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HypertrophiccardiomyopathyHighlights
Summary
Overview
Basics
Definition
EpidemiologyAetiology
Pathophysiology
Classification
Prevention
Primary
ScreeningSecondary
Diagnosis
History & examination
Tests
Differential
Step-by-stepCriteria
Guidelines
Case history
Treatment
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Details
Step-by-step
Emerging
GuidelinesEvidence
Follow Up
Recommendations
Complications
Prognosis
ResourcesReferences
Images
Online resources
Patient leaflets
Credits
EmailPrint
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Share
Add to Portfolio
Bookmark
Add notesHistory & exam
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Key factors FHx of HCM Hx of pre-syncope or syncope systolic ejection murmur left ventricular lift double apical impulse or double carotid pulsation FHx of sudden death
Other diagnostic factors younger male (50 years)
collapse fourth heart sound
History & exam details
Diagnostic tests
1st tests to order ECG CXR echocardiography
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Tests to consider exercise ECG Holter monitoring nuclear imaging exercise test CT coronary arteriography cardiac catheterisation cardiac MRI genetic mutation analysis
Diagnostic tests details
Treatment details
Presumptive
asymptomatic
at high risk of sudden deatho implantable cardioverter-defibrillator +
restriction from high-intensity athletics not at high risk of sudden deatho observation + restriction from high-intensity
athletics
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Acute
symptomatic: predominant left ventricular (LV)
outflow tract obstruction with preservedsystolic function
negative inotropic and chronotropic agents +restriction from high-intensity athletics
implantable cardioverter-defibrillator control of atrial arrythmias
anticoagulation surgical coronary artery unroofing (selected
cases) myectomy/alcohol septal ablation/dual-chamber
pacing + restriction from high-intensity athletics implantable cardioverter-defibrillator control of atrial arrythmias anticoagulation surgical coronary artery unroofing (selected
cases)
symptomatic: predominant non-obstructive withpreserved systolic function
negative inotropic and chronotropic agents +restriction from high-intensity athletics
implantable cardioverter-defibrillator control of atrial arrythmias anticoagulation
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surgical coronary artery unroofing (selectedcases)
Ongoing
symptomatic: end-stage heart failure withsystolic dysfunction
beta-blocker + ACE inhibitor/ARB + considerationfor implantable cardioverter-defibrillator + restriction
from high-intensity athletics digoxin non-aldosterone antagonist diuretics aldosterone antagonists control of atrial arrythmias anticoagulation
surgical coronary artery unroofing (selectedcases) referral for heart transplant
Treatment details
Summary Most common cardiomyopathy and the most
frequent cause of sudden cardiac death in youngpeople. Presentation varies from asymptomatic to
symptoms of heart failure. Physical examination may be normal at rest.
Auscultation along the left sternal border when the
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patient is standing after a brief period of exercise mayelicit a murmur.
Family history may be present. Echocardiography
should be used to screen first-degree familymembers. Has a benign prognosis in the majority of
patients. Medical therapy with beta-blockers, calcium-
channel blockers, or disopyramide is used insymptomatic patients.
A subset of patients with increased risk forsudden death should undergo defibrillatorimplantation.
DefinitionHypertrophic cardiomyopathy (HCM) is a genetic disorder characterised byleft ventricular hypertrophy (LVH) without an identifiable cause. It is the mostcommon genetic heart disease as well as the most frequent cause of sudden
cardiac death in young people.[1] Many patients will have no symptoms atthe time of diagnosis and will be diagnosed following routine examination orfamily screening of an affected family member.[2] [3]
EpidemiologyHCM occurs with an incidence of 1 in 500 people in the general populationand is the most common cause of sudden death in children and adults under35 years.[1] Prevalence in the US, Japan, and China has ranged from 0.16%to 0.29%.[7] The mean age of presentation differs among published series
but in a large community sampling it was noted to be 57 (16 to 87years).[8] While the disease is autosomal dominant with no known sexpredilection, women are more likely to evade diagnosis, presenting at anolder age with a greater likelihood of New York Heart Association (NYHA)class III/IV symptomatology.[9] Sudden death is most common in youngpatients, and death from heart failure or stroke occurs more frequently inmiddle age and beyond.[10] While the disease in general affects all ethnic
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groups, apical HCM is seen much more commonly in Asian people. ApicalHCM accounts for
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increased filling pressures and is argued to be the primary source ofsymptoms in many patients, particularly young people.[2] [18]Myocardial ischaemia is common and is likely to be multifactorial in origin. Itmay be due to increased myocardial oxygen demands and reducedmyocardial capillary density relative to the LVH, small vessel disease,
myocardial bridging or increased diastolic wall tension, and coronary vascularresistance due to abnormal LV relaxation and impaired filling.[2] Thepresence of symptoms may be due to the degree of subaortic stenosis at restand with exercise, impaired diastolic relaxation, arrhythmias, impaired systoliccontraction in the absence of obstruction, and ischaemia.[2]
Classification
Obstructive versus non-obstructive
Patients are classified as obstructive or non-obstructive based on thepresence or absence of left ventricular outflow tract obstruction onechocardiography at rest.[4]A resting pressure gradient between the leftventricle and the aorta is present in 37% of patients. An additional 33% willhave provocable obstruction - for example, obstruction with exercise - suchthat the majority of patients have some degree of obstruction.[5] There is nota strong correlation between symptoms and the degree ofobstruction.[2] Patients with severe obstruction may be asymptomatic.
