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Flashcards in Cardiomyopathies/Tumor Deck (22):
1

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)
  • Types
    • Dilated cardiomyopathy
    • Hypertrophic cardiomyopathy
    • Restrictive cardiomyopathy
    • Arrhythmogenic right ventricular cardiomyopathy

2

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%

3

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

4

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

5

Three Things that Cause LV Hypertrophy

  • Hypertrophic cardiomyopathy
  • Hypertension
  • Aortic stenosis

6

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)

7

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

8

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

9

Which one of the following is a constrictive pericarditis?

  • Amyloidosis
  • Suppurative pericarditis
  • Endomyocardial fibroelastosis
  • Hypertrophic cardiomyopathy
  • Post-viral cardiomyopathy

  • Suppurative pericarditis

10

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

11

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

12

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)
  • 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

13

Congestive Heart Failure

  • Most common presentation of "heart failure"
  • Typically refers to the clinical setting of right & left heart failure

14

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)

15

Scenario 2

  • 78yo man
  • Chronic benign HTN
  • Ascites & lower extremity edema

Right heart failure caused by left heart failure

16

Scenario 3

  • 78yo man
  • Chronic emphysema
  • Ascites & lower extremity edema

Right heart failure caused by pulmonary disease (Cor Pulmonale)

17

Scenario 4

  • 56yo
  • Chronic alcoholic
  • Congestive heart failure
  • Large cardiac silhouette on chest x-ray

Dilated cardiomyopathy (most often idiopathic, can have other associations)

18

Scenario 5

  • 28yo
  • Dies suddenly w/ no known pre-existing conditions
  • 950 gram heart w/ massive LVH

Hypertrophic cardiomyopathy (genetic cause)

19

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"

20

Scenario 7

  • 5mo infant
  • Dies suddenly while sleeping
  • Dense fibrosis of left ventricular endocardium

Endocardial fibroelastosis (unique & rare cause of sudden death in infants)

21

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)

22

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)