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CARDIOMYOPATHY

MBCHB VI

DR D M KILLINGO

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 Cardiomyopathy
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 Definition: Intrinsic or primary myocardial disease

not 2° to congenital, hypertensive, coronary, valvular,
or pericardial disease
 Functional classification: dilated, hypertrophic, or

restrictive
 LV dysfunction 2° to MI often termed "ischemic

cardiomyopathy': is not a true cardiomyopathy –

same for Hypertensive cardiomyopathy
(i.e. primary myocardial disorder) since the primary
pathology is CAD
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 Dilated Cardiomyopathy (DCM)

Definition: unexplained dilation and impaired systolic function of one

or both ventricles
Major Risks Factors for DCM
 Alcohol, cocaine, family history and

 obesity.

Etiology
• Idiopathic (presumed viral or idiopathic) - 50% of DCM
• Alcohol
• Familial/ genetic
• Uncontrolled tachycardia (e.g. persistent rapid AF) referred to as
tachycardiomyopathy
• Collagen vascular disease: SLE, polyarteritis nodosa, dermatomyositis,
progressive systemic sclerosis 7/11/20 03:11 AM
 DCM aetiology
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 Infectious: viral (coxsackie B, HIV), Chagas disease, Lyme disease,

Rickettsial diseases, acute
 Rheumatic fever, toxoplasmosis
 Neuromuscular disease: Duchenne muscular dystrophy, myotonic
dystrophy, Friedreich's ataxia
 Metabolic: uremia, nutritional deficiency (thiamine, selenium, carnitine)
 Endocrine: hyper/hypothyroidism, DM, pheochromocytoma
 Peripartum cardiomyopathy
 Toxins: cocaine, heroin, organic solvents
 Drugs: chemotherapies (doxorubicin, cyclophosphamide), anti-
retrovirals, chloroquine, clozapine, TCA
 Radiation

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 Signs and Symptoms
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DCM may present as:

• CHF
• Systemic or pulmonary emboli
• Arrhythmias
 Myocardial disease /myocarditis as the primary

disease
• Sudden death (major cause of mortality due to fatal
arrhythmia)

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 Investigations
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 Bloodwork: CBC, electrolytes, Cr, bicarbonate, BNP, CK,

troponin, LFTs, TSH, TIBC
 ECG: variable ST-T wave abnormalities, poor R wave
progression, conduction defects (e.g. BBB), arrhythmias (non-
sustained VT)
 CXR: global cardiomegaly (globular heart), signs of CHF, pleural
effusion
 Echocardiogram: chamber enlargement, global hypokinesis,
depressed LVEF, MR and TR, mural thrombi
 Endomyocardial biopsy: not routine, used to rule out a treatable
cause
 Angiography: in selected patients to exclude ischemic heart
disease 7/11/20 03:11 AM
 Management principles

 Treat underlying disease /aetiolgy: e.g. abstinence from alcohol

 Treat CHF
 Thromboembolism prophylaxis: anticoagulation with warfarin
indicated for: AF, history of thromboembolism or documented
thrombus LVEF <30%.
 Treat symptomatic or serious arrhythmias
 Immunize against influenza and S. pneumoniae
 Consider surgical options (e.g. LVAD, transplant, volume
reduction surgery) in appropriate candidates with severe, drug
refractory disease
 lCD may be considered among patients with a LVEF <30-35%

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 Prognosis
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 Depends on etiology
 Better with reversible underlying cause, worst with
infiltrative diseases, HIV, drug-induced
 Cause of death usually CHF (due to pump failure)
or sudden death 2° to ventricular arrhythmias
 Systemic emboli are significant source of morbidity
 20% mortality in 1st yr, 10% per yr after

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 Hypertrophic Cardiomyopathy (HCM)

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 Definition : defined as unexplained ventricular

hypertrophy
 Various patterns of HCM are classified, but most
causes involve asymmetric pattern of septal
hypertrophy

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 Aetiology and Pathophysiology
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 Histopathology features include myocyte disarray,

myocyte hypertrophy, and interstitial fibrosis
 Cause is a genetic defect involving one of the
cardiac sarcomeric proteins
 (>400 mutations associated with autosomal
dominant inheritance, incomplete penetrance)
 Prevalence of 1/500-1/1000 in general population
 Generally presents in early adulthood

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 Hemodynamic Classification

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 Hypertrophic obstructive cardiomyopathy

(HOCM): dynamic LV outflow tract (LVOT)
 Obstruction, either at rest or with provocation,
defined as LVOT gradient of at least 30 mmHg
 Non-obstructive HCM: no LVOT obstruction
 Many patients have diastolic dysfunction (impaired
ventricular filling secondary to LVH which decreases
compliance)

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 Signs and Symptoms

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 Clinical manifestations: asymptomatic (common,
therefore screening is important),
 SOB on exertion, angina, presyncope/syncope (due to

LV outflow obstruction or arrhythmia), CHF,

arrhythmias, SCD
 Pulses: rapid upstroke, "spike and dome" pattern in

carotid pulse (in HCM with outflow tract

obstruction)
 Precordial palpation: sustained, double impulse, 'triple

ripple' (triple apical impulse in HOCM), LV lift occurs.

