MINISTRY OF HEALTH OF UKRAINE

National Pirogov Memorial Medical University, Vinnytsya

"IS CONFIRMED"
At methodical meeting of chair
Internal medicine №1
Head of the department

____________ prof. Stanislavchuk M. A.

«30» August 2021

METHODICAL RECOMMENDATIONS
For students

Subject matter Internal medicine

Substantial module № Features of patient management after cardiac surgery
Subject of the lesson Management of patients after surgery for hypertrophic
cardiomyopathy
Course 6
Faculty Medical № 1

Vinnytsia 2021
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1. Educational goal:
The student must know:
1. Aetiology and pathogenesis of Cardiomyopathies (CMP).
2. Modern classification of Cardiomyopathies.
3. Clinical symptoms of CMP.
4. Clinical symptoms of Hypertrophic cardiomyopathy .
5. Methods of diagnostics of Hypertrophic cardiomyopathy .
6. Principles and methods of treatment of Hypertrophic cardiomyopathy.
7. Indications and contraindications to surgical treatment.
8. Medical support of postoperative patients.

The student must be able:

1. To determine the Clinical symptoms of CMP.
2. To define the main risk factors of CMP.
3. To make the scheme of investigation for the determination of CMP.
4. To assess the investigation study results.
5. To determinate the treatment depending on CMP.
6. To prescribe the proper treatment for the patient with CMP.
7. To determine the Indications and contraindications to surgical treatment.
8. To prescribe the proper treatment for the patient after surgical treatment.

Topicality:
Cardiomyopathy is a complex disease process that can affect the heart of a person of any age. It
is a common problem throughout the world and is the most common diagnosis in persons receiving
supplemental medical financial assistance via the US Medicare program. Cardiomyopathy is an
important cause of morbidity and mortality among the world's ageing population.
The true incidence of CMP is unknown. Annular morbidity - 2-10 on 100.000.
The 5-year mortality rate – 50%

Recommendations for conducting of the lesson

The terms 'cardiomyopathy' usually used for conditions that primarily affect the heart muscle.
A contemporary definition for Cardiomyopathies (CMP) - is a myocardial disorder in which
the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease,
hypertension, valvular disease, and congenital heart disease sufficient to explain the observed
myocardial abnormality.
Cardiomyopathies include a variety of myocardial disorders that manifest with various
structural and functional phenotypes and are frequently genetic. Although some have defined
cardiomyopathy to include myocardial disease caused by known cardiovascular causes (such as
hypertension, ischemic heart disease, or valvular disease), current major society definitions of
cardiomyopathy exclude heart disease secondary to such cardiovascular disorders.
Cardiomyopathies (CMP) are conditions in which the normal muscular function of the
myocardium has been altered by specific or multiple etiologies, with varying degrees of physiologic
compensation for that malfunction.
Cardiomyopathies have multiple etiologies.
Persons with cardiomyopathy may have asymptomatic left ventricular (LV) systolic dysfunction,
LV diastolic dysfunction, or both. When the balance between malfunction and compensation is
disrupted such that cardiac output can no longer be maintained at normal LV filling pressures, the
disease process is expressed with symptoms that collectively compose the disease state known as
congestive heart failure (HF).
Because the etiology for CMP varies, so does the pathophysiology that may lead to its clinical
expression as CHF. Although the pathophysiologic mechanisms are different, the hemodynamic
consequences and neurohormonal abnormalities associated with the different causes of CMP remain
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very similar. Finding a specific cause for a CMP is often difficult, especially when it is multifactorial.
Traditionally, ischemic cardiomyopathy is listed as the most common cause of CMP in North America
and Europe.
Cardiomyopathies
Idiopathic CMP
 Dilated cardiomyopathy
 Hypertrophic cardiomyopathy
 Restrictive Cardiomyopathy
 Arrhythmogenic right ventricular cardiomyopathy
Nonclassificated cardiomyopathies
 Noncompacted myocardium
 Systolic dysfunction with minimal dilatation
 Mitochondrial involvement
 Mildly dilated congestive cardiomyopathy
Idiopathic CMP is the second most common cause, although this may partially reflect
undiagnosed etiologies such as infectious (viral) or toxic (ethanol-induced) CMP. For example, in
Africa, idiopathic congestive cardiomyopathy, endomyocardial fibrosis, and rheumatic heart disease
all are more prevalent than disease due to atherosclerotic coronary artery disease.
Specific cardiomyopathies
- Secondary to other cardiovascular disease (Ischemia, Hypertension, Valvular disease)
- Infectious (Viral, Rickettsial, Bacterial )
- Metabolic - Endocrine diseases - Hyperthyroidism, hypothyroidism, acromegaly,
myxedema, hypo- and hyperparathyroidism, other
- Diabetes mellitus
- Toxic (Drugs, Poisons, Alcohol)
- Collagen vascular disease (SLE, Rheumatoid arthritis)
- Infiltrative (Hemochromatosis, amyloidosis)
- Granulomatous (sarcoidosis)
- Neuromuscular disorders
- Muscular dystrophy - Limb-girdle (Erb dystrophy), Duchenne dystrophy,
fascioscapulohumeral
- Peripartum
- Immunological (Postvaccination, Transplant rejection)
- Specific cardiomyopathies (Myocarditis)

Dilated Cardiomyopathy (ESC; WHO /ISFC) - a clinical diagnosis characterized by dilation

and impaired contraction of the left or both ventricles that is not explained by abnormal loading
conditions or coronary artery disease.
Dilated cardiomyopathy is myocardial dysfunction producing heart failure in which ventricular
dilation and systolic dysfunction predominate.
Dilated cardiomyopathy (DCM) has unidentified cause (and many diseases produce as cardiac
dilation and sings, as DCM, - so-called Specific cardiomyopathies, secondary to other cardiovascular
disease). The most common cause in temperate zones is diffuse coronary artery disease (CAD) with
diffuse ischemic myopathy. More than 20 viruses can cause DCM; in temperate zones, coxsackievirus
B is most common. In Central and South America, Chagas' disease due to Trypanosoma cruzi is the
most common infectious cause.
Normal. Dilated cardiomyopathy
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Etiology and pathogenesis of DCM.

