+

CARDIOMYOPATHY
AND
MYOCARDITIS
HARRISON’S INTERNAL MEDICINE
20TH EDITION
DR GRACE CASTILLO
+
DEFINITION AND
CLASSIFICATION
 Cardiomyopathy is disorders characterized by
morphologically and functionally abnormal myocardium in
the absence of any other disease that is sufficient, by itself,
to cause the observed phenotype.

 It was further specified that many cardiomyopathies will be

attributable to genetic disease.

 Ischemic cardiomyopathy is diffuse dysfunction attributed

to multivessel CAD

 nonischemic cardiomyopathy to cardiomyopathy from

other causes.
+
GENERAL PRESENTATION

 exertional intolerance with breathlessness or fatigue,

usually from inadequate cardiac reserve during exercise.

 unnoticed or attributed to other causes, commonly lung

disease or “getting older.”

 Shortness of breath may occur during routine daily activity

such as dressing and may manifest as dyspnea or cough
when lying down at night.

 peripheral edema may be absent despite severe fluid

retention, particularly in younger patients in whom ascites
and abdominal discomfort may dominate.
+
 Thenonspecific term congestive heart failure
describes only the resulting syndrome of fluid
retention, which is common to all three types of
cardiomyopathy and also to cardiac structural
diseases associated with elevated filling
pressures.

 All3 types of cardiomyopathy can be associated:

 atrioventricular (AV) valve regurgitation
 typical and atypical chest pain
 atrial and ventricular tachyarrhythmias
 embolic events
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DILATED CARDIOMYOPATHY
 An enlarged left ventricle with reduced systolic function as
measured by left ventricular ejection fraction

 Systolic failure is more prominent > diastolic dysfunction.

 Mitral regurgitation commonly develops

 Dilation and decreased function of the right ventricle may

result directly from the initial injury, but more often develops
later in response to elevated afterload presented by
secondary pulmonary hypertension and in relation to
mechanical interactions with the failing left ventricle.

 left bundle branch block precedes clinical heart failure by

many years,

 cardiac resynchronization pacing may be particularly likely

to improve ejection fraction and decrease ventricular size
+
MYOCARDITIS
 Myocarditis (inflammation of the heart) can result
from multiple causes
 direct invasion
 production of cardiotoxic substances
 chronic inflammation with or without persistent
infection.

 Myocarditis cannot be assumed from a presentation

of decreased systolic function in the setting of an
acute infection, as any severe infection can depress
cardiac function transiently.

 Infectious myocarditis -most commonly associated

with viruses and the protozoan Trypanosoma cruzi.
+
INFECTIVE MYOCARDITIS
 Murine models- After viruses gain entry through the
respiratory or gastrointestinal tract, they can infect organs
possessing specific receptors, such as the coxsackie-
adenovirus receptor on the heart.

 Viral infection and replication can cause myocardial injury

and lysis.

 Activation of viral receptor proteins can also activate host

tyrosine kinases, which modify the cytoskeleton to facilitate
further viral entry.
+
 Cytokine release is rapid, followed by triggered
activation and expansion of specific T- and B-cell
populations. This initial response can increase
viral replication and worsen cardiac injury.
 Thesecondary acquired immune response is
specifically addressed against the viral proteins
and can include both T-cell infiltration and
antibodies to viral proteins. If unchecked, can
perpetuate secondary cardiac damage.
 Ongoing cytokine release activates matrix
metalloproteinases that can disrupt the collagen
and elastin scaffolding of the heart, potentiating
ventricular dilation.
+ Clinical Presentation of Viral Myocarditis
symptoms and signs of heart failure

 chest pain suggestive of pericarditis or acute MI

 atrial
or ventricular tachyarrhythmias, or by
pulmonary or systemic emboli from intracardiac
thrombi.

 Electrocardiographic or echocardiographic
abnormalities

 Typical patient with presumed viral myocarditis

 is a young to middle-aged adult who develops
progressive dyspnea and weakness within a few
days to weeks after a viral syndrome, accompanied
by fever and myalgias.
+

 fulminant myocarditis- rapid progression within

hours from a severe febrile respiratory syndrome to
cardiogenic shock that may involve multiple organ
systems, leading to renal failure, hepatic failure, and
coagulopathy.
 Ttypicallyyoung adults who have recently been
dismissed from urgent care settings with antibiotics
for bronchitis or oseltamivir for viral syndromes, only
to return within a few days in rapidly progressive
cardiogenic shock.
 Prompt triage: high-dose intravenous catecholamine
therapy and sometimes with temporary mechanical
circulatory support.
+

 Chronic viral myocarditis is often invoked, but

rarely proven, as a diagnosis when no other
cause of DCM can be identified.

