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Title: Cardiomyopathies

  • Israel Freeman, MD
  • Associate Professor of Clinical Medicine

  • The cardiomyopathies are a diverse group of
    disease that are not related to the usual causes
    of hear disease.
  • Cardiomyopathies are classified according to
    hemodynamicc characteristics and etiology.

Cardiomyopathies WHO Classification
  • Unknown cause (primary)
  • Dilated
  • Hypertrophic
  • Restrictive
  • unclassified
  • Specific heart muscle disease (secondary)
  • Infective
  • Metabolic
  • Systemic disease
  • Heredofamilial
  • Sensitivity
  • Toxic

Br Heart J 1980 44672-673
Cardiomyopathies Functional Classification
  • Dilatated (congestive, DCM, IDC)
  • ventricular enlargement and syst dysfunction
  • Hypertrophic (IHSS, HCM, HOCM)
  • inappropriate myocardial hypertrophy in the
    absence of HTN or aortic stenosis
  • Restrictive (infiltrative)
  • abnormal filling and diastolic function

Morphologic and Hemodynamic Characteristics of
the Cardiomyopathies
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Unknown Etiology   Idiopathic
dilated cardiomyopathy   Peripartum
cardiomyopathy   Hypertrophic cardiomyopathy   En
domyocardial fibrosis   Subendocardial
fibroelastosis   Eosinophilic endomyocardial
disease (also called Löffler endocarditis or
fibroplastic endocarditis)   Right ventricular
dysplasia   Idiopathic restrictive cardiomyopathy
Secondary Cardiomyopathies
  • When a cardiomyopathy has a definite etiology
    (e.g. sarcoidosis or scleroderma) signs of that
    process are usually evident.
  • On rare occasions, cardiac involvement may
    precede other systemic manifestations.

Dilated (Congestive) Cardiomyopathies
  • Idiopathic
  • Known etiology
  • Pregnancy
  • Alcohol
  • Toxic effects of drugs
  • Tachycardia induced
  • Sleep apnea?

Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology Infectious Vir
al and rickettsial myocarditis (e.g., human
immunodeficiency virus, coxsackievirus B,
cytomegalovirus) Septic (bacterial
endocarditis) Syphilis Parasitic disease (e.g.,
Chagas disease, trichinosis, toxoplasmosis) Bac
terial toxins (e.g., diphtheria toxin) or
hypersensitivity (rheumatic fever)   
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology Toxic     Alco
hol     Cobalt     Carbon tetrachloride     Carb
on monoxide     Thioridazine drugs     Anticancer
agents (e.g. daunorubicin doxorubicin,
 cyclophosphamide)     Antimonials     Cocaine   
  Antiretroviral agents (e.g., zidovudine,
dideoxyinosine)     Interferon alfa      
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology Systemic
Diseases     Neuromuscular syndromes       muscu
lar dystrophies (e.g., progressive muscular
dystrophy,   myotonic dystrophy), Friedreich
ataxia     Collagen vascular disease     Sarcoidos
is     Endocrine diseases (e.g.,
thyrotoxicosis, myxedema,pheochromocytoma,
acromegaly)    Is potentially reversible
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology Infiltrative
   Amyloidosis     Hemochromatosis     Glycogen
storage disease     Fabry disease     Hurler
syndrome     Primary or metastatic tumors (e.g.,
lymphoma, melanoma) Usually causes a
restrictive hemodynamic picture
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology Nutritional  
   Beriberi     Selenium deficiency     Kwashior
kor    Is potentially reversible
Etiologic Classification of Cardiomyopathies
Cardiomyopathies of Known Etiology

Ischemic Is potentially
reversible !!!
Idiopathic Dilated Cardiomyopathy
Congestive (Dilated) Cardiomyopathy - Definition
  • a disease of unknown etiology that principally
    affects the myocardium
  • LV dilatation and systolic dysfunction
  • pathology
  • increased heart size and weight
  • ventricular dilatation, normal wall thickness
  • heart dysfunction out of portion to fibrosis

