Cardiomyopathies/Tumor Flashcards
Cardiomyopathy
- Definition
- Types
- Definition
- Heart muscle disease not related to hypertension, atherosclerosis, valvular disease, or congenital heart disease
- Cardio = heart
- Myo = muscle
- Pathy = disease
- May refer to end stage cardiac disease (i.e. ischemic cardiomyopathy)
- Heart muscle disease not related to hypertension, atherosclerosis, valvular disease, or congenital heart disease
- Types
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy
- Arrhythmogenic right ventricular cardiomyopathy

Dilated Cardiomyopathy
- General
- Etiology
- Underlying associations
- Histologic pathology
- Genetics
- Complications
- General
- Most common form
- 4-chamber dilation
- Cardiomegaly (increased size)
- Round, globoid heart appearance
-
Massive cardiac enlargement
- Up to 1200 grams (normal = 350-400)
- Functional mitral & tricuspid insufficiency
- Etiology
- Most often unknown (idiopathic)
- Underlying associations
- Alcohol (directly cardiotoxic)
- Obesity
- Pregnancy (peripartum)
- Post-viral infection (i.e. myocarditis)
- Chagas’ disease
- Cardiotoxic drugs
- Adriamycin (doxorubicin)
- Cancer chemotherapy
- Directly cardiotoxic
- Thiamine deficiency
- Sleep apnea
- Histologic pathology
- Myofiber hypertrophy & disarray
- “Lacy” interstitial fibrosis
- Genetics
- Mutant cardiac proteins
- Cytoskeletal: dystrophin, desmin
- Sarcomeric: myosin heavy chain
- Muscular: actin
- Complications
- Heart failure
- Cardiac arrhthmia
- Pulmonary edema
- Valvular insufficiency
- Stroke
- Sudden cardiac death
- 5-year survival rate = 25%
Peripartum Cardiomyopathy
- aka Cardiomyopathy of pregnancy
- Dilated cardiomyopathy
- Multifactorial etiology
- Extends from latter pregnancy up to 6 months after delivery
- Sudden unexpected death or heart failure
Hypertrophic Cardiomyopathy
- General
- Clinical consideration
- Histologic pathology
- Genetics
- Complications
- General
- Cardiomegaly
- Up to 1000 grams
- Enlarged thickened heart
- Prominent LV
-
100% genetic cause
- No other structural or funcitonal cause (ex. HTN, valve)
- Clinical consideration
- Can cause functional aortic stenosis or obstruction of LV outflow
- Idiopathic Hypertrophic Subaortic Stenosis (IHSS): old term
- Histologic pathology
- Disoriented, tangled, enlarged myofibers
-
Interstitial & myocardial fibrosis
- Infarcts due to increased oxygen demand
- Contrasted w/ “lacy” fibrosis of dilated cardiomyopathy
- Genetics
- 100% of cases
- Mutant cardiac proteins
- Sarcomeric: myosin heavy chain, light chain
- Troponins T &
- Muscular: actin
- Complications
- Cardiac arrhythmia
- Sudden cardiac death
- Esp prominent in young athletes
- Good survival rate w/ medical treatment
Three Things that Cause LV Hypertrophy
- Hypertrophic cardiomyopathy
- Hypertension
- Aortic stenosis
Restrictive Cardiomyopathy
- General
- Main causes
- Other causes
- General
- Stiffening of myocardium or endocardium that prevents efficient contraction of the heart
- Main causes
- Cardiac amyloidosis
- Endocardial fibroelastosis
- Other causes
- Sarcoidosis
- Post-radiation therapy
- Loeffler’s endomyocarditis (eosinophilic)
- Davies’ disease (non-eosinophilic)
Cardiac Amyloidosis
- General
- Gross pathology
- Microscopic pathology
- High yield phrase
- Prognosis
- General
- Build up of abnormal fibril in heart muscle
- Stiffens the myocardium
- Commonly seen in multiple myeloma
- Also can be age-related (senile amyloid)
- Impairs contractility & electrical conduction
- Gross pathology
- Myocardium has a firm “waxy” appearance
- Microscopic pathology
- Amorphous acellular material
- Light pink to red
- In vessels & myocardium
- High yield phrase
- Apple-green birefringence w/ congo red stain
- Prognosis
- Poor prognosis overall
- Symptoms related to inability to contract & impaired electrical conductivity
- Senile (>80yo) has better prognosis
Endocardial Fibroelastosis
- General
- Pathology
- Clinical findings
- Treatment
- General
- Very rare disease of infants & young children
- Thickening & fibrosis of endocardium
- Part of HEC syndrome
- Hydrocephalus
- Endocardial fibroelastosis
- Cataracts
- Pathology
- Marked thickening & fibrosis of endocardium
- Clinical findings
- Related to inability of heart ot contract & pump blood
- Treatment
- Heart transplant
Which one of the following is a constrictive pericarditis?