 However, many cases assumed to result from

“silent” myocarditis will later be recognized as
due to genetic causes or consumption of excess
alcohol or illicit stimulant drugs.
+ Laboratory Evaluation for Myocarditis

 ECG

 echocardiogram

 serum levels of troponin

 creatine phosphokinase fractions

 Magnetic resonance imaging

+
 Endomyocardial biopsy is not often indicated for the initial
evaluation of suspected viral myocarditis unless ventricular
tachyarrhythmias suggest possible etiologies of sarcoidosis
or giant cell myocarditis.

 .When biopsy is performed, the Dallas Criteria for

myocarditis include lymphocytic infiltrate with evidence of
myocyte necrosis and are negative in 80–90% of patients with
clinical myocarditis.

 Negative Dallas Criteria can reflect sampling error or early

resolution of lymphocytic infiltrates, but also the insensitivity
of the test when inflammation results from cytokines and
antibody-mediated injury.
+
 Patientswith recent or ongoing viral syndromes
have been classified into three levels of
myocarditis diagnosis.
 (1) Possible subclinical acute myocarditis
 (2) Probable acute myocarditis
 (3) Definite myocarditis

 Clinical
findings of pericarditis are accompanied
by elevated troponin or CK-MB or abnormal
cardiac wall motion, the terms perimyocarditis
or myopericarditis
+ SPECIFIC VIRUSES IMPLICATED IN
MYOCARDITIS

 RNA viruses
 enteroviruses, coxsackie virus, echovirus, and poliovirus,
Influenza.

 DNA viruses
 adenovirus, vaccinia (smallpox vaccine), and the herpesviruses
(varicella zoster, cytomegalovirus, Epstein-Barr virus, and human
herpesvirus 6 [HHV6])

 Polymerase chain reaction (PCR) detects viral genomes in

the majority of patients with DCM, but also in normal
“control” hearts. Most often detected are parvovirus B19 and
HHV6, which may affect the cardiovascular system
+
 Human immunodeficiency virus (HIV)

 Antiviral drugs to treat chronic HIV can cause

cardiomyopathy, both directly and through drug
hypersensitivity..

 Hepatitis C

 Additional viruses implicated specifically in myocarditis

include mumps, respiratory syncytial virus, the arboviruses
(dengue fever and yellow fever), and arenaviruses (Lassa
fever).
+
THERAPY

 acuteinfection, therapy with anti-inflammatory

or immunosuppressive medications is avoided,
as their use has been shown to increase viral
replication and myocardial injury in animal
models.
 antiviral
agents (such as oseltamivir) has not
been studied in relation to cardiac involvement
 However, neither antiviral nor anti-inflammatory
therapies are currently recommended.
+
Parasitic Myocarditis

 Chagas’ disease

 Features typical of Chagas

 conduction system abnormalities (sinus node
and AV node dysfunction and right bundle
branch block).
 Atrial fibrillation and ventricular
tachyarrhythmias also occur.
 Small ventricular aneurysms are common,
particularly at the ventricular apex.
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 The acute phase of Chagas’ disease with
parasitemia is usually unrecognized, but in fewer
than 5% of cases, it presents clinically within a
few weeks of infection, with nonspecific
symptoms or occasionally with acute myocarditis
and meningoencephalitis.

 In the absence of antiparasitic therapy, the silent

stage progresses slowly for >10–30 years in
almost half of patients to manifest chronically in
the cardiac and gastrointestinal systems.
+
 African trypanosomiasis ,Trypanosoma brucei gambiense and
T. brucei rhodesiense can progress rapidly through
perivascular infiltration to myocarditis and heart failure, with
frequent arrhythmias.

 Toxoplasmosis- may present with encephalitis or

chorioretinitis and, in the heart, can cause myocarditis,
pericardial effusion, constrictive pericarditis, and heart
failure.

 Trichinellosis - larva may occasionally invade the

myocardium, clinical heart failure is rare
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 Clostridial infections cause myocardial damage from the
released toxin. Abscesses can form in the myocardium and
pericardium.