Secondary Congestive (Dilated) Cardiomyopathy
  • Infectious and Autoimmune processes (Myocarditis)
  • Coxsackie virus B e.t.
  • Autoimmune processes
  • Acute Myocarditis vs. Congestive Cardiomyopathy
  • Fever with CHF
  • Pericarditis
  • Elevated CPK MB , TnT ( less than 30 of

Dilated (Congestive) Cardiomyopathy
  • A familial form may be present in 10 30
  • Characterized by diminished myocardial
  • Reflected in reduction of systolic function. ( EF
    ?, LV EDP ?, SV ?, C.O ? )
  • RV dysplasia characterized by RV failure and VT

Dilated (Congestive)Cardiomyopathy Clinical
  • Highest incidence in middle age
  • blacks 2x more frequent than whites
  • men 3x more frequent than women
  • symptoms may be gradual in onset
  • acute presentation
  • misdiagnosed as viral URI in young adults
  • uncommon to find specific myocardial disease on
    endomyocardial biopsy

Dilated (Congestive) Cardiomyopathy
  • Clinical Features
  • Dyspnea and fatigue
  • Palpitation, occasionally syncope
  • Systemic and pulmonary emboli (4 / year)
  • Chest pain ( 1/3 of patients)-atypical
  • Physical Finding
  • Reflects the variable severity of CHF
  • Diagnostic Studies
  • CXR, EKG, Echocardiogram (segmental vs global
  • MRI (Dysplastic RV Cardiomyopathy)
  • Gallium 67 ( sarcoidosis, myocarditis)
  • Cardiac Catheterization


Posteroanterior CXR of a 52 y.o man with
idiopathic dilated cardiomyopathy exhibit diffuse
cardiomegaly. The LA can be seen as a faint
double density.

Lateral view of the same patient
Echocardiogram Severe Dilated Cardiomyopathy

Apical four-chamber echocardiographic view of a
patient with severe dilated cardiomyopathy. All
four cardiac chambers are markedly enlarged. The
left ventricular ejection fraction was 22, and
the left ventricular end-diastolic diameter was
74 mm.
Dilated (Congestive) Cardiomyopathy
  • Endomyocardial Biopsy
  • Low diagnostic yield
  • Uncertainty regarding role of immunosuppressive
  • Led most investigators to abandon it routine use

Endomyocardial Biopsy Myocarditis

A 19y.o. man presented with DCM and VPCs. The
findings of focal lymphocytic infiltrates,
interstitial edema, and myocyte degeneration are
typical of myocarditis. The patient responded to
Biopsy End-Stage Dilated Cardiomyopathy

An 80 y.o. man with end-stage CM. There is marked
hypertrophy of myocytes, interstitial and focal
fibrosis,and no evidence of interstitial
inflammation. Immunosuppressive therapy was not
Dilated Cardiomyopathy (DCM) Course and Prognosis
  • The most common complication of DCM is
    progressive CHF the cause of death in 50 75
  • Sudden death (SD) by arrhythmia is common
    especially in patients with complex ventricular
    ectopy and severe LV dysfunction.
  • Systemic / pulmonary embolism is found at autopsy
    in 50 of patients with DCM emboli can cause
    catastrophic complications but rarely death.
  • Prognosis varies considerably from fulminent
    cases that result in death within a few weeks, or
    conversely some patients do remarkably well for
    years. Most death within 5 years.
  • Spontaneous improvement in LV function occurs in
    20 40, most frequently within 6 months.
  • A peak 02 uptake less than 14 ml/kg /min predicts
    1 year survival of 70.