Site of obstruction
Patients may be classified based on the site of maximal left ventricularobstruction:[2]
Subaortic obstruction (classic)
Mid-ventricular obstructive HCM
Apical HCM
Complex obstructive HCM.
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Children with the condition may also have co-existing mid-ventricular rightventricular obstruction.[6]
Primary preventionThere is no primary prevention for the condition, just secondary prevention for
sudden death for those with the condition.
ScreeningScreening should be performed in two distinct populations:
1.Competitive athletes
2.Family members of affected patients.
Screening of competitive athletes for HCM remains a controversialtopic.[46] [47] [48] The argument has been made that routine screeningshould be performed in all competitive athletes, as HCM is the most commoncause of sudden death in this patient population.[46]Pre-participationscreening with ECG has been routinely performed on all competitive athletes
in Italy for the past 25 years, and it has been suggested that the lowincidence of sport-related sudden death in Italy has occurred as a result ofsuch screening.[48] While ECG increases the sensitivity of detectingunderlying heart disease above physical examination alone, a normal ECGmay be present in up to 25% of asymptomatic patients withHCM.[41]Although in Europe the use of ECG screening in young athleteshas been associated with a decline in the rate of sudden death in thesepeople, this approach has not been endorsed in the US.[41]
As HCM is an autosomal dominant disorder, routine screening of all first-degree family members should be performed. Such screening entailsechocardiographical analysis. As the hypertrophy may not develop until alater age, a negative echocardiogram does not rule out thediagnosis.[13] [28] [37] [38] Initial screening should occur by the age of 12years. Screening may be indicated sooner in people with an abnormalexamination, symptoms, or family history of early onset HCM or suddencardiac death. Clinical screening with history, physical examination, ECG,and echocardiogram should be repeated every 12 to 24 months throughout
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adolescence. Due to the possibility of delayed adult-onset LVH, familymembers >18 years should continue to undergo clinical screening every 5years.[49]Genetic analysis is an important screening tool for extended family memberswhen a genetic mutation has been identified within affected family members.
Genetic screening of relatives can then be used to definitively rule out thediagnosis in unaffected individuals, thereby avoiding the need for lifelongserial echocardiographical screening.[13] [36]
Secondary preventionScreening of all first-degree family members with echocardiography, ECG,and clinical follow-up is required. All patients with HCM should:
Refrain from high-intensity athletics
Undergo serial testing with echocardiogram,Holter monitor, exercise testing, and clinicalevaluation to determine their risk level for suddencardiac death (SCD); implantable cardioverter-defibrilators (ICDs) are indicated for prevention ofSCD in selected individuals at higher risk
Be considered for anticoagulation for strokeprevention if they develop atrial fibrillation
Be referred for consideration of surgical coronary
unroofing if they develop ischaemia due tomyocardial bridging.