 Streptococcal infection with β-hemolytic streptococci is most

commonly associated with acute rheumatic fever and is
characterized by inflammation and fibrosis of cardiac valves
and systemic connective tissue, but it can also lead to a
myocarditis

 Tuberculosis can involve the myocardium directly as well as

through tuberculous pericarditis, but rarely does so when the
disease is treated with antibiotics.

 Whipple’s disease is caused by Tropheryma whipplei-

pericarditis, coronary arteritis, valvular lesions, and
occasionally clinical heart failure may also occur.
+

 Other Infections Spirochetal myocarditis has been diagnosed from

myocardial biopsies containing Borrelia burgdorferi that causes Lyme
disease. Lyme carditis most often presents with arthritis and conduction
system disease that resolves within 1–2 weeks of antibiotic treatment,
only rarely implicated in chronic heart failure.

 Fungal myocarditis can occur due to hematogenous or direct spread

of infection from other sites, as has been described for aspergillosis,
actinomycosis, blastomycosis, candidiasis, coccidioidomycosis,
cryptococcosis, histoplasmosis, and mucormycosis. However, cardiac
involvement is rarely the dominant clinical feature of these infections.

 The rickettsial infections, Q fever, Rocky Mountain spotted fever, and

scrub typhus are frequently accompanied by ECG changes, but most
clinical manifestations relate to systemic vascular involvement.
+
NONINFECTIVE MYOCARDITIS

 occur without apparent preceding infection

 The most commonly diagnosed noninfective inflammation is

granulomatous myocarditis, including both sarcoidosis and
giant cell myocarditis.

 Patients with pulmonary sarcoid are at high risk for cardiac

involvement, but cardiac sarcoidosis also occurs without
clinical lung disease
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 Patients may present with:

 rapid-onset heart failure

 ventricular tachyarrhythmias
 conduction block
 chest pain syndromes
 or minor cardiac findings in the setting
of ocular involvement
 an infiltrative skin rash
 nonspecific febrile illness
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 . Computed tomography of the chest often reveals pulmonary
lymphadenopathy

 Metabolic imaging (positron emission tomography [PET]) of

the whole chest can highlight active sarcoid lesions that are
avid for glucose.

 Magnetic resonance imaging (MRI) of the heart can identify

areas likely to be inflammatory.

 Biopsy of enlarged mediastinal nodes may provide the

highest yield.
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Immunosuppressive treatment for
sarcoidosis

 high-dose glucocorticoids

 Pacemakers and implantable defibrillators are

generally indicated to prevent life-threatening
heart block or ventricular tachycardia,
respectively
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Giant cell myocarditis

 is accounts for 10–20% of biopsy-positive cases of

myocarditis.
 Presents with:
 rapidly progressive heart failure
 tachyarrhythmias.
 Diffuse granulomatous lesions
 eosinophilic infiltration
 Associated conditions are thymomas, thyroiditis,
pernicious anemia, other autoimmune diseases, and
occasionally recent infections.
 Glucocorticoid therapy is less effective than for
sarcoidosis and is sometimes combined with other
immunosuppressive agents.
+
Eosinophilic myocarditis

 An important manifestation of the

hypereosinophilic syndrome, which in Western
countries is often considered idiopathic,
although in Mediterranean and African
countries, is associated with antecedent infection

 seenwith systemic eosinophilic syndromes-

such as Churg-Strauss syndrome or
malignancies.
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Hypersensitivity myocarditis
 is often an unexpected diagnosis, made when the biopsy
reveals infiltration with lymphocytes and mononuclear cells
with a high proportion of eosinophils.

 Most commonly, the reaction is attributed:

 antibiotics
 thiazides
 Anticonvulsants
 indomethacin
 Methyldopa

 High-dose glucocorticoids and discontinuation of the trigger

agent can be curative for hypersensitivity myocarditis.
+
PERIPARTUM
CARDIOMYOPATHY
 Peripartum cardiomyopathy (PPCM) develops during the last
trimester or within the first 6 months after pregnancy,

 Risk factors:
 increased maternal age
 increased parity
 twin pregnancy
 malnutrition,
 use of tocolytic therapy for premature labor
 preeclampsia or toxemia of pregnancy.
 anti-angiogenic signaling through secreted vascular endothelial
growth factor (VEGF) inhibitors, such as soluble FLT1 (sFLT1).
+
TOXIC CARDIOMYOPATHY

 Alcohol is the most common toxin implicated in chronic

DCM. Excess consumption may contribute to more than 10%
of cases of heart failure, including exacerbation of cases with
other primary etiologies such as valvular disease or previous
infarction .

 five to six drinks (about 4 ounces of pure ethanol) daily for 5–

10 years, but frequent binge drinking may also be sufficient.