Survival in Symptomatic Idiopathic Dilated

Survival symptomatic IDC in seven reported
series. Study A, 1986-89 (basis for selection
unspecified) study B, 1975-84 (population
based) study C, 1973-87 (referral based) study
D, 1962-82 (referral based) study E, 1960-73
(referral based) study F, 1972-82 (referral
based) study G, 1965-86 (autopsy series). (N
number of patients)
Dilates Cardiomyopathy Medical Treatment
  • Limit activity based on functional status
  • salt restriction of a 2-g Na (5g NaCl) diet
  • fluid restriction for significant low Na
  • initiate medical therapy
  • ACE inhibitors, diuretics
  • digoxin, carvedilol
  • hydralazine / nitrate combination

Dilates Cardiomyopathy Medical Treatment
  • Principally standard CHF therapy
  • Anticoagulation therapy is controversial some
    arthritis recommend with marked right sided
    failure and/or BF lt 30
  • Antiarrhythmics arrhythmias are common Empirical
    suppression in symptomatic patient is
    contraindicated (CAST) due to proarrhythmic
    effect. In symptomatic patients amiodarone
    (GESICA 26 reduced mortality vs. STAT CHF)
  • Defibriallation SAEKG and EP are not reliable in
    assessing prognosis or guide antiarrhythimic
    therapy in patients with DCM. ICD is indicated
    in patients with sustained V.T. In NSVT the MADIT
    trail demonstrated an advantage for ICD those
    were IHD (inducible ) patient, MADIT doesnt
    apply. Trial results pending.

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Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathy
  • The hallmark of the disease is unexplained
    myocardial hypertrophy
  • In the obstructive form ASH, SAM. MR
  • Obstruction may be fixed or labile
  • Hyper-contractile LV (EF 80 90) with reduced
  • Reduced LV compliance (if patient in CHF
  • Factor that provoke or increase/decrease
  • Change in contractile stale
  • Change chamber size (preload and after load)

Coronal Section in Hypertrophic Cardiomyopathy

The thickening of the IVS is disproportionately
greater than that of the LV wall behind the
posterior mitral leaflet. The LV chamber is small
and elongated.
Myofibrillar Pattern in HCM vs. HTN

Different myofibrillar patterns in tissue taken
from the septum of a patient with HCM (left) and
from the hypertrophied LV of a patient with HTN
(right). Note the chaotic arrangement of the
cells of septal myocardium taken from the HCM. In
contrast, note the orderly parallel arrangement
of myofibrils in ventricular myocardium in the
HTN patient.
Morphologic and Hemodynamic Characteristics of
the Cardiomyopathies
Hypertrophic Cardiomyopath
  • Systole
  • dynamic outflow tract gradient
  • Diastole
  • impaired diastolic filling, ? filling pressure
  • Myocardial ischemia
  • ? muscle mass, filling pressure, O2 demand
  • ? vasodilator reserve, capillary density
  • abnormal intramural coronary arteries
  • systolic compression of arteries

Hypertrophic Cardiomyopath
  • Obstructive (fixed, Rabile)
  • Non Obstructive
  • Concentric
  • Mid ventricular
  • Apical

The vast majority of cases of hypertrophic
cardiomyopathy result from specific defects in
the genes regulating the formation of cardiac
muscle This defect is either sporadic or
hereditary in 50 of cases (autosomal dominant).
Survival Curves in Hypertrophic Cardiomyopathy

The surviv al of patients with hypertrophic
cardiomyopathy caused by different mutations
cardiac troponin T mutations (introns 15 G1 (r)
A, Ile79Asn, DGlu160, and Arg92Gln) is similar to
that in persons with a malignant b-myosin
heavy-chain mutation (Arg403Gln) but
significantly shorter than that observed in
persons with a benign myosin mutation
Factors That Influence the Degree of Obstruction
in Hypertrophic Cardiomyopathy

In general, anything that increases obstruction
will increase the intensity of the associated
murmurs, whereas factors that reduce
obstruction will diminish murmur intensity. May
assist in diagnosis at the bedside.
Hypertrophic Cardiomyopathic Diagnosis
  • Symptoms
  • Angina (recumbent position)
  • Syncope (after exercise, arrhythmia)
  • Palpitations (A.F, VT)
  • CHF (atrail contraction, AF with fast response)
  • Systemic embolism
  • S.D. (even in asymptomatics) lethal arrhythmia
    more likely in young
  • Physical examination
  • EKG, Echocardiography, Cardiac Cath