Monitoring
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HCM patients (particularly those
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are symptomatic or have LVOT obstruction >50 mmdue to changes in contractility, heart rate, and vascucapacitance that occur with pregnancy, labour, and/o
sudden death
see our comprehensive coverage of Cardiac arrestSudden death is the most common mode of death inoccurring with an incidence of 1% per year.[51] Thedeath is ventricular tachycardia secondary to ischaeoccurs in the setting of extreme exertion.Risk factors for sudden death have been identified inrisk factors are: non-sustained ventricular tachycardmonitorings and prior cardiac arrest. Both are indicacardioverter-defibrillator placement.[1] [28] HCM pacardiac arrest and are treated with conventional medyear mortality rate of 33%.[40]
infective endocarditis (IE)
see our comprehensive coverage of Infective endocThe site of the vegetation is usually the thickened an
HCM no longer mandates antibiotic IE prophylaxis uendocarditis or prosthetic valve.
stroke
see our comprehensive coverage of Overview of stroAtrial fibrillation in HCM significantly increases the ri
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Anticoagulation with warfarin is recommended for Hchronic atrial fibrillation.[28][64]
PrognosisIn the majority of cases, HCM carries a benign prognosis. At presentation90% of patients will be asymptomatic, and the majority of those will remainasymptomatic on long-term follow-up. In one prospective study, the onset ofany symptom was delayed until the patient was 70 years or older in 18% ofpatients.[8] Patients presenting with mild to moderate symptoms typicallyexperience slow progression of symptoms with advancing age.
A subgroup of patients (5% of all HCM patients and 30% of tertiary referralpopulations) will develop symptomatic outflow tract obstruction that is
refractory to medical therapy. Patients with resting provocable left ventricular(LV) outflow gradients >50 mmHg and severe limiting symptoms arecandidates for surgical or catheter intervention.[28]In patients who are asymptomatic at presentation, the annual mortality rate islower than in those patients with symptoms (0.9% versus 1.9%). Similarly, theannual rate of sudden death is lower in patients without symptoms atpresentation (0.1% versus 1.4%).[68]Risk factors for sudden death are as follows.[1] [4] [28] [29] [30] [31] [32]
Non-sustained ventricular tachycardia on Holter
monitor.
Abnormal BP response to exercise: defined as arise in systolic BP of 20 mmHg during exercise. Medications mayaffect the BP response and should be considered in
the interpretation of exercise test results.
Massive hypertrophy (left ventricular wallthickness 30 mm).
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Severe outflow obstruction by echocardiogram(left ventricular outflow tract obstruction >30 to 50mmHg). While severe obstruction is considered a
minor risk factor for sudden death, the degree ofoutflow tract obstruction generally does not correlatewith the risk of sudden death. Medical therapy orsurgery to decrease outflow tract obstruction doesnot decrease the risk of sudden death.
Family history of sudden death.
Personal history of unexplained syncope.
Prior cardiac arrest, or sustained ventriculartachycardia.
The identification of risk factors for sudden death continues to be an area ofheightened clinical interest. Cardiac MRI is emerging as a means ofidentifying patients who are at increased risk for arrhythmias. Several studieshave found the presence of myocardial fibrosis by late gadoliniumenhancement to be associated with the occurrence of ventriculararrhythmias.[25][27] [26] [24] [23] The presence of fibrosis has also beenfound to be an independent risk for death.[C Evidence]The annual mortality rate for those patients diagnosed in childhood issubstantially increased over that of the general population (1.3% versus
0.08%). In contrast, annual mortality rate of those diagnosed in adulthood isnot increased above the general population (2.2% versus 1.9%).[69]End-stage heart failure develops in
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history of end-stage disease. Mortality is high once symptomatic heart failureensues, with mean time to death or cardiac transplantation of 2.7 2.1years.[65]
Case history #1
A 21-year-old active college student with no past medical history has suddenloss of consciousness, 1 hour into a game of basketball. CPR is administeredby bystanders. On arrival of emergency medical professionals he hasregained consciousness. The family history is significant for a murmur in hisfather and grandmother only. Physical examination reveals a systolic ejectionmurmur that increases in intensity when going from a supine to standingposition and disappears with squatting.
Case history #2A 60-year-old woman has progressive dyspnoea on exertion over the last 2months. She is otherwise well, with no risk factors for ischaemic heartdisease. Family history is significant for a cousin who died suddenly in hisyouth, and is otherwise unremarkable. Physical examination reveals aprominent jugular a-wave and a double apical impulse. There are nomurmurs audible. An S4 is present. The remainder of the examination isnormal.
Other presentationsOther common symptoms include: chest pain, palpitations, postural light-headedness, resuscitated sudden death, and fatigue.[2] Patients may remainasymptomatic and be diagnosed solely on the basis of family screening.
Differential diagnosis
ConditionDifferentiatingsigns/symptoms Differentiating
Athlete's heart Patient will
typically be a Echocardio
dimension (
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maleenduranceathlete without
cardiacsymptoms.
