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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