n=5; 66] 39.2 (35.9-42.5) [n=10; 206]

Resistance-

Trained (RT) 220 (205-234) [n=25; 510] 11.0 (10.3-11.8) [n=19; 408] 10.4 (9.8-10.9) [n=14; 370]

Sedentary

Controls (CT) 166 (145-186) [n=59; 1239] 9.2 (8.9-9.5) [n=63; 1352] 8.8 (8.6-9.1) [n=53; 1433]

P-value (All

groups) P<0.001 P<0.001 P<0.001 52.4 (51.2-53.6) [n=17; 384] 50.1 (49.5-50.7) [n=56; 1174] P<0.001 131 (120-142) [n=14; 189] 86 (77-95) [n=9; 125] 66 (62-70) [n=7; 85] 1.9 (1.7-2.0) [n=8; 214] * [n=1; 16] * * * * 31.9 (29.7-34.1) [n=2; 58] 135 (125-145) [n=34; 539] 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] 26.1 (16.1-36.1) [n=4; 95] 156 (153-159) [n=6; 150] P<0.001 P<0.001 P=0.365 P=0.014 P=0.014 P<0.01 P=0.347 P=0.627 94 (92-98) [n=4; 66] 34.9 (31.9-37.9) [n=11; 243] P=0.415 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, low intensity 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. Post pubescent 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

• Sport Training Volume 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 Age and Sex Ethnicity There is a higher incidence of excess trabeculations in athletes and these appear to be more associated with T-wave inversion and black ethnicity

The Algorithms

Sport Training Volume Age and Sex Ethnicity Algorithms

The Algorithms

Sport Training Volume Age and Sex Ethnicity Algorithms

CASE 1

 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.  On examination his blood pressure is 120/70 mmHg. His height is 184 cm with a body mass of 75 kg.  HYPERTRABECULATION

The ECG

Prior to Echocardiography

     Ethnicity = Black

athletes) (ethnicity and gender suggests wall thicknesses up to 14mm, high prevalence of hypertrabeculation in black

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 = 1.94cm

2

(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

    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  To Exclude Isolated Left Ventricular Non-Compaction

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

 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.  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

     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 = 1.82cm

2

(normal anthropometry)

ECG = Lateral T wave inversion

(is not consistent with athletic adaptation and should consider pathology)

The Echocardiogram

The Echocardiogram

Echocardiography Key Findings

    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

 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.  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

     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 = 2.09cm

2

(increased BSA)

ECG = Partial RBBB, Inferior T-wave inversion, early repolarisation, isolated voltage criteria for LVH

related adaptation) (with exception of theT wave inversion the rest of the ECG demonstrates training

The Echocardiogram

The Echocardiogram

The Echocardiogram

Echocardiography Key Findings

    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

 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.   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.

The ECG

Prior to Echocardiography

      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 = 1.83cm

2

(normal anthropometry)

ECG = T-wave inversion V1-V3

(Need to exclude ARVC – this is unlikely to be training related without early repolarisation)

Pectus Excavatum

wall) (May impact on the anterior/lateral RV free

The Echocardiogram

The Echocardiogram

Echocardiography Key Findings

   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 UtomiV, 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 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