Cardiomyopathies

Dilated Cardiomyopathy

Presents clinically as heart failure with reduced ejection fraction

Typically all 4 chambers are enlarged and there is impaired systolic function of both the LV and the RV

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Characterized by:

Impared LV contractivity

Reduced cardiac output

Elevated LV EDP

Echocardiogram

Usually all 4 chambers are enlarged and there is impaired systolic function of both the LV and the RV

Evaluate LV size

Evaluate systolic function

Evaluate RV size and systolic function

Evaluate PA pressure

M Mode

Increased EPSS

Reduced AP aortic root function

Delayed Mitral valve closure

Etiology

Usually idiopathic

Other possible causes

Chemotherapy (adriomycin), viral infection, myocarditis, postpartum CM, systemic HTN, bacterial infections, AIDS

Signs and Symptoms

Signs: RV/LV failure, thromboembolic events, arrhythmia’s, rales, decreased arterial pulse, tachycardia, murmurs, pulsus alternans

Symptoms:Dyspnea, orthopnea, nocturnal cough, fatigue, pedal edema, low BP, CP, palpitations, hemoptosis, syncope

Related hx: alcoholism, drug abuse, familial, recent infection such as the flu

Complications

Systemic embolization with neurological events (TIA’s) from thrombus formed in the LV or LA

Sudden death

Valvular Regurgiation

Thrombus in the heart

Treatments

Presence of myocardial inflammation: use immunosuppressive therapy (prednisone)

Hemodynamic state: afterload reduction, heart rate control

Anticoagulants

Diuretics

Oxygen therapy

Dietary modification

Eliminate alcohol

Ace inhibitors

Beta blockers

Hypertrophic Cardiomyopathy

Idiopathic, may or may not be asymmetric, thickening of a non-dilated ventricle.

Autosomal dominant inherited disease of the myocardium related to abnormalities in genes coding for contractile proteins

Characteristics

Asymmetric hypertrophy of LV

Normal ventricular systolic function

Impaired diastolic function

Sub-aortic dynamic obstruction

Obstructive – asymmetric

Non obstructive – concentric and apical hypertrophy

Gradient at rest is >30 mmHg


(about 1/3 of patients) The outflow gradient at rest and with provocation is <30mm Hg

Provocable/Latent

The resting gradient is <30mm Hg but obstruction occurs with exercise

A high risk of sudden death (especially during exertion)

Angina

Cannot tolerate exercse

Syncope

A-fib

Systolic Murmur

The degree of obstruction is increased by a reduction in preload, an increase in contractility, a decrease in afterload

Diastolic dysfunction due to stiffness of the ventricles

LV cavity obliteration

Increased Systolic Function

Increased risk of arrythmias

Restrictive Cardiomyopathy

A non-compliant LV associated with elevated diastolic pressures. Systolic function usually preserved with impaired diastolic function

Infiltrative

Non-infiltrative

Entire myocardium is infiltrated by an abnormal substance. (amyloidosis)

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Endocardium and sub endocardium are fibrosed. (Endomyocardial fibrosis

Causes

Pathophysiology:
Rigid, non-compliant ventricular walls
Restricted diastolic filling
Restricted cardiac size with preserved systolic function

characterized by heart failure with preserved ejection fraction and

predominant diastolic dysfunction due to a stiff and thickened myocardium

Heart failure is due to the inability to maintain a normal cardiac output only with an elevated LV end-diastolic pressure

Usually Idiopathic

Sarcoidosis
Conduction defects and pericardial effusions
Hypersinophilic syndrome
LV thrombus formation
Radiation-induced
RC of both LV and RV
Accelerated calcific valve disease
Coronary atherosclerosis of arts in radiation field
Difficult to differentiate RC from constrictive pericarditis

Signs/Symptoms

Peripheral edema, ascites, atrial arrhythmia, biatrial enlargement, MR/TR. Dyspnea, palpitation, fatigue, poor exercise tolerance, anorexia

Treatment

Treat symptoms, ACE inhibitors, calcium channel blockers, diuretics, anticoagulation therapy, pacemaker. Cardiac transplantation

Echo Findings

2-D: LVH with bright, shiny echogenic appearance (Amyloidosis). Apical obliteration. Normal systolic function with impaired diastolic function. Biatrial enlargement

Doppler: MR/TR often moderate to severe. Pulmonary HTN often present. LVOT gradient may be present. Diastolic dysfunction needs to be assessed

Pericardial effusion commonly seen

Thick walled LV

Abnormal Diastolic function

RV free wall thickening

Enlarged Atria

Pulmonary Hypertension

Primary

Idiopathic

Hemochromocytosis

An iron storage disease that affects multiple organ and tissue systems which can result in tissue damage

Secondary (iron overload due to multiple blood transfusions in chronic anemia pts or alcoholic liver disease

Echo findings: increased LV thickness, LV dilation, LAE

Primary: called “bronze diabetes”. Liver function abnormalities, CHF, cardiomegaly. Liver, skin, heart disease

Treatment is phebectomy

Secondary: High output cardiac failure from anemia.

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