Dilated cardiomyopathy (DCM) represents the third most common cause of heart failure (HF)
and the most frequent cause of heart transplantation in the western world.
Genetic - Approximately 40% of cases are familial
An acquired dilated phenotype may result from a variety of factors including CAD, myocarditis,
tachyarrhythmias, alcohol abuse, drugs, catecholamines, toxins, and metabolic or endocrine
disturbances.
Recent studies on patients with idiopathic DCMP found evidence of viral particles in
endomyocardial biopsy specimens in up to two thirds of the patients.
In such patients, DCM start with acute myocarditis (probably viral in most cases), followed by a
variable latent phase, a phase with diffuse necrosis of cardial myocytes (due to an autoimmune
reaction to virus-altered myocytes), and chronic fibrosis.
Myocardium dilates, often leading to functional mitral or tricuspid regurgitation and atrial
dilation. DCM manifests hemodynamically as decreased cardiac output and increased pulmonary
venous pressure. Anatomically, the heart has greater LV cavity size with little or no wall hypertrophy.
The decrease in systolic function of the myocardium is by far the primary abnormality. This leads to an
increase in the end-diastolic and end-systolic volumes.
Progressive dilation can lead to significant mitral and tricuspid regurgitation, which may further
diminish the cardiac output and increase end-systolic volumes. Early compensation for systolic
dysfunction and decreased cardiac output is accomplished by increasing the stroke volume, the heart
rate, or both (cardiac output = stroke volume X heart rate), which is also accompanied by an increase
in peripheral tone.
The disorder affects both ventricles in most patients, only the left ventricle (LV) in a few
(unless with an ischemic etiology), and only the right ventricle (RV) rarely.
Clinical sings
LV dysfunction causes exertional dyspnea and fatigue due to elevated LV diastolic pressure and
low cardiac output. RV failure causes peripheral edema and neck vein distention. Manifestation of
congestive HF is decreased exercise tolerance, depending on the level of compensation. This leads to
symptoms of shortness of breath, orthopnea, dyspnea upon exertion, and edema. However, these
symptoms occur primarily in the late stages of cardiomyopathy, and atrial fibrillation and ventricular
dysrhythmias may be early signs of myocardial disease.
Palpate for heaves, shifted point of maximal impulse, and cardiomegaly (broad and displaced
point of maximal impulse, right ventricular heave).
The normal apical impulse should be approximately the size of a quarter and should be located
in one (fourth or fifth) intercostal space. The apical impulse is normally within 10 cm of the
midsternal line. In a person with DCM – diffuse, displaced apex beat.
Murmurs, tachycardia, S2 at the base (paradoxical splitting, prominent P2), S3, and S4 may be
noted. Remember that S3/S4 are low-frequency sounds heard best with the bell and that a prominent
pulmonic component of the S2 audible at the apex can be misinterpreted as an S3 if care is not taken
to distinguish the frequency of the sound. Gallops are almost always present.
Atrial fibrillation may be noted.
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Abdomen: Percussion and palpation of the liver may reveal hepatomegaly due to elevated
venous pressure, infiltrative disease, hepatojugular reflux, or ascites.
Infrequently the RV is predominantly affected in younger patients, and atrial arrhythmias and
sudden death due to malignant ventricular tachyarrhythmias are typical. About 25% of all patients
with DCM have atypical chest pain.
The ECG may show sinus tachycardia, low-voltage QRS complexes, and nonspecific ST-
segment depression with low voltage or inverted T waves. Left bundle branch block and Atrial
fibrillation are common.
Chest x-ray shows cardiomegaly usually of all chambers. Pleural effusion, particularly on the
right, often accompanies increased pulmonary venous pressure and interstitial edema.
Echocardiography shows dilated, hypokinetic cardiac chambers and rules out primary valvular
disorders. Segmental wall motion abnormalities, mural thrombus.
MRI is not routinely done but may be useful when detailed imaging of myocardial structure or
function is needed. In cardiomyopathy, MRI may show abnormal myocardial tissue texture.
Lab - In HF with reduced ejection fraction, natriuretic peptides have clinical utility for the
diagnosis and prognostic stratification. In fact, guidelines recommend dosage of brain natriuretic
peptide (BNP) or N-terminal pro-BNP at the time of the first evaluation and systematically during
follow-up.
DCM Complications:
- Heart failure
- Atrial arrhythmias (most common atrial fibrillation) and
- sudden cardiac death (SCD) due to malignant ventricular tachyarrhythmias
- Thromboembolism – most common in patients with atrial fibrillation.
Treatment of DCM - is treatment of heart failure.
ACEi - for patients with asymptomatic LV systolic dysfunction, in order to prevent or delay the
onset of HF, Patients with symptomatic LV systolic dysfunction, in order to reduce HF,
hospitalization, and death.
ARB - Patients with symptomatic LV systolic dysfunction, in order to reduce HF
hospitalization and death, unable to tolerate an ACE-I
Beta-blocker – Patients with symptomatic LV systolic dysfunction, in order to reduce HF,
hospitalization, and death
MRA - Patients with LV systolic dysfunction still symptomatic with an optimized dosage of
ACEi and beta-blocker, in order to reduce HF hospitalization and death
Sacubitril/Valsartan - Patients with LV systolic dysfunction (EF ≤ 35%) still symptomatic
(NYHA II–III) with an optimized dosage of ACEi (or ARB), beta-blocker, and MRA in order to
reduce HF hospitalization and death
Ivabradine – Patients with LV systolic dysfunction (EF ≤ 35%) still symptomatic, in sinus
rhythm and a resting heart rate ≥70 bpm, with an optimized dosage of ACEi(or ARB), beta-blocker,
and MRA in order to reduce HF hospitalization and cardiovascular death.
Aggressive treatment of heart failure and anticoagulants (for Atrial fibrillation) reduces risk of
complications, significant cardiac arrhythmias may be treated with antiarrhythmic drugs.
Permanent pacemakers may be required if AV block occurs (AV block during acute
myocarditis often resolves, so permanent pacemakers are usually not needed). If patients have a
widened QRS interval with a low LVEF and severe symptoms despite optimized medical treatment,
biventricular pacing should be considered.
An implantable cardioverter-defibrillator may be used to prevent sudden arrhythmia-induced
death.
Because prognosis is poor, patients with DCM may become candidates for heart transplantation
– Patients with LV systolic dysfunction (LVEF ≤ 35%), end-stage HF despite OMT in the absence of
contraindications, in order to increase survival, exercise capacity, and quality of life.
Prognosis generally is poor. About 20% died in the first year and then about 10%/yr thereafter;
about 40 to 50% of deaths are sudden, due to a malignant arrhythmia or an embolic event.
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However, both total mortality and SCD rate have been reduced in the last three decades: in the
1980s, SCD rate was up to 18% per year, but it was only around 2–3% in patients medically evaluated
in the early 2000.
This could have several explanations, including a better diagnostic definition, an earlier
diagnosis, the widespread of beta-blockers and mineralocorticoid receptor antagonists (the only drugs
significantly associated with a SCD reduction) and, finally, ICD and resynchronization therapy.

