Cardiomyopathy

Tariku Fekadu
(MD, Internist)
 Outline
• Definition
• Classification
• DILATED CMP
• Arrhythmogenic Cardiomyopathy
• PPCM
• STRESS- INDUCED CMP
• HCM
 Definition
• There is, at present, no universal definition of
cardiomyopathy.
• An American Heart Association definition describes
cardiomyopathies as
– “A heterogenous group of diseases of the associated with
mechanical and/or electrical dysfunction that usually (but not
invariably) exhibit inappropriate ventricular hypertrophy or
dilation and are due to a variety of causes and frequently are
genetic. Cardiomyopathies either are confined to the heart or
are part of a generalized systemic disorder often leading to
cardiovascular death or progressive heart failure-related
disability.”
• The European Society of Cardiology 2007:
– 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 cause the observed myocardial
abnormality
Primary Cardiomyopathies
 Secondary Cardiomyopathies
• Infiltrative*
– Amyloidosis
– Gaucher disease†
– Hurler’s disease†
– Hunter’s disease†
• Storage‡
– Hemochromatosis
– Fabry’s disease†
– Glycogen storage disease†
• Toxicity
– Drugs, heavy metals, chemical
agents
• Endomyocardial
– Endomyocardial fibrosis
– Hypereosinophilic syndrome
(Löeffler’s endocarditis)
• Inflammatory (granulomatous)
– Sarcoidosis
• Endocrine
– Diabetes mellitus†
– Hyperthyroidism
– Hypothyroidism
– Hyperparathyroidism
– Pheochromocytoma
– Acromegaly
• Nutritional deficiencies
– Beriberi (thiamine), pellagra,
scurvy, selenium, carnitine,
kwashiorkor
• Cardiofacial
• Autoimmune/collagen
– Systemic lupus erythematosis
– Dermatomyositis
– Rheumatoid arthritis
– Scleroderma
– Polyarteritis nodosa
– Electrolyte imbalance
• Consequence of cancer therapy
– Anthracyclines: doxorubicin
(adriamycin), daunorubicin
– Cyclophosphamide
– Radiation
– Noonan syndrome†
– Lentiginosis†
– Neuromuscular/neurological
– Friedreich’s ataxia†
– Duchenne-Becker muscular dystrophy†
– Emery-Dreifuss muscular dystrophy†
– Myotonic dystrophy†
– Neurofibromatosis†
– Tuberous sclerosis†
 THE DILATED CARDIOMYOPATHIES

• DCM is characterized by a dilated left ventricle

with systolic dysfunction that is not caused by
ischemic or valvular heart disease.
• it is the third most common cause of heart
failure and the most frequent cause of heart
transplantation.
4 chamber enlargement
4 types

dilated CM

arhythmogenic RV CM
• Dilated Cardiomyopathy
– progressive heart failure and decline in LV
contractile function,
– ventricular supraventricular arrhythmias,
conduction system abnormalities,
thromboembolism,
– sudden or heart failure–related death.
 Approach to pt with DCM
• full history,
• risk factors for coronary artery disease,
• the duration of symptoms
• Angina may occur, even in the absence of epicardial
coronary disease, but SHOULD raise the possibility of
coronary artery disease, either coexistent or as a
major causative factor.
• alcohol consumption .
• A family history is essential,
– suggestive of heart failure , sudden cardiac death,
• Findings on clinical examination reflect the
biventricular dysfunction present in DCM
• Electrocardiography frequently reveals
– nonspecific ST-T wave changes, or bundle
branch block .
– Pathologic Q waves may be present,
• Echocardiography reveals biventricular dilation,
which can range from mild to severe, as can LV
systolic dysfunction
– LV wall thickness is usually within the normal range,
– Most commonly, global LV hypokinesis is present,
– Disproportionate thinning of a dyskinetic wall
should raise the possibility of coronary artery
disease rather than primary cardiomyopathy.
– Mitral and tricuspid regurgitation is frequently
present
• impaired leaflet coaptation, the mitral and tricuspid
valves appear to be structurally normal,
– Diastolic function in DCM ranges from normal to
restrictive
– A restrictive pattern is most commonly seen in
patients with volume overload in
“decompensated” heart failure and often improves
with initiation of diuretic or vasodilator therapy
• Coronary angiography should be considered in all patients
– who have risk factors for coronary artery disease or
– Who are of an age at which this may be a causative factor.
• Alternatively, computed tomography (CT) coronary angiography may be used,.