Changes of Murmer in Obstructive Hypertrophic
Variations in the quality of the murmur
associated with HOCM are observed before (a),
during (b), and after (c) the Valsalva maneuver.
Before the Valsalva maneuver, a soft systolic
murmur is recorded at the apex. The arterial
pulse contour is normal. During the Valsalva
maneuver, there is a dramatic increase in the
intensity of the murmur. After Valsalva release,
the murmur becomes softer, but the carotid pulse
exhibits the classic spike-and-dome configuration
characteristic of obstructive hypertrophic
Change in Gradient and Murmur
Hypertrophic Cardiomyopathic

  • Contractility Preload Afterload valsalva
    (strain) ? ? -- standing ? ?
    --postextrasystole ? ? -- isoproterenol ? ?
    ? digitalis ? ? -- amyl nitrite
    ? ? nitroglycerine ? ? ? exercise
    ? ? ? tachycardia ? ? --hypovolemia ? ? ?

Change in Gradient and Murmur
Hypertrophic Cardiomyopathic
  • Contractility
    Preload Afterload valsalva (overshoot) ? ? --
    squatting ? --- ? passive leg elevation ? ?
    __--phenylephrine ? -- ? beta-blocker
    ? -- -- general anesthesia ? -- --isometric
    grip ? -- ?

Hypertrophic Cardiomyopathy HCM vs Aortic Stenosis
  • HCM Fixed Obstruction carotid
    pulse spike and dome parvus et tardus
  • murmur radiate to carotids ? valsalva,
    standing ? squatting, handgrip ? passive
    leg elevation
  • systolic thrill 4th left interspace 2nd right
    interspace systolic click absent

ECG in Obstructive Hypertrophic Cardiomyopathy

Abnormal Q waves suggestive of an old anterior
myocardial infarct are observed in leads V3
through V6 in an ECG recorded in a 45-year-old
woman with obstructive hypertrophic
Hypertrophic Cardiomyopathy -Echocardiography

Hypertrophic Cardiomyopathy -Echocardiography

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Pressure Tracings Obstructive Hypertrophic
Classic left ventricular and radial arterial
pressure tracings are recorded simultaneously in
a patient with Obstructive HCM. An initial rapid
upstroke is observed in both recordings. As
obstruction develops, a notch (arrow) appears in
the left ventricular tracing, accompanied by a
fall in the arterial pressure. Slower ejection
follows, producing a second rise in the arterial
tracing. The events are observed slightly later
in the radial artery than in the left ventricle
because some delay occurs in the transmission of
the impulse to the artery.
HOCM-Brockenbrough Phenomenon

Simultaneous LV and radial arterial pressure
tracings in a patient with HOCM show the
Brockenbrough phenomenon in response to a VPB.
The gradient across the LVOT is about 80 mm Hg.
After the VPB, there is a compensatory pause. In
the beat that follows the compensatory pause, the
radial pulse pressure narrows, and the arterial
pressure falls despite an increase in the left
ventricular systolic pressure.
Clinical Manifestation
Hypertrophic Cardiomyopathy
  • Asymptomatic, echocardiographic finding
  • Symptomatic
  • dyspnea in 90
  • angina pectoris in 75
  • fatigue, pre-syncope, syncope ? risk of SCD in
    children and adolescents
  • palpitation, PND, CHF, dizziness less frequent

Hypertrophic Cardiomyopathy Course and Prognosis
  • Course and prognosis exceedingly variable
  • Most patient do very well
  • Some experience progression of symptoms
  • S.D.
  • More common in children and young adults (younger
    than 25 years) with the familial form of the
  • May result from arrhythmia even without
    significant LVH or LVOT obstruction
  • May result from development of LVOT obstruction
    with exercise
  • Hyperthrophy progresses, some patient with no
    obstruction will develop it later in life
    (progression is faster in the younger)