Hypertrophic cardiomyopathy (HCM) - is a congenital or acquired disorder characterized

by marked ventricular hypertrophy with diastolic dysfunction but without increased afterload (eg, from
valvular aortic stenosis, coarctation of the aorta, systemic hypertension).
HCM is a common genetic heart disease reported in populations globally. Inherited in an
autosomal dominant pattern, the distribution of HCM is equal by sex, although women are diagnosed
less commonly than men. The prevalence of HCM depends on whether subclinical or clinically evident
cases are being considered, is age dependent, and may have racial/ethnic differences.
The prevalence of unexplained asymptomatic hypertrophy in young adults in the United States
has been reported to range from 1:200 to 1:500.2 Symptomatic hypertrophy based on medical claims
data has been estimated at <1:3000 adults in the United States; however, the true burden is much
higher when unrecognized disease in the general population is considered.
Clinical definition of HCM - a disease state in which morphologic expression is confined solely
to the heart. It is characterized predominantly by LVH in the absence of another cardiac, systemic, or
metabolic disease capable of producing the magnitude of hypertrophy evident in a given patient and
for which a disease-causing sarcomere (or sarcomere-related) variant is identified, or genetic etiology
remains unresolved. (AHA / ACC 2020)
HCM is defined by the presence of a wall thickness ≥15 mm in one or more left ventricular
myocardial segments that is not solely explained by abnormal loading conditions.
Hypertrophic cardiomyopathy - a primary disorder characterized by:
- marked LV hypertrophy with normal or decreasing LV cavity,
- diastolic dysfunction
- high risk of Arrhythmias and Sudden Сardiac death.
Etiology and pathogenesis of HCM.
In up to 60% of adults with HCM, the disease is an autosomal dominant trait caused by
mutations in cardiac sarcomere protein genes (myosin-binding protein C, cardiac troponin I and T etc.)
A substantial proportion of patients with HCM are currently without any evidence of a genetic
etiology to their disease, including a subgroup who also have no other affected family members (“non-
familial” HCM). These observations suggest that other novel pathophysiologic mechanisms may be
responsible or contribute to phenotypic expression in these affected patients with HCM.
In HCMP, mutations may impair protein interactions, result in ineffectual contraction of the
sarcomere, and produce hypertrophy and disarray of myocytes.
Hypertrophy results in a stiff, noncompliant left ventricle (LV), which resists diastolic filling,
elevating end-diastolic pressure and increasing pulmonary venous pressure – LV diastolic dysfunction.
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In the most common form, the upper interventricular septum below the aortic valve is markedly
hypertrophied and thickened, with little or no hypertrophy of the LV posterior wall; this pattern is
called asymmetric septal hypertrophy.
During systole, the septum thickens, and sometimes the anterior leaflet of the mitral valve,
already abnormally oriented because of the abnormally shaped ventricle, is sucked toward the septum -
systolic anterior motion (SAM) - by a Venturi effect of high velocity blood flow, further obstructing
the outflow tract and decreasing cardiac output – LV outflow tract obstruction (LVOTO) - so-called
hypertrophic obstructive cardiomyopathy.
LVOTO, either at rest or with provocation, is present in ~75% of patients with HCM. Two
principal mechanisms are responsible for LVOTO: 1) septal hypertrophy with narrowing of the LVOT,
leading to abnormal blood flow vectors that dynamically displace the mitral valve leaflets anteriorly;
and 2) anatomic alterations in the mitral valve and apparatus, including longer leaflets as well as
anterior displacement of the papillary muscles and mitral valve apparatus, which makes the valve more
susceptible to the abnormal flow vectors.
Consequently, there is systolic anterior motion of the mitral valve leaflets (SAM), which leads
to LVOTO, high intracavitary pressures, and MR from the loss of leaflet coaptation. Systolic anterior
movement of the mitral valve (SAM) and mitral-septal contact is believed to be responsible for LVOT
obstruction in the majority of cases.
Apical hypertrophy can also occur but does not obstruct outflow.
Clinical sings
Many patients with HCM are asymptomatic and identified incidentally or as a result of
screening. The first clinical manifestation of the disease may be Sudden cardiac death, likely from
ventricular tachycardia or fibrillation.
Clinical history includes a detailed cardiac history and family history (3 generations) to identify
relatives with HCM or with unexpected/sudden death. Assessment of overall fitness and functional
capacity, with emphasis on training regimen and symptoms in response to exertion—chest pain,
dyspnea, palpitations, and syncope.
Coronary blood flow may be impaired, causing angina pectoris, syncope, or arrhythmias in the
absence of coronary artery disease. Typically, symptoms appear in age 20 - 40 and are exertional -
tachycardia decreases time for LV filling during diastole - symptoms appear mainly during exercise or
tachyarrhythmias. They include dyspnea, chest pain (usually resembling typical angina due to relative
coronary insufficiency), palpitations, and syncope.
Opposite angina, Nitrates worse chest pain due to decrease LV diastolic filling.
Because systolic function is preserved, fatigability is seldom reported.
Syncope usually occurs without warning during exertion either because outflow obstruction
worsens with the increased contractility or because of nonsustained ventricular or atrial arrhythmia.
Syncope is a marker of increased risk of sudden death, which is thought to result from ventricular
tachycardia or fibrillation.
The apex beat may have a sustained thrust due to LV hypertrophy. S4 is often present and is
associated with a forceful atrial contraction against a poorly compliant LV in late diastole.
Septal hypertrophy produces a systolic ejection-type murmur - crescendo-decrescendo murmur,
which is best heard at the left sternal edge in the 3rd or 4th intercostal space and radiates to the
suprasternal notch but not to the carotid arteries or neck - in patients with LV outflow tract obstruction
(LVOTO). SAM of the mitral valve leads to LVOTO and resultant harsh crescendo-decrescendo
systolic murmur best heard over the lower left sternal border.
The murmur and the gradient across the LV outflow tract increase with any reduces venous
return (and preload) - provocative maneuvers Valsalva maneuver, standing from the squatting
position, nitroglycerin.
Handgrip increases aortic pressure, thereby reducing the murmur's intensity. Mitral
regurgitation (MR) can occur mitral regurgitation murmur due to distortion of the mitral apparatus and
contributes to symptoms of dyspnea.
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Classically, patients with HCM have a systolic murmur, prominent apical point of maximal
impulse, abnormal carotid pulse, and a fourth heart sound. A carotid double pulsation, known as
pulsus bisferiens, and an S4 from a noncompliant left ventricle may be present.
Patients without LVOTO (provocable or resting) may have a normal physical examination.
The ECG usually shows voltage criteria for LV hypertrophy (S wave in lead V1 plus R wave in
lead V5 or V6 > 35 mm). Deep, narrow (“dagger-like”) Q waves in the lateral (V5-6, I, aVL) and
inferior (II, III, aVF) leads – signs of Asymmetrical septal hypertrophy. Deep symmetric T-wave
inversion in I, aVL, V5, and V6, ST-segment depression in the same leads – signs of ischemia of
hypertrophic LV wall.
Bundle branch block and arrhythmias: atrial fibrillation, supraventricular tachycardias, PACs,
PVCs, VT – are common.
Echocardiography can differentiate the forms of CMP.
A clinical diagnosis of HCM in adult patients can be established by imaging with 2D
echocardiography or cardiovascular magnetic resonance (CMR) showing a maximal end-diastolic wall
thickness of ≥15 mm anywhere in the left ventricle, in the absence of another cause of hypertrophy in
adults.
More limited hypertrophy (13–14 mm) can be diagnostic when present in family members of a
patient with HCM or in conjunction with a positive genetic test.
LVOT obstruction is a hallmark of HCM, and patients with HCM are currently classified based
on the presence or absence of LVOT obstruction either at rest or under stress conditions. LVOT
pressure gradients are routinely measured by echocardiography at rest and after physical exercise,
Valsalva manoeuvre or amyl nitrite administration.
Obstructive HCM is defined as a peak instantaneous Doppler LV outflow tract gradient of ≥30
mm Hg, - LVOTO.
Nonobstructive HCM - gradient across the LV outflow tract < 30 mm Hg in rest and exertion.
The presence of a peak LVOT gradient of ≥30 mm Hg is considered to be indicative of
obstruction, with resting or provoked gradients ≥50 mm Hg generally considered to be the threshold
for septal reduction therapy (SRT) in those patients with drug-refractory symptoms.
Differential diagnosis of HCM include physiologic remodeling of the athlete, long-standing
systemic hypertension, renal disease, or infiltrative diseases (amyloid cardiomyopathy). The likelihood
of HCM can be determined by identification of a diagnostic sarcomere mutation or inferred by marked
LV thickness > 15 mm and/or LVOT obstruction with systolic anterior motion (SAM) and mitral-
septal contact.
Annual mortality is 1 to 3% for adults but is higher for children. Prognosis is worse for young
patients with a family history of sudden death and for patients > 45 yr with angina or exertional
dyspnea.
HCM Treatment
 β-Blockers
 Rate-limiting Ca channel blockers
 Avoidance of nitrates, diuretics, ACE inhibitors
 Possibly implantable cardioverter-defibrillator
 Surgery
Beta-blockers were the first studied medication for treatment of dynamic outflow tract
obstruction and are generally considered the first-line agent for most patients with obstructive HCM.
Medications should be titrated to a dose where there is symptom benefit but not declare failure of beta-
blockade until there is demonstrated physiologic evidence of beta-blockade (ie, suppression of resting
heart rate).
Diltiazem and verapamil have both been demonstrated to provide relief of symptoms in
patients with obstructive HCM. Both of these agents can have vasodilating properties, in addition to
the negative inotropic and negative chronotropic effects, which can be limiting. This afterload-
reducing effect can be particularly dangerous in patients with very high resting gradients (>80 to 100
mm Hg) and signs of congestive heart failure.
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Disopyramide in HCM cardiomyocytes, it is best characterized as a multichannel blocker,
also blocking the late sodium channel, the calcium channel, stabilizing the ryanodine receptor release
of calcium from the sarcoplasmic reticulum, and markedly decreasing cytosolic calcium. In vitro, it
decreases early and late after depolarizations.
Drugs that reduce preload (nitrates, diuretics, ACE inhibitors, angiotensin II receptor
blockers) decrease chamber size and worsen symptoms and signs. Vasodilators increase the outflow
tract gradient and produce a reflex tachycardia that further worsens ventricular diastolic function.
Inotropic drugs (digitalis glycosides, catecholamines) worsen outflow tract obstruction, do not relieve
the high end-diastolic pressure, and may induce arrhythmias.
Possibly implantable cardioverter-defibrillator,
Pacemaker implantation
Surgery - Septal reduction therapy - has been advanced as preventing sudden death by dint of
its removing LV outflow tract obstruction, and thereby lowering afterload, ischemic and energetic
burdens from the myocardium. However, patients do die suddenly after myectomy, even when
performed at a skilled surgical center. The reduction in LV outflow gradient may not correlate with a
risk reduction for sudden death or overall mortality.
Septal reduction therapy (SRT):
- Left ventricular myomectomy,
- Catheter septal ablation
Mitral valve replacement,
Septal reduction therapy should only be performed as part of a dedicated HCM program. It is
generally reserved for patients whose symptoms are not relieved by medical therapy and impair quality
of life, usually consistent with NYHA functional class III or class IV.
Surgical septal myectomy is the gold standard for those patients with HCM who are
symptomatic with a resting LVOT gradient of ≥30 mmHg and refractory to maximal medical therapy.
Long-term survival after surgical myectomy is similar to an age-matched general population, and
recurrent outflow tract obstruction is rare.
In 1968, Morrow and colleagues pioneered the surgical technique of limited transaortic septal
myectomy (the “Morrow procedure”) in which a limited region of hypertrophied myocardium at the
basal septum was excised.
Transaortic extended septal myectomy is an appropriate treatment for the broadest range of
symptomatic patients with obstructive HCM. Techniques of myectomy have evolved and allow
gradient relief at any level of obstruction within the ventricle, with demonstrated mortality <1% and
clinical success >90% to 95%.
In patients with symptomatic obstructive HCM who have associated cardiac disease requiring
surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral
leaflet, intrinsic mitral valve disease, CAD, valvular aortic stenosis), surgical myectomy performed by
experienced operators provides the opportunity to correct all of the structural/anatomic issues with a
single procedure.
- Avoid septal reduction therapy in asymptomatic adults with HCM who have normal exercise
tolerance or whose symptoms are controlled/minimized on medical therapy.
- When septal reduction therapy is a feasible treatment option to relieve LVOT obstruction in
eligible patients, do not perform mitral valve replacement as an alternative therapy.
- Avoid performing alcohol septal ablation in (1) patients with HCM and comorbid conditions
that also require surgical repair, in whom myectomy can be performed concomitantly; (2) pediatric
patients with HCM (age <21 years); and (3) adults younger than 40 years in whom myectomy is a
feasible alternative therapy.
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Complications of septal myectomy surgery include possible death, arrhythmias, infection,