• Cardiac magnetic resonance imaging (MRI) can be helpful in evaluating

cardiomyopathies.

• MRI is able to evaluate the extent of myocardial fibrosis in DCM and may
provide information complementary to that obtained with cardiac biopsy.
• Unless a specific condition is suspected, cardiac biopsy is often
unrewarding in the evaluation of DCM, but it may occasionally provide an
unexpected diagnosis.
 Genetics of Dilated Cardiomyopathy

• Extensive family-based studies have shown that if clinical screening with an

electrocardiogram (ECG) and/or echocardiogram is conducted in the first-
degree family members of patients with DCM, evidence of DCM will be found
in at least 20% to 35% of them, thereby establishing a diagnosis of
familial DCM.

• Recent studies in families with familial DCM suggest that a genetic cause
can be identified in at least 30% of cases and perhaps in as high as 40% to
50% as extrapolated from studies of individual genes or small numbers of
genes in gene discovery publications .

• Patients with DCM typically have an asymptomatic phase for many

years before symptomatic heart failure, an arrhythmia, or an embolic event
develops later in the course of the disease.
• The time span needed for clinical disease to
develop illustrates the remarkable ability of the
myocardium to maintain normal—or close to
normal—cardiac output and filling pressure for years
despite easily detectable asymptomatic DCM.
• SO family history is much less sensitive than clinical
screening via echocardiography in detecting DCM
among family members of an individual with a
new diagnosis of idiopathic DCM
 Therapy for Dilated Cardiomyopathy

• Therapy for DCM is similar to that for all types of systolic

dysfunction .
• avoidance of excessive dietary sodium is crucial.
• Beta-blocking drugs and ACE inhibitors/angiotensin receptor
blockers (ARBs) are the mainstay of therapy to prevent
progressive disease, even in the absence of symptoms,
• diuretics are the cornerstone of therapy to reduce peripheral
edema and pulmonary congestion in those with
symptomatic disease, with the addition of aldosterone
antagonists (spironolactone and eplerenone) in more
advanced cases.
• In selected patients, cardiac resynchronization
therapy should be considered,
• sometimes even early in the course of the
disease, and in those with advanced disease,
referral for a ventricular assist device or
cardiac transplantation may be needed
Arrhythmogenic Cardiomyopathy
• ACM is a genetically determined
cardiomyopathy characterized by fibrofatty
replacement of the myocardium.
• Formerly called “arrhythmogenic right
ventricular dysplasia/cardiomyopathy,”
– biventricular involvement occurs in up to 50%
of cases and that a small proportion of cases
affect predominantly the left ventricle .
• The disorder is conceptualized as having three stages:
1. an early subclinical phase -in which imaging studies are
negative but during which sudden cardiac death can still occur;
2. next, a phase in which (usually) right ventricular (RV)
abnormalities are obvious without any clinical manifestation of RV
dysfunction but with the development of symptomatic ventricular
arrhythmia; and
3. finally, progressive fibrofatty replacement and infiltration of the
myocardium leading to severe RV dilation and aneurysm
formation and associated right-sided heart failure.
4. LV dilation and failure may also arise at this stage or may occur
later (sometimes referred to as phase 4).
• The electrical manifestations of ACM are a reflection of the
pathologic disturbance.
• In the early stage, slow conduction and electrical uncoupling
may lead to a fatal arrhythmia.
• As the disease progresses, fibrofatty infiltration results in
inhomogeneous activation and a further delay in conduction.
• The predominant site of RV involvement is often the “triangle
of dysplasia,” an area involving the RV outflow tract, an area
below the tricuspid valve, and the RV apex; this is the most
common area of RV thinning, regional dilation, and aneurysm
formation.
• This anatomic area and its abnormalities in
ACM are responsible for the typical ECG
appearance during sinus rhythm
 Diagnosis
• The more advanced the disease, the easier the diagnosis,
• with increasing use of cardiac MRI for the diagnosis
of cardiac pathology, a trend toward overdiagnosis of
ACM
• normal variant of fatty infiltration of the right
ventricle is misinterpreted as ACM