 Cocaine, amphetamines, and related catecholaminergic

stimulants can produce chronic cardiomyopathy as well as
acute ischemia and tachyarrhythmias.

 Pathology reveals microinfarcts consistent with small vessel

ischemia, similar to those seen with pheochromocytoma
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 CHEMOTHERAPY agents are the most common
drugs implicated in toxic cardiomyopathy.

 Anthracyclines (e.g., doxorubicin) cause

characteristic histologic changes of vacuolar
degeneration and myofibrillar loss.

 Doxorubicincardiotoxicity generally results in

minimal ventricular dilation, perhaps due to
accompanying fibrosis.

 strokevolume may be severely reduced with an

ejection fraction of 30–40%
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 Trastuzumab (Herceptin) is crucial for some

tumor growth and for cardiac adaptation.

 Although considered to be more often

reversible, trastuzumab cardiotoxicity does not
always resolve, and some patients progress to
clinical heart failure and death.

 cyclophosphamide and ifosfamide, 5-Fluorouracil,

cisplatin, and some other alkylating agents can
cause recurrent coronary spasm that
occasionally leads to depressed contractility.
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 Many small-molecule tyrosine kinase inhibitors that affect VEGF
are under use for different malignancies are associated with a
~2.7-fold increased risk of heart failure.

 Proteasome inhibitors used to treat multiple myeloma are

associated with an increased risk of heart failure. Carfilzomib,
appears more cardiotoxic than bortezomib.

 Ipilimumab and nivolumab- multisystem autoimmune

inflammatory toxicities (e.g., thyroiditis, hypophysitis,
pancreatitis, and pneumonitis) and rarely myocarditis. However,
combination therapy with two checkpoint inhibitors can cause
fulminant myocarditis with associated systolic dysfunction,

 hydroxychloroquine, chloroquine, emetine, and antiretroviral

therapies.

 Toxic exposures can cause arrhythmias or respiratory injury

acutely during accidents. Chronic exposures implicated in
cardiotoxicity include hydrocarbons, fluorocarbons, arsenicals,
lead, and mercury.
+ METABOLIC CAUSES OF
CARDIOMYOPATHY

 Hyperthyroidism and hypothyroidism

 Pheochromocytoma is rare, but should be considered when

a patient has heart failure and very labile blood pressure and
heart rate, sometimes with episodic palpitations . often have
postural hypotension.

 Diabetes and Obesity- Most heart failure in diabetes results

from epicardial coronary disease, with further increase in
coronary artery risk due to accompanying hypertension and
renal dysfunction
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Nutritional deficiencies

 Beri-beri heart disease due to thiamine deficiency, This

disease is initially a vasodilated state with very high output
heart failure that can later progress to a low output state;

 Abnormalities in carnitine metabolism can cause dilated or

restrictive cardiomyopathies, usually in children.

 Deficiency of trace elements such as selenium can cause

cardiomyopathy (Keshan’s disease).

 Calcium is essential for excitation-contraction

 Hypophosphatemia can develop during starvation and early

refeeding following a prolonged fast, and occasionally
during hyperalimentation

 Hemochromatosis
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FAMILIAL DCM

 MUSCULAR DYSTROPHIES

 Both Duchenne’s and the milder Becker’s dystrophies result

from abnormalities in the X-linked dystrophin gene of the
sarcolemmal membrane. Skeletal myopathy is present in
multiple other genetic cardiomyopathies , some of which are
associated with creatine kinase

 A prominent family history of sudden death or ventricular

tachycardia before clinical cardiomyopathy suggests genetic
defects in the desmosomal proteins

 Originally described as affecting the right ventricle

(arrhythmogenic right ventricular cardiomyopathy [ARVC]), this
disorder (arrhythmogenic cardiomyopathy) can affect either or
both ventricles with ventricular tachycardia.
+
Left ventricular noncompaction
 is
a condition of unknown prevalence that is
increasingly revealed with the refinement of
imaging techniques

 The diagnostic criteria include the presence of

multiple trabeculations in the left ventricle distal
to the papillary muscles, creating a “spongy”
appearance of the apex
+ TAKOTSUBO CARDIOMYOPATHY

 occurs typically in older women

 after sudden intense emotional or physical stress

 The ventricle shows global ventricular dilation with basal

contraction, forming the shape of the narrow-necked jar
(takotsubo) used in Japan to trap octopuses.