Hypertrophic Cardiomyopathy
  • Treatment of symptomatic patients
  • Beta blockers and calcium channel blockers
  • Provide relief of angina, dyspnea syncope and
    improve exercise capacity
  • Diuretics
  • Digitalis glycosides (AF?)
  • Prophylactic measures (secondary)
  • Endocarditis
  • S.D. Markers of increased risk young age
  • massive hypertrophy,m VT, NSVT, VF
  • h/o syncope and family h/o of S.D.
  • Troponin T gene mutations
  • Treatment of asymptomatic patients Primary
  • Beta Blockers
  • If strong family history of S.D amioclorone /
  • Prohibition from engagement in competitive sports

Hypertrophic Cardiomypathy Surgical Treatment
  • Cardiac Pacing
  • Septostomy
  • Chemical septal ablation


Chemical Septal Ablation in Hypertrophic
Results of chemical septal ablation in a 77-year
old woman with HOCM. Simultaneous left
ventricular and radial artery pressure recordings
(a) show a gradient of approximately 140 mm Hg
across the LVOT. Moments after injection of
alcohol into the interventricular septum (b), the
gradient is abolished. (LV-left ventricle
RAD-radial artery)
Risk Factors for SCD
Hypertrophic Cardiomyopathy
  • Young age (lt30 years)
  • Malignant family history of sudden death
  • Gene mutations prone to SCD (ex. Arg403Gln)
  • Aborted sudden cardiac death
  • Sustained VT or SVT
  • Recurrent syncope in the young
  • Nonsustained VT (Holter Monitoring)
  • Brady arrhythmias (occult conduction disease)

Br Heart J 1994 72S13
Hypertrophic Cardiomyopathy Recommendations for
Athletic Activity
  • Avoid most competitive sports (whether or not
    symptoms and/or outflow gradient are present)
  • Low-risk older patients (gt30 yrs) may participate
    in athletic activity if all of the following are

Hypertrophic Cardiomyopathy Recommendations for
Athletic Activity
  • Low-risk older patients (gt30 yrs) may participate
    in athletic activity if all of the following are
  • ventricular tachycardia on Holter monitoring
  • family history of sudden death due to HCM
  • history of syncope or episode of impaired
  • severe hemdynamic abnormalities, gradient ?50
  • exercise induced hypotension
  • moderate or sever mitral regurgitation
  • enlarged left atrium (?50 mm)
  • paroxysmal atrial fibrillation
  • abnormal myocardial perfusion

Hypertrophic Cardiomyopathy Key Points
  • Medical therapy with b-blockers is first-line
    management of hypertrophic cardiomyopathy
    verapamil is an alternative.
  • Patients with syncope, a family history of sudden
    death, or documented ventricular tachycardia are
    at highest risk of sudden death and should be
    promptly referred for evaluation by an
  • For patients with hypertrophic cardiomyopathy
    refractory to medical therapy, surgical septal
    myectomy or percutaneous ethanol septal ablation
    can be considered. Pacing therapy has limited
  • Participation in all but low-intensity
    competitive sports is contraindicated for
    patients with hypertrophic cardiomyopathy.

Other Causes of Hypertrophy
  • Clinical mimics
  • glycogen storage, infants of diabetic mothers,
  • Genetic
  • Noonans, Friedreichs ataxia, Familial
    restrictive cardiomyopathy with disarray
  • Exaggerated physiologic response
  • Afro-Caribbean hypertension, old age hypertrophy,
    athletes heart

Hypertrophic Cardiomyopathy HCM vs Athletes Heart
  • HCM Athlete
  • Unusual pattern of LVH - LV cavity lt45
    mm - - LV cavity gt55 mm LA
    enlargement - Bizarre ECG
    paterns - Abnormal LV filling - Female
    gender - - ? thickness with deconditioning
    Family history of HCM -

