Cardiomyopathy: Dr. Ibrahim Abuasbeh

cardiomyopathy
Dr. Ibrahim Abuasbeh

Definition
• The term cardiomyopathy is purely descriptive, meaning disease of
the heart muscle
• 2006 AHA defined cardiomyopathies as:
– “a heterogeneous group of diseases of the myocardium associated with

mechanical &/or electrical dysfunction that usually (but not invariably) exhibit
inappropriate ventricular hypertrophy or dilatation and are due to a variety of
causes that frequently are genetic.
– Cardiomyopathies either are confined to the heart or are a part of generalized

systemic disorders, often leading to cardiovascular death or progressive heart
failure-related disability.”
Functional Classification
• Dilated
– Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction
• Hypertrophic
– Inappropriate myocardial hypertrophy, with or without left ventricular
obstruction
• Restrictive (Infiltrative)
– Abnormal ventricular filling with diastolic dysfunction
Dilated Cardiomyopathy
Dilated Cardiomyopathy -
Definition
• Primary (idiopathic) is a disease of unknown etiology that principally
affects the myocardium leading to LV dilation and systolic dysfunction
• Secondary causes include ischemia, alcoholic, peripartum, post-

infectious, viral
• Most common of the cardiomyopathies

Schematic of Dilated CM
DCM - Incidence and Prognosis
• Prevalence is 36 per 100,000 population
• Third most common cause of heart failure
• Most frequent cause of heart transplantation
• DCM accounts for approximately 10,000 deaths and 46,000
hospitalizations per year in the US
• Complete recovery is rare
Clinical Manifestations
• 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
Clinical Manifestations (Cont)
• Symptoms/Signs of heart failure
– Pulmonary congestion (left heart failure)

dyspnea (rest, exertional, nocturnal), orthopnea
– Systemic congestion (right heart failure)
edema, nausea, abdominal pain, nocturia
– Low cardiac output
– Hypotension, tachycardia, tachypnea
– Fatigue and weakness
• Arrhythmia
– Atrial fibrillation, conduction delays, complex PVC’s, sudden death
Diagnostics
• CXR (enlarged heart, CHF)
• Electrocardiogram (tachycardia, A-V block, LBBB, NSSTT changes,

PVC’s)
• 24-hour Holter monitor

– if lightheadedness, palpitation, syncope
• Echocardiogram (left ventricular dilation, global hypokinesis, low EF)
• Myocardial biopsy, rare
• Cardiac catheterization (R/O CAD)