• true ACM, a combination of fatty infiltration and fibrous

replacement is present rather than isolated fatty
infiltration.
• The diagnosis of ACM currently rests on the
combination of clinical, electrocardiographic,
and genetic findings, which are divided into
“major” and “minor” diagnostic criteria as
proposed in a 2010 revision of the original
1994 diagnostic criteria .
• Cardiac Biopsy
• Endomyocardial biopsy for ACM is one of the
diagnostic criteria but should be undertaken
with great caution
 Treatment
• Currently, the mainstay of therapy for ACM is suppression and
prevention of ventricular arrhythmias and the risk for sudden
cardiac death.
• Antiarrhythmic drugs are often unsuccessful in completely
abolishing the arrhythmias.
• The classic monomorphic VT in ACM with predominant RV
involvement is generally well tolerated, even at a rapid rate,
possibly because of preserved LV function in most patients.
• Nevertheless, VT of a different morphology may occur and sudden
death is not uncommon.
• Beta blockers have not been shown to be of value in treating the
usual VT of ACM.
• Accordingly, an implantable defibrillator is
recommended in patients with aborted sudden
death, syncope, or decreased LV function.
• In well-tolerated monomorphic VT, catheter
ablation of the VT is an option.
• Catheter ablation is associated with greater
than an 85% arrhythmia-free survival rate at 3
years, with VT recurrence being well tolerated in
most of the remaining patients.
• Based on the theory that mechanical stress is
a trigger for disease progression,
• pharmacotherapeutic load reduction slows
progression of the disease,
• the use of ACE inhibitors and/or nitrates has
been suggested,
• although no clinical trial evidence is
available to support such treatment
 Peripartum Cardiomyopathy

• Peripartum cardiomyopathy (PPCM) is a DCM that occurs in

a temporal relationship to pregnancy
• No definition has been universally accepted, but the
European Society of Cardiology Working Group, stressing
that PPCM is a diagnosis of exclusion, has defined it as
• “an idiopathic cardiomyopathy presenting with heart
failure secondary to LV systolic dysfunction towards the
end of pregnancy or in the months following delivery when
no other cause of heart failure is found. The LV may not be
dilated but the ejection fraction is nearly always below
45%.”
Causes of New or Exacerbated Heart Failure
in the Peripartum Period
• The incidence and clinical features of PPCM may
differ among geographic regions, with the U.S.
incidence estimated to be between 1 in 1150 and 1 in
3200 live births as compared with 1 in 1000 in South
Africa and 1 in 300 in Haiti.
• In the United States, PPCM is disproportionately
found in black patients.
• Although this might represent a genetic
predisposition to the disorder,
• .
• it may reflect the known risk factors for the
development of PPCM, which may be higher
in the black population (preexisting
hypertension, hypertension of pregnancy, and
preeclampsia).
• Older age and multiple fetal pregnancies appear
to be risk factors, and other factors suggested
include selenium deficiency, viral myocarditis,
abnormal immune responses, and malnutrition
 Clinical Features