No familyhistory of HCMor sudden
death.
Hypertrophy willregress withcessation ofexercise.[44]
symmetric L
Wall thickne
mm).[44] LV filling pa The use of
differentiatin
Discretesubaorticstenosis
No familyhistory of HCMor suddendeath.
Not associatedwith suddendeath. Systolicmurmur istypically
Echocardiomyocardiumtissue Dopp
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present in allpositions (i.e.,supine and
squatting).
LVH due tohypertension
Hx of long-standinghypertension.
Echocardiomyocardium
History & examinationKey diagnostic factorshide allFHx of HCM (common)
Autosomal dominant pattern but variable
penetrance.Hx of pre-syncope or syncope (common)
Syncope with exertion or without a prodrome isparticularly concerning and may be due to eitheroutflow tract obstruction or a ventriculararrhythmia.
systolic ejection murmur(common) Audible at the lower left edge, accentuated by
exercise and standing and lessened by lyingsupine or squatting.[2]
left ventricular lift (common)
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Best palpated at the left ventricular apexdouble apical impulse or double carotidpulsation (common)
An initial upstroke of the apical impulse or pulsemay be felt, followed by a brief collapse and asecond impulse. This transient interruption incardiac output occurs when the anterior leaflet ofthe mitral valve is pulled into the left ventricularoutflow tract during systole (systolic anteriormotion of the mitral valve, or SAM).
FHx of sudden death (uncommon) Affected family members may have presented
with sudden death, thereby escaping a definitivediagnosis.[2]
Other diagnostic factorshide allyounger male (
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dysfunction, or end-stage heart failure related toHCM.
angina (common)
Angina is present in 75% of those patients whoare symptomatic.[3] Chest pain with exertion is particularly concerning
and may be due to massive hypertrophy withimpaired coronary perfusion, outflow tractobstruction, or myocardial bridging (tunnelling ofcoronary arteries into heart muscle).
Atherosclerotic coronary artery disease shouldalso be considered in the adult with exertionalchest pain.
palpitations (common) May represent either ventricular arrhythmias or
atrial fibrillation.
irregularly irregular pulse (common) A sign of atrial fibrillation. Atrial fibrillation predisposes to thrombus
formation and warrants anticoagulation as well asanti-arrhythmic therapies.
older female (>50 years) (uncommon)
Females are much more likely to be diagnosed ata later age and be symptomatic at the time ofdiagnosis.[10]
collapse (uncommon)
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Patients may present with resuscitated suddendeath or syncope with extreme exertion. Thismay be the only symptom.[2] [28] [40]
fourth heart sound (uncommon) A fourth heart sound (S4) occurs late in diastole
and suggests a stiff ventricle or impaired diastolicfilling related to hypertrophy.
Risk factorshide all
Strongfamily history of HCM or sudden cardiac death
Affected family members may have presentedwith sudden death, thereby escaping a definitivediagnosis.[2]
Diagnostic tests1st tests to orderhide all
Test
ECG
The most common ECG abnormality is that of abnordepression or giant T wave inversion.[3] View imag
Prominent Q waves are seen in leads II, III, aVF, V5
LVH is present with QRS complexes that are tallest Patients with localised hypertrophy and no symptom
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is present in 15% to 25% of patients.[3][41]An abnohypertrophy on echocardiography.[42]
CXRThis test is not particularly sensitive. Patients may hatrial enlargement, or the CXR may be normal.[2] [3
echocardiography
This test is used for family screening of an affected isudden cardiac death in patients with a known diagn
The cardinal feature is LVH. Considerable variabilityhypertrophy.