 Originally described in Japan, it is increasingly recognized

elsewhere during emergency cardiac catheterization and
intensive care unit admissions for noncardiac conditions.

 Presentations include pulmonary edema, hypotension, and chest

pain with ECG changes mimicking an acute infarction.

 The left ventricular dysfunction extends beyond a specific

coronary artery distribution and generally resolves within days
to weeks.
+

 Coronary angiography may be required to rule

out acute coronary occlusion.
 No therapies have been proven beneficial, but
reasonable strategies include nitrates for
pulmonary edema, intraaortic balloon pump if
needed for low output, combined alpha and beta
blockers rather than selective beta blockade if
hemodynamically stable, and magnesium for
arrhythmias related to QT prolongation.
 Anticoagulation is generally withheld due to the
occasional occurrence of ventricular rupture.
+
IDIOPATHIC DCM
 IS A DIAGNOSE OF EXCLUSION, WHEN ALL OTHER KNOWN
FACTORS HAVE BEEN EXCLUDED.

 2/3 OF DILATED CARDIOMYOPATHIES ARE STILL LABELED

AS IIDIOPATHIC
+
OVERLAPPING TYPES OF
CARDIOMYOPATHY

 Cardiomyopathy with reduced systolic function

but without severe dilation can represent early
DCM, “minimally dilated cardiomyopathy,” or
restrictive diseases without marked increases in
ventricular wall thickness.

 For example, sarcoidosis and hemochromatosis

can present as dilated or restrictive disease
+
RESTRICTIVE
CARDIOMYOPATHY
 dominated by abnormal diastolic function,

 often with mildly decreased contractility and ejection fraction

(usually >30–50%).

 often present with relatively more right-sided symptoms, such as

edema, abdominal discomfort, and ascites, although filling
pressures are elevated in both ventricles

 A fourth heart sound is more common than a third heart sound in

sinus rhythm, but atrial fibrillation is common

 Jugular venous pressures often show rapid Y descents & may

increase during inspiration (positive Kussmaul’s sign).
+
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INFILTRATIVE DISEASE
 Amyloidosis is the major cause of restrictive cardiomyopathy

 Cardiac amyloid is classically suspected from thickened

ventricular walls with an ECG that shows low voltage.

 Therapy for all types of amyloid is predominantly for symptoms of

fluid retention, which often requires high doses of loop diuretics.

 Digoxin bound to the amyloid fibrils can reach toxic levels, and
should therefore be used only in very low doses, if at all.

 The risk of intracardiac thrombi may warrant chronic

anticoagulation.

 The prognosis is worst for primary amyloid, with a median survival

of 6–12 months after presentation, but that has improved
substantially with the use of the proteasome inhibitor bortezomib.
+
FIBROTIC RESTRICTIVE
CARDIOMYOPATHY

 Progressive fibrosis can cause restrictive myocardial disease

without ventricular dilation.

 Thoracic radiation, common for breast and lung cancer or

mediastinal lymphoma, can produce early or late restrictive
cardiomyopathy.

 Patients with radiation cardiomyopathy may present with a

possible diagnosis of constrictive pericarditis, as the two
conditions often coexist.

 Scleroderma causes small vessel spasm and ischemia that can

lead to a small, stiff heart with reduced ejection fraction without
dilation.
+
ENDOMYOCARDIAL DISEASE

 The physiologic picture of elevated filling pressures with atrial

enlargement and preserved ventricular contractility with normal
or reduced ventricular volumes can result from extensive
fibrosis of the endocardium, without transmural myocardial
disease.

 For patients who have not lived in the equatorial regions, this
picture is rare, and when seen is often associated with a history
of chronic hypereosinophilic syndrome (Löffler’s endocarditis?),
which is more common in men than women

 persistent hypereosinophilia of >1500 eos/μL for at least 6

months can cause an acute phase of eosinophilic injury in the
endocardium with systemic illness and injury to other organs.
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 In tropical countries, up to one-quarter of heart failure may
be due to endomyocardial fibrosis, affecting either or both
ventricles.

 Medical treatment focuses on glucocorticoids and

chemotherapy to suppress hypereosinophilia when present.

 Fluid retention may become increasingly resistant to diuretic

therapy.