• In patients with PPCM, symptoms and signs of heart failure develop

during pregnancy or after delivery, similar to those of any patient
with heart failure caused by LV systolic dysfunction.
• The disorder generally progresses more rapidly, but recovery is
also more likely to occur.
• Most diagnoses are made in the 4 months postpartum, with
prepartum diagnosis most commonly made in the last month.
• However, the disorder has also been described in early pregnancy
(pregnancy-associated cardiomyopathy).
• Because symptoms similar to those of heart failure (dyspnea,
fatigue, and edema) may occur in normal pregnancy, it is possible
that a proportion of cases have a delayed diagnosis.
• Given the rarity of the disease, it is not possible to
precisely determine the incidence of PPCM in
subsequent pregnancies of patients who have
had a previous episode.
• However, recurrence appears to be related to the
degree of recovery from the initial episode, with it
being less likely to occur in women who enter
second pregnancy with a normal ejection fraction
than in those with a persistent reduction in the
ejection fraction
• With standard medical therapy, the LV ejection fraction
returns to normal in approximately 50% of patients with
PPCM, although they may still be at risk for recurrent PPCM.
• The remainder often stabilize with medical therapy;
however, a small proportion of patients may experience
progressive heart failure.
• Following delivery, treatment of PPCM is the same as for
other causes of systolic dysfunction.
• However, if heart failure occurs during pregnancy, ACE
inhibitors/ARBs are contraindicated because of the risk
for fetotoxic effects.
• Diuretics should be used with caution and
metoprolol should be used rather than
carvedilol.
• Eplerenone should be avoided, but
spironolactone can be used cautiously later in
pregnancy.
• Heart transplantation has been performed in
patients with severe PPCM.
• In the United States, approximately 5% of all
women undergoing cardiac transplantation have
PPCM as their primary indication;
• It represents the fourth most common cause in
women.
• Post-transplant outcomes of PPCM are similar to
those for other indications.
 Takotsubo Cardiomyopathy or
stress-induced cardiomyopathy
• Acute, reversible condition first recognized in the
1990s.
• The clinical features are variable;
– most common manifestation is acute-onset regional LV
dysfunction, frequently associated with chest pain,
sometimes with heart failure, and often with ST-segment
changes that may mimic acute myocardial infarction.
• it is most common in postmenopausal women, and
there is frequently a preceding emotional or physical
event that is believed to act as a trigger.
• It has been estimated that approximately 1.2% of
patients with troponin-positive, suspected acute
coronary syndrome have takotsubo cardiomyopathy,
• with a presumptive trigger being identifiable in
most cases,
• this condition accounts for more than 6500 annual
admissions in the United States,
• 90% of which involve women and most cases being
reported between the ages of 66 and 80 years
• LV contractile abnormalities are prominent, and although most
commonly involving the LV apex ,regional wall motion
abnormalities may be limited to the midventricular or other LV
walls.
• The wall motion abnormalities are characterized by their lack of
a single coronary artery distribution, and coronary angiography
reveals no evidence of acute obstructive coronary disease.
• Compensatory hyperdynamic contraction of the basal LV segments
with associated apical LV dyskinesis may result in acute LV
outflow tract obstruction because of systolic anterior motion of
the mitral valve with an associated outflow tract gradient and
hypotension.
• Although the long-term prognosis is good, an
in-hospital mortality of 1.2% has been
reported to be due to the rare complications
of irreversible cardiogenic shock, LV rupture,
or embolization of LV thrombi.
• Malignant ventricular arrhythmia, particularly
torsades de pointes associated with takotsubo-
related QT prolongation, may occur, as
(rarely) may complete heart block
• The mechanism of myocardial dysfunction in stress-
induced cardiomyopathy has not been fully elucidated,
but a leading hypothesis suggests that a
catecholamine
– surge results in regional microvascular dysfunction in
susceptible patients, accompanied by cellular calcium overload.

• Recurrence of takotsubo cardiomyopathy is uncommon

and estimated to occur in between 2% and 5% of cases,
 Therapy
• Takotsubo cardiomyopathy is a self-limited disorder with rapid
resolution of the symptoms and LV dysfunction.
• Because of the occasional association with acute QT prolongation,
care should be taken to avoid using QT-prolonging medications, such as
macrolide antibiotics or certain antiarrhythmic agents.
• In patients with hypotension associated with takotsubo
cardiomyopathy, pressors should be used with caution because LV
outflow tract obstruction may be precipitated.
• Occasionally, thrombus formation may occur in the dyskinetic segment.
• Even though this is an obvious indication for anticoagulation, routine
anticoagulation is not recommended despite dyskinesis because of the
rapid resolution of the condition
 RESTRICTIVE AND INFILTRATIVE
CARDIOMYOPATHIES
• The RCMs are a heterogenous group of diseases characterized by a
nondilated left ventricle, often with a well-preserved ejection
fraction.
• The predominant manifestation is diastolic dysfunction as a result
of myocardial disease,
• Some infiltrative cardiac diseases such as amyloidosis produce an
RCM, whereas others, such as sarcoidosis, have an infiltrative
component but are predominantly manifested as DCM.
• Thus just as DCM is a morphologic definition that encompasses
several causes of cardiomyopathy,
– the terms restrictiveand infiltrative cardiomyopathy are pathophysiologic and
anatomic definitions of a cardiomyopathy that has overlaps in several well-
defined conditions.
Approach to Identifying a Cause of Restrictive Cardiomyopathy

