Cardiomyopathies Flashcards
what is a cardiomyopathy?
What does it typically lead to?
a disorder within the cardiac myocytes themselves which results in abnormal cellular and hence cardiac performance
typically leads to irreversible decline in cardiac function
T/F: in cardiomyopathies, LVEF is the most powerful risk stratifer
TRUE
cardiomyopathies can have primary and secondary causes, describe each
- primary cause → pathologic processes intrinsic to cardiac myocytes themselves
- secondary cause → result of a pathologic change in cardiac myocytes brought on by a systemic disease process
List the types of cardiomyopathies (3)
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy
what is the most common form of cardiac myopathy?
dilated cardiomyopathy
describe dilated cardiac myopathy
does it affect systolic function?
increased heart weight, 1 or more chambers are dilated and walls are thinned
impaired systolic function with cardiac enlargement
fibrosis is common
T/F: in dilated cardiac myopathy, there are hypertrophied myocytes with mitochondrial abnormalities
TRUE
thinner walls = fewer myocytes available to do work and the available myocytes have to work harder
List causes of dilated cardiomyopathy (6)
- ETOH/toxic substances → most common cause
- poor nutrition (B1 deficiency)
- Idiopathic, family history
- AIDS
- CT
- cancer therapies
what are symptoms of dilated cardiomyopathy? (4)
- fatigue
- exertional dyspnea, SOB, cough
- orthopnea, paroxsymal nocturnal dyspnea
- increasing edema, weight, or abdominal girth
list signs of dilated cardiomyopathy?
- Tachypnea: increased RR
- Tachycardia
- HTN or hypotension
- Characteristic heart circular shape, almost like a bowling ball
List other pertinent findings for dilated cardiomyopathy (5)
- signs of hypoxia (cyanosis, nail clubbing)
- jugular venous distension (JVD)
- pulmonary edema (crackles and/or wheezes)
- enlarged liver
- ascities or peripheral edema
Describe nonpharmacologic management of dilated cardiomyopathy
- sodium diet restricted to 2 g/day
- fluid restriction
describe hypertrophic cardiomyopathy
charactertized by a thick LV wall with a nondilated cavity
the resulting cardiac hypertrophy is out of proportion to the hemodynamic load
9 gene deficits which cause defects in sarcomeric proteins
describe the pathology/progression of hypertrophic cardiomyopathy (4 steps)
- genetic defect in myocytes
- normal BP percieved as excessive by defective myocytes
- hypertrophy occurs as a compensatory mechanism
- ultimately heart decompensate
T/F: hypertrophic cardiomyopathy is the single most common cause of death in apparently young people
TRUE
first clinical manifestation is often sudden death
describe restrictive/infiltrative cardiomyopathy
characterized by restricted diastolic filling/loss of compliance and idiopathic fibrosis
systolic function is normal but EDVs are diminished because chambers cannot expand
what are ESVs and EFs like in restrictive/infiltrative cardiomyopathy?
normal
what are the symptoms of restrictive/infiltrative cardiomyopathy? (4)
- exertional dyspnea
- abdominal swelling
- ankle edema
- fatigue
List some causes of restrictive/infiltrative cardiomyopathy (7)
- scleroderma
- diabetes
- sarcoidosis (fibrotic scaring secondary to myocardial infiltrates)
- hemochromatosis (excessive deposition of Fe)
- metastatic cancers
- secondary to anthracycline trx
- radiation (mediastinal)
List other cardiac pathologies
- Valvular disease
- Arrthymias
- Pericardial disease
- Pericarditis
- Myocarditis
- Aneursym
- Diabetic heart disease
list 2 types of valvular diseases in the heart
- stenosis
- regurgitation
describe the valvular disease, regurgitation
valve leaflets fail to completely close or the edges do not fully meet. Permits backward flow of blood. Functional and anatomic implications
describe the valvular disease stenosis
the leaflets do not provide a full opening for blood to flow through.
what is an arrthymia?
a disturbance of rate and/or rhythm
describe the pericardial disease, pericardial effusion
increased fluid in the pericardial cavity will result in the heart having to work much harder against that pressure which reduces the amount the heart can fill and pump blood effectively
medical emergency
define pericarditis
a swelling or irritation of the thin saclike membrane surrounding the heart
may be caused by a viral infection or heart attack. In many cases it is idiopathic
list symptoms of pericarditis (3)
- sharp chest pain
- pain worsened by lying supine, inhaling deeply or cough
- leaning forward decreases pain
what is myocarditis?
what 3 things can it lead to?
inflammation of myocardium
usually caused by a viral infection
can lead to HF, arrhytmia, sudden death
what is an aneursym?
an abnormal stretching (dilation) in the wall of an artery, vein, or the heart with a diameter that is at least 50% greater than normal
named according to the specific site of formation
T/F: an aneurysm is an area of weakness that can rupture?
TRUE
what is a false aneurysm?
the wall ruptures and a blood clot is retained in an outpouching of tissue
what are 4 things that can lead to diabetic heart disease?
- Metabolic effects due to FFA, insulin resistance
- Structural: myocardial fibrosis and ECM changes
- Reduced perfusion due to small vessel disease
- Autonomic dysfunction reduced HRR