incessant bleeding, septal perforation/defect, and stroke.[
Catheter septal ablation
Percutaneous transluminal septal myocardial ablation, otherwise known today as alcohol septal
ablation (ASA), was introduced by Sigwart in 1994 as an alternative septal reduction therapy at a time
when the operative mortality from septal myectomy was much higher than it is today (up to 8%). The
procedure is minimally invasive and is performed via the same approach as cardiac catheterization and
coronary angiography.
The procedure involves infusing 96% ethanol down the first septal branch of the left anterior
descending artery and inducing a therapeutic infarction of the proximal interventricular septal
myocardium. This leads to a remodeling of the septum, which decreases the marked septal thickening
characteristic of HCM and results in a decrease of the gradient across the LV outflow tract.
Alcohol septal ablation has the potential for greater patient satisfaction because of the absence
of a surgical incision and general anesthesia, less overall discomfort, and a much shorter recovery
time.
Septal alcohol ablation is associated with a higher risk of AV block, requiring permanent
pacemaker implantation and larger residual LV outflow tract gradients. Alcohol septal ablation greater
need for repeat intervention because of residual obstruction; repeat alcohol septal ablation or
myectomy is reported in 7% to 20% of patients after alcohol septal ablation.
Septal Radiofrequency Ablation may be less effective in patients with extensive septal scarring
on CMR and in patients with very severe hypertrophy (≥30 mm).
The likelihood of implantation of a permanent pacemaker is 4- to 5-fold higher after septal
ablation than after septal myectomy.
Historically, concomitant mitral valve replacement was proposed to resolve the associated SAM
and mitral regurgitation (1976). However, it is now accepted that adequate septal myectomy is capable
of correcting SAM and thus the mitral regurgitation in the majority of patients, and that mitral valve
repair or replacement should only be undertaken for those patients with primary mitral valve
pathology, such as mitral valve prolapse. The main surgical principle is to separate connections
between the subvalvular apparatus and the septum or free wall while maintaining the attachments to
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the anterior leaflet (2004). An annuloplasty ring may be required to minimise the risk of recurring
SAM after the repair.
The addition of mitral valve replacement to myectomy increases hospital mortality (>10-fold)
and length of hospitalization compared with patients undergoing isolated septal myectomy (2020).

Key perspectives from the 2020 AHA/ACC guideline for the management of patients with
hypertrophic cardiomyopathy (HCM)
1. Shared decision making is recommended for all aspects of HCM care including genetic testing,
activity, lifestyle, and therapy choices.
2. Referral to an HCM center of excellence should be considered to address complex management
decisions.
3. Initial diagnostic evaluation for all HCM patients should include a comprehensive physical
exam with a three-generation family history. Clinical features associated with HCM
phenocopies should be assessed.
4. An initial electrocardiogram (ECG) and 24- to 48-hour ambulatory ECG monitoring is
recommended followed by surveillance ECG every 1-2 years. For palpitations or
lightheadedness, extended monitoring is recommended, which should only be considered
diagnostic if symptoms occurred while monitoring.
5. For all patients with suspected HCM, a transthoracic echocardiogram (TTE) is recommended.
If resting left ventricular outflow tract (LVOT) gradient is <50 mm Hg, provocative testing
should be performed. For symptomatic patients without provoked gradients, an exercise
echocardiogram should be performed. For clinically stable HCM patients, surveillance
echocardiograms should be considered every 1-2 years to assess for changes in extent of
hypertrophy, obstruction, and cardiac function.
6. Cardiac magnetic resonance imaging should be obtained in HCM patients when
echocardiography is inconclusive, if there is a suspicion for an alternative diagnosis (like
infiltrative/storage diseases), for sudden cardiac death risk stratification, and may be needed for
selection and planning of septal reduction therapy.
7. For HCM patients with symptoms of myocardial ischemia, computed tomography (CT) or
invasive coronary angiography should be considered. Prior to surgical myectomy, invasive or
CT angiography should be obtained. For patients who are candidates for septal reduction
therapy but there is uncertainty regarding presence of LVOT obstruction on noninvasive
studies, invasive hemodynamic assessment is recommended.
8. Genetic testing should be offered to HCM patients to elucidate the genetic basis and to allow
for family screening. Pre- and post-testing genetic counseling is recommended for individuals
undergoing genetic testing. For HCM patients with genetic variants of uncertain significance,
serial re-evaluation of test results is recommended to assess for variant reclassification, as this
may trigger testing for family members. Preconception and prenatal reproductive and genetic
counseling should also be offered.
9. In first-degree relatives of patients with HCM, clinical screening should include ECG and TTE
during the initial evaluation followed by periodic follow-up according to their age (1-2 years in
adolescents, 3-5 years in adults) or if clinical status changes. If the proband has a pathogenic or
likely pathogenic variant on genetic testing, cascade genetic testing should be offered. For
relatives who test negative on cascade genetic test, additional clinical screening is not
recommended.
10. For HCM patients with cardiac arrest or sustained ventricular tachycardia (VT), single-chamber
transvenous or subcutaneous implantable cardioverter-defibrillator (ICD) implantation is
recommended as a Class I recommendation. As a Class IIa recommendation, it is reasonable to
offer an ICD for patients with massive LV hypertrophy ≥30 mm, history of suspected cardiac
syncope, LV apical aneurysm, systolic dysfunction with ejection fraction (EF) <50%, or family
history of sudden cardiac death due to HCM. For patients without these risk factors, ICDs
 12
should not be implanted, especially for the sole purpose of participation in competitive
athletics.
11. For symptomatic HCM patients with LVOT obstruction, nonvasodilating beta-blockers (BBs)
are recommended. If BBs are ineffective or not tolerated, verapamil or diltiazem are
recommended. Verapamil and diltiazem are contraindicated in case of hypotension, severe
dyspnea at rest, children <6 weeks old, and for resting gradients over 100 mm Hg. For
symptoms refractory to all these agents, disopyramide may be added or septal reduction
therapy may be offered at high-volume centers. If surgical septal reduction is contraindicated,
alcohol septal ablation can be considered at experienced centers.
12. For symptomatic patients with nonobstructive HCM and preserved LVEF, BBs, verapamil, or
diltiazem are recommended.
13. For patients with HCM and clinical atrial fibrillation, anticoagulation is recommended,
irrespective of CHA2DS2-VASc score, with direct-acting oral anticoagulants as first line
followed by warfarin. For HCM patients with subclinical atrial fibrillation, anticoagulation is
recommended if atrial fibrillation lasts for over 24 hours.
14. For HCM patients with VT or recurrent ICD shocks despite BB use, antiarrhythmic therapy
with amiodarone, mexiletine, sotalol, or dofetilide may be considered. If VT remains refractory
to antiarrhythmics, heart transplant evaluation should be considered.
15. For HCM patients who develop systolic dysfunction with EF <50%, coronary artery disease
should be ruled out and guideline-directed therapy for heart failure with reduced EF should be
initiated. For patients with New York Heart Association class III-IV symptoms, a
cardiopulmonary stress test should be considered to quantify functional limitation and help
with decision making regarding advanced heart failure therapies.
16. For HCM patients participating in athletics, comprehensive evaluation and shared decision
making regarding risk of participating in sports is recommended. For most HCM patients, mild
to moderate, recreational, noncompetitive exercise for the purpose of leisure is beneficial.
17. For pregnant HCM patients, BBs should be continued with monitoring of fetal growth and care
should be coordinated between cardiology and obstetrics. If anticoagulation is indicated for
atrial fibrillation or other reasons, low molecular weight heparin or warfarin (if maximum dose
<5 mg daily) are recommended