• Because RCM is not always an isolated cardiac

disease but may arise secondary to other
acquired or genetic diseases, the diagnostic
approach is challenging .
• Endomyocardial biopsy may be much more relevant for the
diagnosis of a specific cause in patients with RCM than in those
with DCM or HCM insofar as RCM may be caused by an
infiltrative cardiac process without systemic involvement or
with subclinical involvement of other organs.
• When a cause cannot be identified, the condition is known as
idiopathic RCM.
• Unlike DCM, familial RCM is distinctly uncommon.
• Regardless of whether a cause can be found, a comprehensive
family history should always be obtained and clinical
screening of first-degree relatives should be strongly considered.
 Clinical Features of Idiopathic
Restrictive Cardiomyopathy
• Idiopathic RCM has been described in individuals from infancy to
late adulthood
• poor prognosis, especially in children.
• The disease is rare, and the largest adult series contains only 91 cases
seen over a 17-year period.
• Symptoms of idiopathic RCM are nonspecific and reflect the presence of
heart failure, with a median age at diagnosis of approximately 68
years.
• Physical examination is usually consistent with biventricular heart
failure, with jugular venous distention noted in most patients but ascites
and significant edema being found in advanced cases.
• Atrial fibrillation is common, murmurs are not a feature, and a third
heart sound is heard in one in four patients.
• The ECG has normal
voltage with only a minority
of patients showing
intraventricular conduction
delay.
• Echocardiographyreveals a
typical pattern of biatrial
enlargement and
nondilated ventricles with a
normal LV ejection fraction
and LV wall thickness .
• At cardiac catheterization, both RV and LV filling pressure is
elevated.
• Endomyocardial biopsy demonstrates nonspecific findings such
as myocyte hypertrophy, interstitial fibrosis, and not
uncommonly, endocardial fibrosis.
• Survival is reduced in comparison to an age- and sex-
matched population, with 5- and 10-year observed survival
rates from the time of diagnosis of 64% and 37%, respectively.
• Most deaths are associated with cardiac causes, either
suddenly or secondary to heart failure, although a third die of
noncardiac causes related to progressive age.
• The differential diagnosis of idiopathic RCM includes:
– infiltrative cardiomyopathies, such as amyloidosis, or
– constrictive pericarditis.
• Constrictive pericarditis is more difficult to differentiate
from RCM because most of the clinical features overlap
between the two disorders.
– A thickened pericardium noted on echocardiography, CT, or
cardiac MRI in a patient with heart failure and a preserved
ejection fraction without wall thickening suggests constrictive
pericarditis; however, it bears emphasis that 18% of patients
with constrictive pericarditis have normal pericardial thickness.
• endomyocardial biopsy may be required
unless an alternative diagnosis is clear.
• Treatment of idiopathic RCM is generally
limited to medical treatment of heart
failure, but in selected advanced cases,
• cardiac transplantation has been performed
with similar outcomes as in those with
nonrestrictive cardiomyopathy
 Hypertrophic Cardiomyopathy
• most common of the genetic cardiovascular
diseases,
• caused by a multitude of mutations in
genes encoding proteins of the cardiac
sarcomere.
• characterized by heterogeneous clinical
expression, unique pathophysiology, and
diverse natural history.
• HCM is characterized by a thickened but
nondilated left ventricle in the absence of
another cardiac or systemic condition (e.g.,
aortic valve stenosis, systemic hypertension,
some expressions of physiologic “athlete’s
heart”) capable of producing the magnitude
of left ventricular (LV) hypertrophy evident in
this disorder
• .
GENETIC BASIS AND LABORATORY TESTING

• HCM is transmitted as a mendelian trait with an

autosomal dominant pattern of inheritance;
– every offspring of an affected relative has a 50%
chance of developing the disease.
• HCM is now known to be caused by mutations
in 11 or more genes
– encoding proteins of the thick and thin contractile
myofilament components of the cardiac sarcomere
or the adjacent Z-disc
MORPHOLOGY AND ROLE OF CARDIAC
IMAGING
• Hypertrophic Cardiomyopathy Phenotype/ Left 
Ventricular Hypertrophy
• The clinical diagnosis of HCM is conventionally made
by imaging with two-dimensional echocardiography.