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 Precordial auscultation: normal or paradoxically split

S2, S4,
 Harsh systolic diamond-shaped murmur at LLSB or
apex,
 Murmur secondary to LVOT obstruction -enhanced by
squat to standing position or Valsalva (as compared to
AS)
 Often with pansystolic murmur of mitral regurgitation
due to systolic anterior motion (SAM ) of anterior
mitral valve leaflet.
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 Investigations
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• ECG: LVH, high voltages across precordium,

prominent Q waves (lead I, aVL, VS, V6),
 Tall R wave in V1, P wave abnormalities

• Echo-Doppler study: asymmetric septal hypertrophy

(less commonly apical), SAM of mitral valve and
MR;
 LVOT gradient can be estimated by Doppler

measurement
• Cardiac catheterization (only when patient being
considered for invasive therapy)
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 Management

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• Avoid factors which increase obstruction (e.g.

volume depletion)
• Avoidance of all competitive sports
• Treatment of HCM (with LVOT obstruction)
• Medical agents: B-blockers, disopyramide,
veraparmill (only in patients without resting or
provocable obstruction)
• Avoid nitrates, diuretics and ACEI as they decrease
outflow tract diameter and worsen symptoms

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 Patients with drug-refractory symptoms
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• Surgical myectomy
• Septal ethanol ablation
• Dual chamber pacing / ICD
• Treatment of ventricular arrhythmias: amiodarone or
lCD
• First-degree relatives of patients with HCM should be
screened annually during adolescence
(physical, ECG, 2D echo), then serially every 5 yrs
during adulthood
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 Myomectomy in HOCM
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 Prognosis

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 Potential complications: AF, VT, CHF, sudden cardiac death

(1% risk/yr; most common cause of SCD in young athletes)
 Major risk factors for sudden death (consider lCD placement):

• history of survived cardiac arrest/sustained VT

• family history of multiple premature sudden deaths
 Other factors associated with increased risk of sudden cardiac

death include;
• syncope (presumed to be arrhythmic in origin)
• non-sustained VT on ambulatory monitoring
• marked ventricular hypertrophy (maximum wall thickness ~30
mm)
• abnormal BP in response to exercise (in young patients with
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HCM)
 Restrictive Cardiomyopathy (RCM)
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Definition: defined as impaired ventricular filling

with reserved systolic function in a non-dilated,non-
hypertrophied ventricle secondary to factors that
decrease myocardial compliance (fibrosis and/or
infiltration)

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 Aetiology
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• Infiltrative: amyloidosis, sarcoidosis

• Non-infiltrative: scleroderma, idiopathic myocardial
fibrosis
• Storage diseases: hemochromatosis, Fabry's disease,
Gaucher's disease, glycogen storage diseases
• Endomyocardial fibrosis, Loeffler's endocarditis or
eosinophilic endomyocardial disease
• Radiation heart disease
• Carcinoid syndrome (may have associated tricuspid
valve or pulmonary valve dysfunction)
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 Clinical Manifestations
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 CHF (usually with preserved LV systolic function),

 Arrhythmias
 Elevated JVP with prominent x and y descents,
 Kussmaul's sign
 S3, S4, MR, TR may be present
 Thromboembolic events

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 Investigations
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• ECG: low voltage, non-specific, diffuse ST-T wave

changes± non-ischemic Q waves
• CXR: mild cardiac enlargement
• Echocardiogram: LAE, RAE; specific Doppler
findings with no significant respiratory variation
• Cardiac catheterization: increased end -diastolic
ventricular pressures
• Endomyocardial biopsy: to determine etiology
(especially for infiltrative RCM)

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 Principles of Management
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 Exclude constrictive pericarditis

 Treat underlying disease: control HR, anticoagulate
if AF present
 Supportive care and treatment for CHF,
arrhythmias
 Heart transplant: might be considered for CHF
refractory to medical therapy
 Prognosis - depends on etiology

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