 Anticoagulation is recommended.

 Atrial fibrillation is associated with worse symptoms and

prognosis, but may be difficult to suppress.

 Surgical resection of the apices and replacement of the

fibrotic valves can improve symptoms, but surgical morbidity
and mortality and later recurrence rates are high.
+
HYPERTROPHIC
CARDIOMYOPATHY

 defined as left ventricular hypertrophy that develops in the

absence of causative hemodynamic factors:
 such as hypertension, aortic valve disease, or systemic infiltrative
or storage diseases.

 It has previously been termed hypertrophic obstructive

cardiomyopathy (HOCM), asymmetric septal hypertrophy
(ASH), and idiopathic hypertrophic subaortic stenosis (IHSS).
However, the accepted terminology is now hypertrophic
cardiomyopathy with or without obstruction.
+

 Hypertrophic cardiomyopathy is characterized by age-

dependent and incomplete penetrance. The defining phenotype
of left ventricular hypertrophy is rarely present at birth and
usually develops later in life.

 In MYBPC3 mutation carriers, the average age of disease

development is 40 years, while 30% remain free from
hypertrophy after 70 years.
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 At the level of the sarcomere, hypertrophic cardiomyopathy
mutations lead to enhnced calcium sensitivity, maximal force
generation, and ATPase activity.

 Calcium handling is affected through modification of regulatory

proteins.

 Sarcomere mutations lead to abnormal energetics and

impaired relaxation, both directly and as a result of hypertrophy.

 Hypertrophic cardiomyopathy is characterized by misalignment

and disarray of the enlarged myofibrils and myocytes

 Hypertrophy is the defining feature of hypertrophic

cardiomyopathy, fibrosis and microvascular disease are also
present
+

 Increasethickness and decreased luminal area of

the intramural vessels in hypertrophied
myocardium contribute to microvascular ischemia
and angina.

 Microinfarction
of hypertrophied myocardium is a
hypothesized mechanism for replacement scar
formation.

 Macroscopically,
hypertrophy is typically manifest
as nonuniform ventricular thickening
+
+
TREATMENT

 Management focuses on treatment of symptoms and

prevention of sudden death and stroke

 Left ventricular outflow tract obstruction can be controlled

medically in the majority of patients.

 β-Adrenergic blocking agents and L-type calcium channel

blockers (e.g., verapamil) are first-line agents that reduce the
severity of obstruction by slowing heart rate, enhancing
diastolic filling, and decreasing contractility.

 Persistent symptoms of exertional dyspnea or chest pain can

sometimes be controlled with the addition of disopyramide,
an antiarrhythmic agent with potent negative inotropic
properties.
+

 Patients with or without obstruction may develop heart failure

symptoms due to fluid retention and require diuretic therapies
for venous congestion

 surgical myectomy effectively relieves outflow tract obstruction

by excising part of the septal myocardium involved in the
dynamic obstruction.

 Alcohol septal ablation in patients with suitable coronary

anatomy can relieve outflow tract obstruction via a controlled
infarction of the proximal septum, which produces similar
periprocedural outcomes and gradient reduction as surgical
myomectomy.
+

 Patients with hypertrophic cardiomyopathy have an

increased risk of sudden cardiac death from ventricular
tachyarrhythmias.

 Vigorous physical activity and competitive sport are

prohibited

 an implantable cardioverter-defibrillator is advised for

patients with two or more risk factors and is advised on a
selected basis for patient with one risk factor.
+
+

 Atrial fibrillation is common in patients with hypertrophic

cardiomyopathy and may lead to hemodynamic deterioration
and embolic stroke.

 β-Adrenergic blocking agents and L-type calcium channel

blockers slow AV nodal conduction and improve symptoms;
cardiac glycosides should be avoided, as they may increase
contractility and worsen obstruction.

 Disopyramide and amiodarone are the preferred antiarrhythmic

agents, with radiofrequency ablation considered for medically
refractory cases.

 Anticoagulation to prevent embolic stroke in atrial fibrillation is

recommended.
+

 The sudden death risk is <1% per year; however, up to 1 in

20 patients will progress to overt systolic dysfunction with a
reduced ejection fraction with or without dilated remodeling
(“burned out” or end-stage hypertrophic cardiomyopathy).
These patients suffer from low cardiac output and have a high
risk of death from progressive heart failure and sudden
death unless they undergo cardiac transplantation.
+
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