Restrictive cardiomyopathy
 Restrictive cardiomyopathy (RCM) - a myocardial disease characterized by restrictive filling
(resist diastolic filling) and reduced diastolic volume of either one or both ventricles with normal or
near-normal systolic function and wall thickness.
RCM refers to a group of disorders in which the heart chambers are unable to fill with blood
properly (due to diastolic relaxation abnormality) because of stiffness of the heart.
RCM is a rare disease of the myocardium - 5% of all cases of primary heart muscle disease. 2-
year mortality rate in patients with advanced myocardial fibrosis - 35-50%.
Etiology and pathogenesis of RCM.
RCM may be idiopathic or associated with other diseases.
Idiopathic / primary CMP - heart muscle disease of unknown cause that is manifested by heart
failure and restrictive hemodynamics but without significant ventricular hypertrophy, endocardial
thickening or fibrosis, associated eosinophilia, or other diagnostically distinct histopathological
changes.
Infiltrative (Between Myocytes)
 Amyloidosis
 Primary (light chain amyloid)
 Familial (abnormal transthyretin)
 Senile (normal transthyretin or atrial peptides)
 Inherited metabolic defects
Storage (Within Myocytes)
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 Hemochromatosis (iron)
 Inherited metabolic defects
 Fabry disease
 Glycogen storage disease (II, III)
Fibrotic
 Radiation
 Scleroderma
Endomyocardial
 Possibly related fibrotic diseases
 Tropical endomyocardial fibrosis
 Hypereosinophilic syndrome (Löffler endocarditis)
 Carcinoid syndrome
 Radiation
 Drugs: e.g., serotonin, ergotamine
Endocardial thickening or myocardial infiltration (sometimes with death of myocytes, papillary
muscle infiltration, compensatory myocardial hypertrophy, and fibrosis) may occur in one, typically
the left, or both ventricles with mitral or tricuspid valves dysfunction, leading to regurgitation. If nodal
and conduction tissues are affected, the sinoatrial node and AV dysfunctions (block).
The main hemodynamic consequence is diastolic dysfunction with a rigid, noncompliant
ventricle, impaired diastolic filling, and high filling pressure, leading to pulmonary venous
hypertension.
Patients typically have diastolic heart failure: systolic function is normal, but LV has increased
diastolic stiffness and cannot fill adequately at normal diastolic pressures, leading to a reduction in
cardiac output due to reduced LV filling volume.