Circulation 1995 911596
Hypertensive HCM of the Elderly Characteristics
  • modest concentric LV hypertrophy (lt12 mm)
  • small LV cavity size
  • associated hypertension
  • ventricular morphology greatly distorted with
    reduced outflow tract
  • sigmoid septum and grandma SAM


Restrictive Cardiomyopathy
Restrictive Cardiomyopathies
  • Hallmark abnormal diastolic function
  • rigid ventricular wall with impaired ventricular
  • bear some functional resemblance to constrictive
  • importance lies in its differentiation from
    operable constrictive pericarditis

Restrictive Cardiomyopathy
  • Usually results from diseases that infiltrate the
  • Amyloidosis
  • Hemochromatosis
  • Glycogen storage diseases
  • Many cases of restrictive cardiomyopathies are

Restrictive Cardiomyopathies Ethiology
  • Idiopathic
  • Myocardial
  • 1. Noninfiltrative
  • Idiopathic
  • Scleroderma
  • 2. Infiltrative
  • Amyloid
  • Sarcoid
  • Gaucher disease
  • Hurler disease
  • 3. Storage Disease
  • Hemochromatosis
  • Fabry disease
  • Glycogen storage
  • Endomyocardial
  • endomyocardial fibrosis
  • Hyperesinophilic synd
  • Carcinoid
  • metastatic malignancies
  • radiation, anthracycline

Restrictive Cardiomyopathy
  • Etiology
  • Usually a product of infiltrative disease such
    as Aermytoidosis, Hemochromatosis Glycogen
    stroge disease.
  • Idiopathic not uncommon.
  • Pathophysiology
  • Rigid myocardium impeding ventricular LVEDP
    increased, systolic performance is often reduced
  • Diagnosis
  • CHF often predominant right sided failure
    (Kussmaul sign)
  • Differential diagnosis constrictive
  • Unequal diastolic pressures, more ventricular
    independence in constriction, pulmonary HTN more
    in restriction .

Clinical Manifestations
Restrictive Cardiomyopathies
  • Symptoms of right and left heart failure
  • Jugular Venous Pulse
  • prominent x and y descents
  • Echo-Doppler
  • abnormal mitral inflow pattern
  • prominent E wave (rapid diastolic filling)
  • reduced deceleration time (? LA pressure)

Restrictive Cardiomyopathy- Pressure Tracing  

Shown is a right atrial pressure tracing in a
patient with restrictive cardiomyopathy secondary
to amyloidosis some functional tricuspid
regurgitation is present. The cardiac rhythm is
atrial fibrillation. Large systolic (V) waves
appear, followed by a typical early diastolic
dip-and-plateau configuration.
Constrictive - Restrictive Pattern Square-Root
Sign or Dip-and-Plateau
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Constrictive Pericarditis
  • Fibrous scarring, adhesion and calcification of
    the pericardium (shell around the heart)
  • Similar to tamponade in restricting diastolic
    filling of the heart. Kussmaul sign v.s.
    paradoxical pulse.
  • Difficult diagnosis Venous congestion, fatigue,
    effusions with normal LV function, normal
    pulmonary pressure and a small cardiac silhouette
    no pulmonary congestion.
  • CT / MRI pericardial thickening
  • Echo / Doppler Flow respiratory variation
  • Treatment Conservative / Surgical

Restriction vs Constriction
  • History provide can important clues
  • Constrictive pericarditis
  • history of TB, trauma, pericarditis, sollagen
    vascular disorders
  • Restrictive cardiomyopathy
  • amyloidosis, hemochromatosis
  • Mixed
  • mediastinal radiation, cardiac surgery

Restrictive Cardiomyopathies Treatment
  • No satisfactory medical therapy
  • Drug therapy must be used with caution
  • diuretics for extremely high filling prssures
  • vasodilators may decrease filling pressure
  • ? Calcium channel blockers to improve diastolic
  • digitalis and other inotropic agents are not

Restrioctive Cardiomyopathy Key Points

Restrictive cardiomyopathy often manifests as an
insidious onset of exercise intolerance and
right-sided heart failure similar to constrictive