NOTES

NOTES
CARDIOMYOPATHY

GENERALLY, WHAT IS IT?

PATHOLOGY & CAUSES DIAGNOSIS
▪ Broad term, describes any issue resulting DIAGNOSTIC IMAGING
from disease of myocardium ▪ Chest X-ray
▪ Primary cardiomyopathy: issue develops of ▪ Echocardiogram/cardiac MRI
its own accord
▪ Secondary cardiomyopathy: issue develops OTHER DIAGNOSTICS
as compensation for another underlying
▪ ECG
disease

RISK FACTORS TREATMENT

▪ Positive family history
MEDICATIONS
COMPLICATIONS ▪ See individual diseases
▫ Heart failure, arrhythmias, sudden
cardiac death SURGERY
▪ Implantable cardioverter-defibrillator (ICD)
▪ Heart transplant
SIGNS & SYMPTOMS
▪ Can be asymptomatic OTHER INTERVENTIONS
▪ Heart failure signs, symptoms ▪ Lifestyle changes
▪ Heart murmurs

OSMOSIS.ORG 33
 DILATED CARDIOMYOPATHY
osms.it/dilated-cm
▫ Related to pregnancy-associated
PATHOLOGY & CAUSES hypertension
▫ Half of individuals recover following
▪ Dilation of all four chambers of heart pregnancy
▪ Most common type of cardiomyopathy
▫ New sarcomeres added in series, Sarcoidosis
creates larger chambers with relatively ▪ Growth of granulomas in heart → dilation
weak walls, less muscle for contraction
→ low systolic function
RISK FACTORS
▫ Chambers stretch → valves stretch →
▪ Alcoholism, past family history of diseases
blood regurgitates back into atria
implicated in DMD

CAUSES COMPLICATIONS
▪ Primary dilated cardiomyopathy most often
▪ Systolic heart failure
idiopathic
▫ Valve regurgitation: as chambers
Genetic mutations/conditions stretch, so do valves
▪ Duchenne muscular dystrophy (DMD), ▪ Arrhythmias: stretching muscle irritates
hemochromatosis conduction system

Myocarditis
▪ Can progress from myocarditis to dilated MNEMONIC: ID BIG MAPS
cardiomyopathy Causes of Dilated
Cardiomyopathy
Infection
Idiopathic
▪ Coxsackievirus B: leads to myocarditis,
Drugs/Doxorubicin (and
heart muscle inflammation
cocaine)
▪ Chagas disease: protozoal infection
Beriberi (wet)
Linked to alcoholism Infection
▪ Alcohol, metabolites have direct toxic effect Genetic
on heart muscle Myocarditis
Alcoholism
Linked to certain drugs
Peripartum cardiomyopathy
▪ Chemotherapy: doxorubicin, daunorubicin
Sarcoidosis
▪ Cocaine

Wet beriberi
▪ Beriberi: illness caused by thiamine (vitamin
B1) deficiency
▪ Wet beriberi: affects heart; ↓ thiamine
levels impair myocardium energy
production

Peripartum cardiomyopathy
▪ Can develop in third trimester of pregnancy/
weeks after delivery

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 Chapter 6 Cardiomyopathy

SIGNS & SYMPTOMS TREATMENT

▪ Fatigue, dyspnea
▪ Lateral displaced point of maximum
impulse (PMI)
▪ Chest pain on exertion
▪ Holosystolic murmur (mitral valve
regurgitation during systole)
▪ S3 sound (blood rushing into, slamming
into dilated ventricular wall during diastole)
MEDICATIONS
▪ Angiotensin-converting-enzyme (ACE)
inhibitor, angiotensin receptor blocker, beta
blocker
▫ Slows disease progression
SURGERY
▪ Heart transplant (extreme cases)

DIAGNOSIS OTHER INTERVENTIONS

▪ Left ventricular assist device (LVAD):
DIAGNOSTIC IMAGING mechanical pump assists heart in delivering
blood to body
X-ray
▪ Cardiomegaly, pulmonary edema, pleural
effusion

OTHER DIAGNOSTICS
ECG
▪ Shows ventricular dilation, reduced ejection
fraction

Figure 6.2 A chest radiograph demonstrating

enlargement of the heart due to dilated
cardiomyopathy. The heart occupies more
than half the width of the chest.
Figure 6.1 Gross pathology of dilated
cardiomyopathy. Note the large ventricles
and thin ventricular walls.