History of RCM includes signs of CHF as result of decreased stroke volume and cardiac output:
worsening shortness of breath, progressive exercise intolerance, orthopnea, paroxysmal nocturnal
dyspnea, fatigue, later - abdominal discomfort or liver tenderness, weight loss, in terminal stage -
cardiac cachexia.
Chest pain, primarily in patients with amyloidosis or due to angina or chest pain mimicking
myocardial ischemia possibly due to myocardial compression of small vessels.
Atrial and ventricular arrhythmias and AV block are common.
Physical signs include symptoms of RV failure: jugular venous distention, pitting edema of the
lower extremities, hepatomegaly due to fluid or amyloid infiltration, ascites, pleural effusions.
Easy bruising, periorbital purpura, macroglossia, and other systemic findings (eg, carpal tunnel
syndrome) may be evidence of amyloidosis.
Cardiac system examination demonstrate decreased pulse volume, normal S 1 and S 2 heart
sounds, Loud early diastolic filling sound (S 3), systolic murmurs due to mitral and tricuspid valve
regurgitation (usually not hemodynamically significant).
Thromboembolic complications can have one third of patients: pulmonary emboli secondary to
legs viens trombosis; in atrial fibrillation - left atrial clots and systemic emboli.
For all patients with suspected RCM, a transthoracic echocardiogram (TTE) is recommended. It
shows normal left ventricle (LV) - nondilated or reduced in cavity size, nonhypertrophied, normally
contracting with normal LV ejection fraction. Tissue Doppler imaging frequently suggests elevated LV
 14
filling pressures, restriction of diastolic filling. Other common findings include dilated atria. Mural
thrombus and cavity obliteration are features of obliterative RCM.
In amyloidosis an unusually bright echo pattern from the myocardium may be observed.
Technetium-99m pyrophosphate cardiac imaging is also useful in differentiating immunoglobulin light
chain (AL) from transthyretin-related (ATTR) cardiac amyloid. Identifying the type of amyloid has
implications for treatment, genetic counseling, and overall prognosis.
Chest X-ray - signs of CHF without cardiomegaly.
ECG - nonspecific changes, conduction disturbances and low-voltage QRS complexes are
common with amyloidosis or sarcoidosis.
Serum brain natriuretic peptide (BNP) level - nearly normal in patients with constrictive
physiology of heart failure and grossly elevated in patients with restrictive physiology, despite nearly
identical clinical and hemodynamic presentation.
The histologic features include interstitial fibrosis.
Eosinophilic cardiomyopathy and endomyocardial fibrosis/.
Loeffler endocarditis and endomyocardial fibrosis are RCM, defined as diseases of the heart
muscle that result in impaired ventricular filling with normal or decreased diastolic volume of either or
both ventricles.
Systolic function and wall thickness may remain normal, especially early in the disease.
Eosinophilic cardiomyopathy (Loeffler endocarditis, Löffler's syndrome) - a subcategory of
hypereosinophilic syndrome with primary cardiac involvement, begins as an acute arteritis with
eosinophilia, followed by thrombus formation on the endocardium, chordae, and atrioventricular (AV)
valves, progressing to fibrosis. Loeffler endocarditis is a rare RCM caused by abnormal
endomyocardial infiltration of eosinophils, with subsequent tissue damage from eosinophils’s
degranulation, leading to fibrosis.
Although an uncommon entity, it is still a disease with significant morbidity and mortality.
Hypereosinophilic syndrome (HES) mostly includes temperate and tropical areas of Asia, Africa.
Stages of eosinophilic endomyocardial disease (Adapted from Alderman, 1999):
1 - Necrotic stage (early stage – 1-5 week)
Hypereosinophilia with Acute myocarditis (20-50%) and systemic illness (20-30%) due to
arteriitis : Fever, Sweating, Chest pain, Lymphadenopathy, Splenomegaly
Anorexia, Weight loss, Cough, Pulmonary infiltrates, Skin and retinal lesion,
2 - Thrombotic stage (10-12 months) - Thrombotic emboli (10-20%):
Cerebral, splenic, renal, and coronary infarction, Splinter hemorrhages
3 - Fibrotic stage (late stage)
Restrictive myopathy (10%) - AV valvular regurgitation, Right and left heart failure
Pathologic specimens in Loeffler endocarditis (LE) show eosinophilic myocarditis, a tendency
toward endomyocardial fibrosis and clinical manifestations of thromboembolism, and acute heart
failure.
Endomyocardial fibrosis, which is observed exclusively in equatorial Africa and less frequently
in Asia with India and South and Central America, in young adults of lower socioeconomic status, was
believed to be the end stage of eosinophilic endomyocarditis.
It is characterized by fibrosis of the LV and RV endocardium which cause restrictive
cardiomyopathy.
It is 15-25% of heart failure deaths in equatorial Africa. 80-90% die within 1-2 years.
Severe prolonged eosinophilia (hypereosinophilic syndrome) due to any cause (eg, allergic,
autoimmune, eosinophilic leukemia, carcinoma, lymphoma, drug reactions, parasites or idiopathic) can
lead to eosinophilic infiltration of the myocardium. The intracytoplasmic granular content of activated
eosinophils is believed to be responsible for the toxic damage to the heart.
This eosinophilic cardiomyopathy, also known as Loeffler endocarditis, is associated with
endomyocardial fibrosis, intraventricular thrombus formation, and obliteration of the ventricular cavity
in its late stages. The fibrosis of the endocardium may extend to involve the atrioventricular valves and
cause regurgitation. Two forms of endomyocardial fibrosis exist—an active inflammatory eosinophilia
 15
and chronic endomyocardial fibrosis. This condition demonstrates pathology that is similar to Loeffler
endocarditis and is grouped under obliterative RCM. However, endomyocardial fibrosis now is
considered a separate entity because it does not exhibit eosinophilia.
The hallmark characteristic of the condition is the fibrotic obliteration of the affected ventricle,
focal or diffuse endocardial thickening and fibrosis which leads to restrictive physiology.
Clinical
History: Initial cardiac involvement in about 20-50% of cases: patients may present with weight
loss, fever, cough, rash, and symptoms of congestive HF (weakness, dyspnea); chest pain is rare.
Physical: Signs of biventricular failure (eg, pedal oedema, elevated jugulovenous pressure,
pulmonary oedema, third heart sound [S3] gallop). Murmur of mitral regurgitation - in patients with
LV lesion. Systemic embolism is frequent and may lead to neurologic and renal dysfunction.
It is important to suspect RCM in any patient with a normal or close to normal systolic function
and evidence of diastolic dysfunction with a restrictive filling pattern on echocardiogram.
For those that present symptomatically, there is a wide range of presentations. Some may present
in full-blown heart failure (jugular venous distension, ascites, lower extremity edema, and less
commonly pulmonary edema). Some may complain of poor exercise tolerance or be newly diagnosed
with an arrhythmia such as atrial fibrillation. In the less fortunate cases, some present as sudden
cardiac arrest (SCD). Other less common presentations include ischemia, thrombus, and misdiagnosed
as hypertrophic cardiomyopathy with left ventricular outflow obstruction.