– if age >40, ischemic history, high risk profile, abnormal ECG
DCM - Treatment
• Limit activity based on functional status
• Salt restriction
• Fluid restriction
• Initiate medical therapy
– ACE inhibitors, diuretics
– B blockers, aldosterone anagonists
– Digoxin,
– Hydralazine/nitrate combination
– Anticoagulation prn (EF <30%, hx of embolic events)
– Implantable defibrillators
DCM – Treatment (Cont)
• Cardiac transplantation
– This disorder is the most common indication for cardiac
transplantation
– Survival after transplant is
• 80% one year
• 70% 5 years
Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathy
(HCM)
• Defined
– First described in 1869 and accepted as a clinical entity in the 1950’s
– Prevalence 2:1000
– Genetic disease characterized by hypertrophy of the left ventricle with
marked variable clinical manifestations morphologic and
hemodynamic abnormalities
– Small LV cavity, septal hypertrophy which can be asymmetric (ASH),
systolic anterior motion of the mitral valve leaflet (SAM), +/-
obstruction of left ventricular outflow with low stroke volume, but
elevated EF
Variants of HCM
8 - 10% 15 - 20%
65 -
70%
HCM – ASH Without Obstruction
• The major abnormality of the heart in HCM is
an excessive thickening of the muscle.
Thickening usually begins during early
adolescence and stops when growth has
finished. It is uncommon for thickening to
progress after this age
• The left ventricle is almost always affected,
and in some patients the muscle of the right
ventricle also thickens
• Hypertrophy is usually greatest in the septum.
The muscle thickening in this region may be
sufficient to narrow the outflow tract. This
thickening is associated with obstruction to
the flow of blood out of the heart into the
aorta
HCM – ASH With Obstruction
• Asymmetric septal hypertrophy with
obstruction to the outflow of blood
from the heart may occur. The mitral
valve touches the septum, blocking
the outflow tract. Some blood is
leaking back through the mitral valve
causing mitral regurgitation
Pathophysiology of HCM
• Dynamic LV outflow tract obstruction
– Outflow tract gradient (>30 mm Hg), considered severe if >50 mm Hg
(occurs in 25-30% of cases leading to name hypertrophic obstructive
cardiomyopathy)
• Diastolic dysfunction
– Impaired diastolic filling, ↑ filling pressure
• Myocardial ischemia
• Mitral regurgitation
• Arrhythmias
• Asymptomatic
– Echocardiographic finding only
• Symptomatic
– Dyspnea in 90%
– Angina pectoris in 75%
– Fatigue, pre-syncope, syncope,↑ risk of SCD
– Palpitation, PND, CHF, dizziness
– Atrial fibrillation, thromboembolism
EKG Findings
• Abnormal in 85-90% of cases
• LVH, Strain pattern
• Abnormal ST-T’s, giant T wave inversions
• Abnormal Q’s,
• Bundle Branch Block
• Left atrial enlargment
• Ventricular arrhthymias
Echocardiogram
• Left ventricular hypertrophy >1.3 cm (usually >1.5 cm)
• Septal to posterior wall ratio >1.3:1
• Mitral regurgitation
• Systolic anterior motion of the mitral valve (SAM)
• Premature midsystolic closure of the aortic valve
• Asymmetric septal hypertrophy (ASH)
• Diastolic dysfunction
• Left ventricular outflow tract obstruction (LVOT)
Echocardiogram
• LVH usually develops between 5-15 years of age in HCM
• A normal ECHO in a young child does not R/O the diagnosis
• Serial ECHOs are recommended up to the age of 20 yr where there is
a family history of HCM
Natural History & Clinical Course
• Clinical presentation from infancy to old age
• Variable clinical course 25 % of cohort achieve normal longevity
• Annual mortality 3% in referral centers probably closer to 1% for all patients
• Course may be punctuated by adverse clinical events: sudden cardiac death,
embolic stroke, and consequences of heart failure
• Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden death
Natural History & Clinical Course
• Risk of SCD higher in children, may be as high as 6% per year,

majority have progressive hypertrophy
• Accounts for 36% of deaths in athletes <35 years
• Clinical deterioration usually is slow
• Poor prognosis in males, young age of onset, family Hx of SCD, Hx of
syncope, exercise induced hypotension (worst)
• Progression to DCM occurs in 10-15%
Risk Factors For SCD
• Young age (<35 years)

• “Malignant” family history of sudden death
• Aborted sudden cardiac death
• Sustained VT or SVT
• Non-sustained VT on holter monitoring
• Atrial fibrillation
• Dilated left ventricle
• NYHA classes III or IV
Risk Factors For SCD
• Syncope
• Severe hypertrophy (>3.0 cm)
• Abnormal BP response to exercise
• Coronary artery disease
• Strenuous exercise or work
Recommendations for Athletic
Activity
• Low-risk older patients (>30 years) may participate in athletic
activity if all of the following are absent:
– Ventricular tachycardia on Holter monitoring
– Family history of sudden death due to HCM
– History of syncope
– Severe hemodynamic abnormalities, gradient ≥50 mmHg
– Exercise induced hypotension
– Moderate or severe mitral regurgitation
– Enlarged left atrium (≥5.0 cm)
– Paroxysmal atrial fibrillation
– Abnormal myocardial perfusion
Management of HCM
• Beta-adrenergic blockers (atenolol)