• CMR allows for the quantification of late gadolinium

enhancement,
– which is a marker of myocardial fibrosis potentially relevant
to risk stratification in HCM, as well as identification of
disease progression to the end-stage phase.
 CLINICAL FEATURES
• Symptoms
• Symptoms of heart failure (with preserved LV systolic
function) may
• develop unpredictably at any age,
– exertional dyspnea or fatigue, and
– in advanced stages, accompanied by orthopnea or paroxysmal
nocturnal dyspnea.
– chest pain (in the absence of atherosclerotic coronary artery
disease).
– syncope or near-syncope
– Palpitations
• Physical Examination
• In patients with LV outflow obstruction, a medium-pitch systolic ejection murmur is
characteristically heard at the lower left sternal border and apex that varies in intensity
with the magnitude of the subaortic gradient, increasing with Valsalva maneuver, during
or immediately after exercise, or on standing.
• Such variability, together with the characteristic lack of radiation of the murmur to the
neck, aids in differentiating dynamic subaortic obstruction from fixed aortic stenosis.
• Most patients with HCM and loud murmurs of at least grade 3/6 are likely to have LV
outflow gradients greater than 30 mm Hg; also, arterial pulses may rise rapidly with
bisferiens contour.
• Initial clinical suspicion for HCM may be triggered by recognition of a heart murmur on
• routine examination or before sports participation, although most
• HCM patients are identified by virtue of symptom onset or cardiac
• events. Physical findings in patients without subaortic gradients are
• more subtle, with no or a soft systolic murmur, although a forceful
• outward systolic thrust at the apex may arouse suspicion of HCM
• Electrocardiographic Findings
• The 12-lead ECG is abnormal in
– > 90% of probands with HCM
– approximately 75% of asymptomatic relatives.
– LV hypertrophy,
– ST-T changes (including marked T wave inversion in the lateral
precordial leads),
– left atrial enlargement,
– deep and narrow Q waves, and
– diminished R waves in the lateral precordial leads.
• Normal ECG patterns are most commonly associated with less
severe phenotypes
• higher risk patients may proceed along
specific adverse pathways:
• (1) sudden and unexpected death;
• (2) progressive heart failure and
• (3) atrial fibrillation, with the risk for embolic
stroke and heart failure.
• The principal determinants of progressive heart
failure and heart failure–related death in HCM are:
– LV outflow obstruction,
– atrial fibrillation,
• diastolic dysfunction, However, in contrast with
sudden death risk
• (which is related to particularly marked LV
hypertrophy), greater LV wall thickness is not
associated with an increased likelihood of progressive
heart failure symptoms
 Epidemiology of Sudden Death and Risk 
Stratification Strategies

• Sudden death in HCM may occur at a wide range of

ages, most commonly in adolescents and young
adults before the age of 30 to 35 years.
• Sudden death risk extends into mid-life, but at a
lower rate, and is significantly less common in
patients 60 years of age or older,
• Most sudden deaths occur while the victim is
sedentary or engaged in only modest physical
activity, such events also may be associated with
vigorous exertion,
• For primary prevention,risk markers include one or more of the
following, which assume greater weight in younger patients (<50
years of age)
– (1) family history of one or more premature HCM-related deaths,
– (2) unexplained syncope,
– (3) hypotensive or attenuated blood pressure response to exercise;
– (4) multiple, repetitive (or prolonged) nonsustained bursts of
ventricular tachycardia on serial ambulatory ECGs; (
– 5) massive LV hypertrophy (wall thickness, ≥30 mm) (Fig. 66-2C,G)
• The presence of one or more major risk factors justifies
consideration for a primary prevention implantable
cardioverterdefibrillator (ICD) .
Medical Treatment of Heart Failure
• Limiting symptoms of heart failure (i.e., exertional
dyspnea) are attributable to diastolic dysfunction, outflow
tract obstruction, microvascular ischemia, or any
combination of these pathophysiologic variables.
• Betaadrenergic receptor blocking drugs have been
used extensively to relieve symptoms of heart failure
in obstructive or nonobstructive HCM by:
– slowing heart rate and
– reducing the force of LV contraction,
– So, augmenting ventricular filling and decreasing myocardial
oxygen consumption.
• Verapamil has the potential to improve symptoms
and exercise capacity, largely in patients without
marked obstruction to LV outflow,
• In patients with outflow obstruction, disopyramide
may be a third option (in combination with a beta
blocker) to ameliorate symptoms.

• Diuretic agents- to reduce pulmonary congestion and

LV filling pressures; but with caution .
– Dehydration worsens obstruction and should be avoided.
• Therapeutic strategies for patients with
systolic dysfunction in the end stage
– similar to those used for congestive heart failure
in other cardiac diseases,
• Atrial Fibrillation
• Atrial fibrillation is the most common sustained arrhythmia in HCM68-73
• Atrial fibrillation, either paroxysmal or chronic, occurs in about 25% of patients
with HCM, increasing in incidence with age and magnitude of left atrial
enlargement and dysfunction.
• Because of potential for clot formation and embolization, initiation of
anticoagulant therapy is prudent in patients with atrial fibrillation.
• Such decisions are tailored to individual patients after consideration of lifestyle
modifications, hemorrhagic risk, and expectations for compliance.
• The CHADS risk score is not validated in HCM,
• Although data specifically in HCM are limited, amiodarone is regarded as
the most effective drug in reducing recurrence of atrial fibrillation.
• Beta blockers and verapamil usually are administered to control heart rate in
patients with persistent atrial fibrillation.
• Thank you