OSMOSIS.ORG 35
 HYPERTROPHIC CARDIOMYOPATHY
(HCM)
osms.it/hypertrophic-cm
COMPLICATIONS
PATHOLOGY & CAUSES ▪ Arrhythmias, sudden cardiac death
▪ Myocardium becomes thick, heavy,
hypercontractile RISK FACTORS
▪ Myocytes become disorganized, new ▪ Positive family history of HCM/conditions
sarcomeres added in parallel to existing known to be associated with HCM (e.g.
ones Friedreich’s ataxia)
▪ Left ventricle most often affected
▫ Muscle growth asymmetrical →
interventricular septum grows larger
SIGNS & SYMPTOMS
relative to free wall
▪ Many individuals asymptomatic
▪ Hypertrophy → walls taking up more
space, ↓ blood fills ventricle ▪ Auscultation: crescendo-decrescendo
murmur
▫ Walls become stiff, less compliant →
less filling → low stroke volume → ▫ ↑ intensity with ↓ venous return
dysfunction in diastolic filling of left (Valsalva, standing), ↓ in intensity with ↑
ventricle → diastolic heart failure venous return (handgrip, squatting)
▪ Arrhythmias: larger muscles require more ▪ Symptoms arise as complications arise
oxygen, coupled with heart having difficulty ▫ Dyspnea: left ventricle stiffening, atrium
delivering blood to tissues → ischemia → increasing back pressure into lungs →
arrhythmias interstitial lung congestion
▫ Fatigue
CAUSES ▫ Exertional chest pain: ischemia
▫ Syncope with exertion: brain receiving
Genetic missense mutation, inherited as low oxygen
autosomal dominant trait ▫ Palpitations: ischemia, arrhythmias
▪ Different genetic mutations affect different ▫ Sudden cardiac death
sarcomere proteins ▪ May exhibit bifid pulse: two pulses felt
▪ Friedreich’s ataxia: autosomal recessive ▫ Mitral valve moves toward outflow tract
neurodegenerative disease → ↑ obstruction mid-systole
Hypertrophic obstructive cardiomyopathy
(subtype)
▪ Interventricular septum growth blocks left
ventricular outflow tract during systole
→ blood must flow quickly through small
opening, ↓ pressure in this area ( Venturi
effect) → low pressure pulls anterior leaflet
of mitral valve toward septum → further
mitral valve obstruction towards septum →
further obstruction overall

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 Chapter 6 Cardiomyopathy

Disopyramide
DIAGNOSIS ▪ Can be used for its negative inotropic
properties
DIAGNOSTIC IMAGING
Digoxin contraindicated
Echocardiography/cardiac MRI
▪ ↑ force of contraction, can ↑ obstruction
▪ Enlarged heart chambers/↓ ejection fraction

Chest X-ray SURGERY

▪ ↑ ratio of distance between heart, thoracic ▪ Implantable cardioverter-defibrillator
cage
Surgical septal myectomy
LAB RESULTS ▪ Involves removing portion of
interventricular septum, ↓ obstruction
Genetic testing
Septal ablation
▪ Cardiomegaly-implicated gene mutations
▪ Chemical myomectomy to partially ablate
septum
OTHER DIAGNOSTICS
Heart transplant
ECG ▪ If unresponsive to all other forms of
▪ Detectable electrical changes, such as left treatment
ventricular hypertrophy
OTHER INTERVENTIONS
TREATMENT Lifestyle change
▪ Cessation of high-intensity athletics
MEDICATIONS
Beta blockers
▪ ↓ heart rate, contractile force

Calcium channel blockers

▪ If beta blockers not tolerated
▪ Slows down heart rate

Figure 6.4 The histological appearance of

the myocardium in a case of hypertrophic
cardiomyopathy. There is complete myocyte
disarray. The myocytes display bizarre forms
with side to side branching and are arranged
in a whorled configuration.