The classic pattern of asymmetrical septal hypertrop1.5 times the posterior wall diameter in diastole. Vie
Other echocardiographical features include: a septa(SAM) of the mitral valve, a left ventricular (LV) outflfunction present in 80% of patients independent of thand mitral insufficiency.[2] [3] [20]Diastolic dysfunction should be assessed by Doppleechocardiographical screening test of first-degree re
the onset of overt LVH.[21]
Tests to considerhide all
Test
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exercise ECG
Exercise testing is performed to aid in risk stratificati
Abnormalities associated with an increased risk of ssystolic BP response of
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cardiac catheterisation
Patients with exertional chest pain, ischaemia on nuof CAD based on risk factors should undergo cardiaatherosclerotic coronary disease or myocardial bridg
cardiac MRI
May increase the diagnostic yield in patients with su
visualisation by echocardiogram of the left ventricula
Systolic and diastolic function can also be assessed
The use of late gadolinium enhancement techniquesthat may be a marker for adverse outcomes, or mayathletic heart.[22]
Several studies have found the presence of myocardenhancement to be associated with the occurrence arrhythmias.[23] [24] [25] [26] [27] The presence of independent risk for death.[20][29] [C Evidence]
genetic mutation analysis
Currently identified disease-causing genes are thougdisease, and the sensitivity of commercially availablon the number of genes screened by the particular lasarcomeric mutations are screened, the clinical sensWhen a mutation is identified, genetic testing is usef
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determine requirement for ongoing cardiology follow
Clinical variability exists despite identical gene muta
Genetic counselling should be available for patients results can be reviewed and the clinical significance
Step-by-step diagnostic approachIn suspected cases, an evaluation with history including family history,physical examination, ECG, and echocardiography should be performed. The
latter establishes the diagnosis. Asymptomatic patients are usually diagnosedat the time of routine heart examination or family screening.[2] [3] Patientsare most commonly diagnosed after the onset of clinical manifestations, withonly 32% of patients diagnosed on routine medical evaluation.[7]LaboratoryDNA analysis for mutant genes is the most definitive method for establishingthe diagnosis of HCM, but is usually used only for screening purposes.
History
A family history of syncope, heart failure, or sudden or premature deathshould be taken. Family history may be negative, as the disease hasincomplete penetrance. Patients may be symptom-free. However, it isimportant to note any symptoms of pre-syncope or syncope, particularly whenoccurring with exercise, dyspnoea on exertion, palpitations, or chest pain.Patients over 50 years of age may present with atrial fibrillation or symptomsof a stroke.[19]
Physical examinationExamination may be remarkable for a left ventricular (LV) lift; a double apicalimpulse; a brisk carotid upstroke; a systolic ejection murmur at the lower leftedge that is accentuated by exercise and standing and lessened by lyingsupine or squatting; and a fourth heart sound.[2]
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Diagnostic testingECG may show prominent Q waves in leads II, III, avF, V5, and V6. LVH, STdepression, and pre-excitation may be present.View image CXR may showcardiomegaly.View image Echocardiography must be performed to establishthe diagnosis, and will typically show asymmetrical septalhypertrophy.[2] View imageView imageOther echocardiographical features include:[2] [3] [20]
Septal diameter >12 mm
Systolic anterior motion (SAM) of the mitral valve
A LV outflow tract gradient
Abnormalities of diastolic function (present in80% of patients independent of the presence ofoutflow tract obstruction)
Mitral insufficiency.
Diastolic dysfunction should be assessed by Doppler tissue imaging as partof the echocardiographical screening test of first-degree relatives, as thisabnormality may precede the onset of overt LVH.[20] [21]The use of cardiac MRI may increase the diagnostic yield in patients withsuspected HCM who have poor visualisation by echocardiogram of the leftventricular walls or left ventricular apex. Systolic and diastolic function canalso be assessed by MRI.The use of late gadolinium enhancement
techniques can identify areas of myocardial fibrosis that may be a marker foradverse outcomes, or may aid in differentiating HCM from an athleticheart.[22] Cardiac MRI is emerging as a means of identifying patients whoare at increased risk for arrhythmias. Several studies have found thepresence of myocardial fibrosis by late gadolinium enhancement to beassociated with the occurrence of ventricular
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arrhythmias.[23] [24] [25] [26] [27] The presence of fibrosis has also beenfound to be an independent risk for death.[20] [C Evidence]
Risk stratification
After diagnosis, patients should also undergo risk stratification includingHolter monitoring and exercise ECG, unless contraindicated, to further definetheir risk of sudden death.[1] [28] [29]Risk factors for sudden death are asfollows:[1] [4] [28] [29] [30] [31] [32]
Non-sustained ventricular tachycardia on Holtermonitor.
Abnormal BP response to exercise: defined as arise in systolic BP of 20 mmHg during exercise. Medications mayaffect the BP response and should be considered inthe interpretation of exercise test results.
Massive hypertrophy (left ventricular wallthickness 30 mm).
Severe outflow obstruction by echocardiogram(left ventricular outflow tract obstruction >30 to 50mmHg). While severe obstruction is considered aminor risk factor for sudden death, the degree ofoutflow tract obstruction generally does not correlatewith the risk of sudden death. Medical therapy orsurgery to decrease outflow tract obstruction doesnot decrease the risk of sudden death.