When performing the evaluation and physical in a patient suspected of RCM, it is essential to
look for extracardiac manifestations such as carpal tunnel, which may be present in amyloidosis or
bilateral hilar infiltrates seen in sarcoidosis.
Hemochromatosis may present with the classic bronze skin, cirrhosis, arthralgias, and
endocrinopathies such as diabetes mellitus.
Lab Studies: CBC counts - eosinophilia should not be considered mandatory for the diagnosis of
Loeffler endocarditis.
Cytogenetics, fluorescent in situ hybridization (FISH), and molecular analysis.
Chest radiograph - Atrial dilatation causing increased cardiothoracic ratio, normal ventricular
size, and possible bilateral pleural effusions.
ECG: some nonspecific ST-T wave changes, but rhythm disorders (notably atrial fibrillation),
conduction abnormalities, low QRS voltage, atrial hypertrophy.
The echocardiographic hallmark of RCM includes a restrictive ventricular filling with relatively
preserved LV systolic function: - nondilated, normally contracting, nonhypertrophied LV and marked
dilatation of both atria; normal or reduced the ventricular cavity size.
Differential Diagnosis: Constrictive pericarditis is the most commonly mistaken for RCM. Juglar
venous distension, Kussmaul sign, and diastolic sounds are both seen in RCM and Constrictive
pericarditis. However, there are some subtle differences. For example, S3 and elevated BNP are far
more common in RCM. Whereas a pericardial knock, pericardial calcifications on chest x-ray,
pericardial thickening on imaging, and BNP levels less than 100 are more likely seen in constrictive
pericarditis. Furthermore, one clear difference between the two diseases is the presence of ventricular
interdependence. Ventricular dependence seen only in constrictive pericarditis is described as an
increased filling of one of the ventricles only, with a reciprocal decreased filling of the other ventricle.
Complications of RCM may include:
Thromboembolism
Dysrhythmias
Heart failure
Cardiac cirrhosis
Extra-cardiac manifestations depending on etiology.
Treatment for RCM includes treating the underlying cause and heart failure symptoms that may
arise secondary to the disease. Currently, there is no cure for RCM, but there are some treatments
available to alleviate the symptoms of the disease.
 16
For heart failure symptoms, diuretics are the mainstay of treatment to reduce volume overload
but must be monitored closely to prevent excessive diuresis as patients with RCM rely on high filling
pressures to maintain cardiac output. The use of beta-blockers or calcium channel blockers is
sometimes introduced to increase the filling time. They may also be beneficial in
treating dysrhythmias, which are common in this patient population. Angiotensin-receptor blockers
may also be used, especially if concurrent systolic heart failure develops.
For sarcoidosis, antiarrhythmics are a common therapy choice due to the high incidence of
conduction disease. Immunosuppressive agents such as corticosteroids and steroid-sparing agents are
also sometimes used to treat sarcoidosis.
For hemochromatosis, the treatment of choice is therapeutic phlebotomy. Advanced heart failure
treatment, such as cardiac transplant or left ventricular assist devices, may be appropriate for some
patients.
Ultimately, the choice of a specific therapy depends on the clinical condition, the risk of
dangerous events, and the ability of the patient to tolerate the therapy.
The treatment of Loeffler endocarditis consists of correctly identifying the condition before the
end-stage fibrosis occurs; administration of corticosteroids, cytotoxic agents (eg, hydroxyurea), and
interferon to suppress the intense eosinophilic infiltration of the myocardium.
Surgical Care:
Pacemaker Implantation
Patients with idiopathic restrictive cardiomyopathy (RCM) may have fibrosis of the sinoatrial
and atrioventricular nodes that result in complete heart block and, therefore, require permanent pacing.
If cardioversion to treat atrial fibrillation is attempted, particularly in patients with amyloidosis, the
abnormal sinus node may fail as an effective pacemaker. Patients with sinus node dysfunction and/or
advanced conduction system disease also require treatment with implantation of a pacemaker.
Endomyocardectomy - In the fibrotic stage of Loeffler endocarditis, surgical therapy, with
excision of the fibrotic endocardium and replacement of the mitral and tricuspid valves, is palliative
but may provide symptomatic improvement. The operative mortality is in the range of 15% to 25%.
Cardiac transplantation or ventricular mechanical support (left ventricular assist device
[LVAD]) therapy can be considered in highly selected patients with refractory symptoms who have
idiopathic or familial restrictive cardiomyopathy (RCM) and amyloidosis, hemochromatosis, cardiac
sarcoidosis.
Elevated pulmonary vascular resistance excludes patients from cardiac transplantation, and
LVAD implantation may be required as a bridge to transplantation.
Cardiac transplantation is a widely accepted treatment to improve long-term survival in patients
with advanced RCM. Unfortunately, patients face long waiting periods. Many develop irreversible
pulmonary hypertension and die from heart failure complications before they can receive a donor
heart. Persistent right heart failure, thickened left ventricular walls and small left ventricular chamber
sizes make LVAD implantation challenging in RCM.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by
the patchy replacement of myocardium by fatty or fibrofatty tissue. These changes lead to structural
abnormalities including right ventricular enlargement and wall motion abnormalities that can be
detected by echocardiography, angiography, and MRI. ARVC/D is a genetically heterogeneous
disorder, since it has been linked to several chromosomal loci. Myocarditis may also be a contributing
etiological factor.
Patients are typically diagnosed during adolescence or young adulthood.
Presenting symptoms are generally related to ventricular arrhythmias.
Concern for the risk of sudden cardiac death may lead to the implantation of an intracardiac
defibrillator (ICD).
 17
Tests for the determining of basis knowledge
Task #1
A 50-year-old man comes to the physician for the evaluation of recurrent palpitations and a
feeling of pressure in the chest for the past 6 months. He also reports shortness of breath when walking
several blocks or while going upstairs. There is no personal or family history of serious illness. He
does not smoke. He has a 30-year history of drinking 7–10 beers daily. His temperature is 37°C
(98.6°F), pulse is 110/min, respirations are 18/min, and blood pressure 130/80 mm Hg. Pulse oximetry
on room air shows an oxygen saturation of 92%. There are jugular venous pulsations 9 cm above the
sternal angle. Crackles are heard at both lung bases. Cardiac examination shows an S3 gallop and
a displaced point of maximum impulse. There is pitting edema below the knees.
1. Make a diagnosis.
2. Which of the following is the most appropriate step in the management of the
underlying cause of this patient's current condition?
3. Specify the tactics of patient management, Prescribe therapy with name of the group of
drugs and the representatives.

