Clin Med - Cardiomyopathy Flashcards
Cardiomyopathy definition
diseases of the myocardium associated with mechanical and/or electrical dysfunction
Primary cardiomyopathy
genetic, mixed, acquired
Secondary cardiomyopathy
Multi-organ
Hypertrophic Cardiomyopathy
- Autosomal dominant (MYH7, MYBPC3, TNNT2, TNNI3)
- Septal hypertrophy
- Blood that normally goes into aorta is compromised because the septum covers it
Septal hypertrophy causes:
- Decrease in cardiac output
- Left ventricle becomes stiff
Sudden death in hypertrophic cardiomyopathy
Symptoms appear mainly during exercise d/t myocardial oxygen demand (often seen in young athletes)
Hypertrophic Cardiomyopathy Symptoms
- Dyspnea
- Chest pain
- Palpitations
- -Excitement of myocardium due to PVCs
- -Enough PVCs in a row gets you Vtach - Syncope* - most significant
Hypertrophic Cardiomyopathy Signs
- Systolic ejection murmur
- -Murmur increased with Valsalva/squatting
- -Murmur decreased with hand grip - Prominent, nondisplaced point of maximal impulse
Hypertrophic Cardiomyopathy diagnostic gold standard
Echocardiogram
Hypertrophic cardiomyopathy ECHO
- Increased septum to LV wall thickness (greater than 1.3 to 1)
- EKG – LV hypertrophy
- Genetic test – cardiac muscle biopsy (myofibril disarray) muscle fibers are not lined up parallel
Hypertrophic cardiomyopathy prognosis & treatment
Prognosis: 1-3% mortality
Treatment: -Slow HR, allow more time for diastolic filling -Increase cardiac output beta blockers and calcium channel blockers -Implantable Cardioverter Device (ICD) -Patient’s die from fibrillation -Cardiac surgery -Heart transplantation
Hypertrophic cardiomyopathy patient education
- No competitive sports
- Screening of family members
- Healthy diet
- Immunizations
- Medical bracelet
Referral – needs to be followed by a cardiologist
Dilated Cardiomyopathy Physiology
- Dysfunction in the ability of the heart to contract
- Decreased EF
- Heart chambers “dilate” to compensate
- Dilation gets progressively worse, ventricles first, atrium second
Dilated cardiomyopathy etiology
- Genetic: protein in muscles cells that do not allow muscles to contract
- Myocarditis
- Pregnancy
- Idiopathic
Dilated cardiomyopathy S&S
Symptoms
-Fatigue, DOE, Swelling, Edema, Ascites
Signs
-Tachycardia, Tachypnea, HTN
Dilated cardiomyopathy signs when disease is in later stages
- Cyanosis/clubbing
- Jugular venous distention
- Pulmonary edema (crackles or wheezes)
- S3 gallop
- Hepatomegaly
Dilated Cardiomyopathy Labs and Diagnostic Findings
- BNP – elevated in conditions with “stretching” of the heart – not specific
- EKG – abnormal, non-specific
- CXR – cardiomegaly
- Echocardiogram – dilation of chambers and decreased EF
Dilated Cardiomyopathy Treatment
Treat the same as for CHF (ACE, B-blocker, Diuretics, etc)
Dilated Cardiomyopathy Patient Education
- Exercise
- Quit smoking
- No alcohol/drug use
- Maintain healthy weight
- Eat heart healthy diet
Refer to cardiologist
Primary Restrictive Cardiomyopathy Etiology
- Rare, only 5% of world population
- Disease of the heart muscle
- Fibrosis or scarring of muscles (via radiation, idiopathic)
Primary Restrictive Cardiomyopathy Infiltration (4)
- Amyloidosis – protein
- Sarcoidosis – granulomas
- Genetic diseases where metabolites are deposited
- Hemochromatosis – excessive iron in the parenchyma tissue (lungs or heart)
Primary Restrictive Cardiomyopathy characteristics (4)
- Ventricles fail to relax (dilate)
- Muscle stays the same size and the heart can not fill properly
- Decreased filling = decrease volume of blood leaving heart
- Diastolic heart failure
Primary Restrictive Cardiomyopathy Clinical Manifestations
- Shortness of breath
- Weakness
- Fatigue
- Edema
- Ascites
Primary Restrictive Cardiomyopathy Diagnostic Tests
- CXR – pulmonary congestion +/- with NORMAL heart size
- EKG – low voltage QRS complexes
- Echocardiogram – increased ventricular thickness and maybe atrial enlargement
Primary Restrictive Cardiomyopathy Prognosis
- Usually fatal when diagnosed in infancy
- Progressively worse
- Heart transplant