- Amyloidosis
- Suppurative pericarditis
- Endomyocardial fibroelastosis
- Hypertrophic cardiomyopathy
- Post-viral cardiomyopathy
- Suppurative pericarditis
Arrhythmogenic Right Ventricular Cardiomyopathy
- aka
- Hallmakr feature
- Origin
- Clinical features
- aka
- Arrhythmogenic right ventricular dysplasia
- Hallmark feature
- Fatty infiltration & fibrosis of right ventricular myocardium
- Origin
- Genetic
- Clinical features
- Rare autosomal dominant disease
- Sudden death due to arrhythmia
- Right heart failure
- Typically discovered in younger patients (early adult life)
- Naxos syndrome: ARVC + hyperkeratosis of palms
Left Heart Failure
- General
- Caused by…
- Leads to…
- Pathology
- General
- Left side of heart doesn’t work
- Blood backs up into lungs
- Caused by…
- HTN
- Atherosclerosis
- Aortic or mitral valve disease
- Leads to…
- Pulmonary edema
- Congestion
- Pathology
- Pulmonary congestion & alveolar edema
- “Heart failure cells” in alveoli
- Respiratory failure symptoms
Right Heart Failure
- General
- Caused by…
- Leads to…
- Pathology
- General
- Right side of heart doesn’t work
- Blood backs up into systemic & portal circulation
- Caused by…
-
Left heart failure (most common cause)
- Right heart failure is most common in the setting of left hear failure
- Cor pulmonale: when RHF occurs alone
- Pulmonary disease (COPD, pulmonary HTN)
-
Left heart failure (most common cause)
- Leads to…
- Systemic / peripheral edema
- Pathology
-
Nutmeg liver
- Blood backs up into hepatic sinusoids
- Becomes congested & dilated
- Begins in centrilobular region (hepatic veins)
- Congestive hepatosplenomegaly
- Lower extremity edema
- Ascites
-
Nutmeg liver
Congestive Heart Failure
- Most common presentation of “heart failure”
- Typically refers to the clinical setting of right & left heart failure
Scenario 1
- 78yo man
- Chronic benign HTN
- Symptoms of pulmonary edema
- Shortness of breath
- Cough
- Frothy sputum
Left heart failure caused by systemic vascular disease (HTN)
Scenario 2
- 78yo man
- Chronic benign HTN
- Ascites & lower extremity edema
Right heart failure caused by left heart failure
Scenario 3
- 78yo man
- Chronic emphysema
- Ascites & lower extremity edema
Right heart failure caused by pulmonary disease (Cor Pulmonale)
Scenario 4
- 56yo
- Chronic alcoholic
- Congestive heart failure
- Large cardiac silhouette on chest x-ray
Dilated cardiomyopathy (most often idiopathic, can have other associations)
Scenario 5
- 28yo
- Dies suddenly w/ no known pre-existing conditions
- 950 gram heart w/ massive LVH
Hypertrophic cardiomyopathy (genetic cause)
Scenario 6
- 78yo man
- Signs of heart fialure
- Biopsy shows amorphous material in myocardium w/ “apple-green birefringence”
Restrictive cardiomyopathy: amyloid deposition, “amyloid heart disease”
Scenario 7
- 5mo infant
- Dies suddenly while sleeping
- Dense fibrosis of left ventricular endocardium
Endocardial fibroelastosis (unique & rare cause of sudden death in infants)
Scenario 8
- 31yo woman
- Progressive “chest fluttering” for many years
- Signs of right heart failure
- Fatty replacement of right ventricular myocardium
Arrhythmogenic right ventricular cardiomyopathy (fat tissue in RV)
Scenario 9
- 25yo woman
- Congestive heart failure 3 months after delivering her first child
- No other pre-disposing conditions or illnesses
Cardiomyopathy of pregnancy (can occur up to 6 months after delivery)