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Family history of sudden death.
Personal history of unexplained syncope.
Prior cardiac arrest, or sustained ventriculartachycardia.
Patients with angina or ST depression on exercise ECG should be evaluatedfor ischaemia with nuclear imaging or CT arteriography if the likelihood ofcoronary artery disease (CAD) is relatively low. CT arteriography or cardiaccatheterisation are indicated if there is a higher likelihood of CAD given other
patient factors.[20] [29] [33] Myocardial bridging (tunnelling of coronaryarteries into the heart muscle) should also be considered in the setting ofangina or ischaemia. Cardiac catheterisation or CT arteriography can beused to evaluate bridging with specific attention given by the interpreter tothis possible diagnosis.[34] Ischaemia may be related to myocardial bridging,outflow tract obstruction, or massive hypertrophy with reduced myocardialperfusion. The presence of ischaemia is a weak risk factor for sudden death.
Genetic mutation analysisThe clinical utility of genetic testing has limitations. Currently identifieddisease-causing genes are thought to account for only 80% of cases of thedisease. Moreover, the sensitivity of commercially available genetic testingdepends on the number of genes screened by the particular laboratory andmay be
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While identification of a gene mutation in a patient indicates that thedevelopment of HCM is very likely, genotype-phenotype variability exists.Despite identical gene mutations, the gene mutation may manifest as HCM,restrictive cardiomyopathy, dilated cardiomyopathy, or no clinically apparentabnormality. For those without apparent disease, late-onset penetrance must
be considered.[37]Additionally, the risk of sudden death may be low or highfor the same mutation.[36]Genetic counselling should be available for patients who undergo genetictesting so that results can be reviewed and the clinical significanceappropriately determined. Irrespective of whether or not a gene mutation isidentified in an affected patient, the patient (mother or father) should beoffered genetic counselling before planned conception. Genetic counselling isan important component of care given the autosomal dominant inheritanceand the importance of screening in the offspring.[29]
Click to view diagnostic guideline references.Diagnostic criteriaAmerican College of Cardiology/European Society of
Cardiology Clinical Expert Consensus Document on
hypertrophic cardiomyopathy[28]1.LVH, typically asymmetric in distribution, in the
absence of another cardiac cause or systemiccondition capable of producing the magnitude ofhypertrophy, such as hypertension.
2.LVH is defined as a maximal LV wall thicknessabove the normal limit in an adult (>12 mm). Mild
hypertrophy related to intense athleticconditioning may occur but should normalise aftera period of deconditioning.
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The Cardiac Society of Australia and New Zealandguidelines for the diagnosis and management of
hypertrophic cardiomyopathy[45]
1.LVH, typically asymmetric in distribution, in theabsence of another cardiac cause or systemiccondition capable of producing the magnitude ofhypertrophy, such as hypertension.
2.LVH is defined as a maximal LV wall thicknessabove the normal limit in an adult (>12 mm). Mildhypertrophy related to intense athleticconditioning may occur but should normalise aftera period of deconditioning.
3.Myocardial biopsy is not needed for the diagnosisbut shows characteristic features of myocyte
hypertrophy, fibre disarray, and interstitial fibrosis.
Treatment Options
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Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
8/22/2019 Hipertrof Kmp
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Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andpriorcardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
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Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
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Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
8/22/2019 Hipertrof Kmp
35/68
Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
8/22/2019 Hipertrof Kmp
36/68
Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
8/22/2019 Hipertrof Kmp
37/68
Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
http://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-2&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-3&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-4&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-5&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-1&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-2&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-3&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-4&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=179173-5&optionId=expsec-2&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/monograph/409/treatment/evidence/score/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-28http://bestpractice.bmj.com/best-practice/monograph/409/resources/references.html#ref-288/22/2019 Hipertrof Kmp
38/68
Patient group
Treatment line Treatmenthide all
asymptomatic
at high risk ofsudden death
1st implantable cardioverter-
defibrillator + restriction
from high-intensity
athletics Strong risk factors for
sudden cardiac deathinclude non-sustained ventriculartachycardia onsequential Holtermonitorings, andprior cardiac arrest.Both are indicationsfor implantablecardioverter-defibrillator (ICD)
placement. Norandomisedcontrolled trialsstudying the effect ofICD placement havebeen performed in
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