Task #2
16-year-old boy comes to the physician for a routine health maintenance examination. He feels
well. He has no history of serious illness. He is at the 60th percentile for height and weight. Vital signs
are within normal limits. The lungs are clear to auscultation. A grade 3/6 ejection systolic murmur is
heard along the lower left sternal border. The murmur decreases in intensity on rapid squatting and
increases in intensity when he performs the Valsalva maneuver.
1. Make a diagnosis.
2. This patient is at increased risk for which of the following complications?
3. Specify the tactics of patient management, Prescribe therapy with name of the group of drugs
and the representatives.

Recommended literature :
А. Main:
1. Davidson’s Principles and Practice of Medicine, 2020
Б. Additional:
1. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic
Cardiomyopathy / A Report of the American College of Cardiology/American Heart Association Joint
Committee on Clinical Practice Guidelines - CirculationVol. 142, No. 252020 AHA/ACC Guideline
for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy
2. 2019 HRS Expert Consensus Statement on Evaluation, Risk Stratification, and Management
of Arrhythmogenic Cardiomyopathy // ACC, 2020
3. Dilated Cardiomyopathy // https://doi.org/10.1007/978-3-030-13864-6 // Gianfranco Sinagra,
Marco Merlo, Bruno Pinamonti// 2019

Indications for Surgery in Obstructive Hypertrophic Cardiomyopathy - Mark V. Sherrid/

https://doi.org/10.1161/JAHA.120.019419 Journal of the American Heart Association.
2021;10:e019419

Methodic chart made by Ph.D. Berko G.K.