Figure 6.3 Gross pathology of hypertrophic

cardiomyopathy. The myocardium has
become so enlarged that both ventricles are
almost entirely obliterated.

OSMOSIS.ORG 37
 Figure 6.5 Illustration showing the Venturi effect: low blood pressure pulls the anterior
leaflet of the mitral valve towards the septum, creating an obstruction. Blood can’t get
through the small opening, leading to a crescendo-descrescendo heart murmur.

RESTRICTIVE CARDIOMYOPATHY
osms.it/restrictive-cm
heart tissue
PATHOLOGY & CAUSES
Endocardial fibroelastosis
▪ Cardiomyopathy: heart wall is rigid, has ▪ Fibrosis develops in endocardium (inner
difficulty stretching, pumping lining of heart) and subendocardium (layer
▪ Ventricles restrict filling, ↓ cardiac output underneath endocardium)

CAUSES
▪ Infiltrative diseases, storage diseases,
endomyocardial diseases.
Amyloidosis
▪ Amyloids are misfolded proteins →
insoluble → deposit in tissues, organs →
organs less compliant
▪ Familial amyloid cardiomyopathy
▪ Mutant transthyretin (TTR) protein;
misfolded deposits preferentially in heart
tissue
▪ Senile cardiac amyloidosis; TTR protein/
wild type TTR deposits in heart over time
Sarcoidosis
▪ Immune cell collections form granulomas in
Loffler syndrome
▪ Eosinophils accumulate in lung tissue
▪ Loeffler endocarditis/Loeffler
endomyocarditis: eosinophils also
accumulate in endocardial layer of heart
tissue → inflammation, endocardial fibrosis
→ restrict heart tissue
Hemochromatosis
▫ Iron deposits in heart tissue, contributes
to restricted tissue
Other causes
▪ Heart tissue radiation
▫ Radiation generates reactive oxygen
species → inflammation over time
→ myocardial fibrosis → tissue stiff,

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 Chapter 6 Cardiomyopathy

restrictive

COMPLICATIONS
▪ Can → diastolic heart failure
▫ Stiff ventricles → cannot stretch → less
filling → low cardiac output → heart
failure

MNEMONIC: LASHER
Causes of Restrictive
cardiomyopathy Figure 6.6 Histological appearance of the
myocardium in a case of cardiac amyloidosis;
Loffler syndrome
a cause of restrictive cardiomyopathy. Cardiac
Amyloidosis myocytes (dark purple) are surrounded by
Sarcoidosis amyloid deposits (light pink).
Hemochromatosis
Endocardial fibroelastosis
post-Radiation TREATMENT
MEDICATIONS
SIGNS & SYMPTOMS Loop diuretics
▪ ↓ systemic, pulmonary congestion
▪ Auscultation: stiff ventricle → S4 heart
sound Beta-blocker, calcium channel blocker, an-
▪ Presents as congestive heart failure: giotensin converting enzyme inhibitors
dyspnea; paroxysmal nocturnal dyspnea; ▪ Slows heart rate, ↑ ventricular-filling time
orthopnea; crackles; intraalveolar
hemorrhage; fatigue; inability to exercise;
appetite loss; abdomen swelling; swelling SURGERY
of feet, ankles; uneven/rapid pulse; chest ▪ Heart transplant
pain; low urine output; nocturia

DIAGNOSIS
OTHER DIAGNOSTICS
ECG
▪ Low-amplitude signals: peak to nadir
measurement of QRS complex being <
5mm (limb leads)/< 10mm (precordial
leads). Low voltage produced due to loss of
viable myocardium
▪ Small QRS complexes: QRS complexes
represent ventricular contraction, restricted
tissue → weaker contraction

OSMOSIS.ORG 39
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