• Calcium channel blockers (norvasc, cardizem, etc)
• Anti-arrhythmics – Amiodarone,
• Pacemakers (ICD)
• Myomectomy (resection of septum)
• Alcohol septal ablation (controlled MI through septal perforator
perfusing basal septum) wall thinningdecreases in LVOTO
• Transplantation
HCM vs. Athletic Heart
HCM Athletic heart
• Can be asymmetric • Concentric & regresses
• Wall thickness: > 15 mm • < 15 mm
• LA: > 40 mm • < 40 mm
• LVEDD : < 45 mm • > 45 mm
• Diastolic function: always abnormal • Normal
• Occurs in about 2% of elite althetes – typical
sports, rowing, cycling, canoeing
• Former athletes & weekend warriors do NOT
develop athletic heart
• Elite female athletes do NOT develop athletic
heart
Hypertensive HCM of the Elderly
• Characteristics
– Modest concentric LV hypertrophy (<22 mm)
– Small LV cavity size
– Associated hypertension
– Ventricular morphology greatly distorted with reduced outflow tract
Restrictive Cardiomyopathy
Restrictive Cardiomyopathies
• Hallmark: abnormal diastolic function

• Rigid ventricular wall with impaired ventricular filling
• Bear some functional resemblance to constrictive pericarditis
• Importance lies in its differentiation from operable constrictive pericarditis
• Much less common then DCM or HCM outside the tropics, but frequent cause of
death in Africa, India, South and Central America and Asia primarily because of
the high incidence of endomyocardial fibrosis in those regions
Classification
• Idiopathic • Endomyocardial
• Myocardial – Endomyocardial fibrosis
– Noninfiltrative
– Hyperesinophilic synd
• Idopathic
• Scleroderma – Carcinoid
– Infiltrative – Metastatic malignancies
• Amyloid
– Radiation, anthracycline
• Sarcoid
• Gaucher disease
• Hurler disease
– Storage Disease
• Hemochromatosis
• Fabry disease
• Glycogen storage
• Symptoms of right and left heart failure

• Jugular Venous Pulse
• Echo-Doppler
– Abnormal mitral inflow pattern
– Prominent E wave (rapid diastolic filling)
– Reduced deceleration time (↑ LA pressure)
Restriction vs. Constriction
• History can provide important clues

– Constrictive pericarditis
• History of TB, trauma, pericarditis, collagen vascular disorders
– Restrictive cardiomyopathy
• Amyloidosis, hemochromatosis
– Mixed
• Mediastinal radiation, cardiac surgery
Treatment
• No satisfactory medical therapy

• Drug therapy must be used with caution
– Diuretics for extremely high filling pressures
– Vasodilators may decrease filling pressure
– (?) Calcium channel blockers to improve diastolic compliance
– Digitalis and other inotropic agents are not indicated
Questions?

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cardiomyopathy

Dr. Ibrahim Abuasbeh

• 2006 AHA defined cardiomyopathies as:

– “a heterogeneous group of diseases of the myocardium associated with

– Cardiomyopathies either are confined to the heart or are a part of generalized

• Secondary causes include ischemia, alcoholic, peripartum, post-

• Most common of the cardiomyopathies

• Symptoms may be gradual in onset

• Symptoms/Signs of heart failure

– Pulmonary congestion (left heart failure)

• Electrocardiogram (tachycardia, A-V block, LBBB, NSSTT changes,

• 24-hour Holter monitor

• Echocardiogram (left ventricular dilation, global hypokinesis, low EF)

• Myocardial biopsy, rare

• Cardiac catheterization (R/O CAD)

• Risk of SCD higher in children, may be as high as 6% per year,

• Young age (<35 years)

• Beta-adrenergic blockers (atenolol)

• Hallmark: abnormal diastolic function

• Symptoms of right and left heart failure

• History can provide important clues

• No satisfactory medical therapy

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