PATH: 10-2 Cardiomyopathy Flashcards
A: Define Cardiomyopathy
B: What are the 3 patterns and which is MOST COMMON / which is least common?
A: Heart disease resulting from a primary abnormality in the myocardium itself
B: Dilated (most common) / Hypertrophic / Restrictive (least common)
[Dilated Cardiomyopathy] occurs from progressive _____ dysfunction and has MULTIPLE Etiologies:
What are they? (7)
[Dilated Cardiomyopathy] occurs from progressive systolic dysfunction and has MULTIPLE Etiologies:
“the PIG PAID for Dilated Cardiomyopathy”
- Post Myocarditis from [Coxsackie B Enterovirus Infection]
- Alcoholic Cardiomyopathy from long term EtOH usage (direct toxicity vs. nutritional deficiency)
- [Doxorubicin and Daunarubicin Chemotherapy]= Cardiotoxicity is dose-dependent
- Peripartum - (late in pregnancy vs. 5 mo. post partum)
- Genetic= affects cytoskeleton
- Iron Overload: [Hereditary Hemochromatosis] or [Multiple Blood Transfusion Hemosiderosis] = Iron accumulates and interferes with metal-dependent enzyme system in myoctyes
- Idiopathic
Tx for Dilated Cardiomyopathy (2)
Tx for Dilated Cardiomyopathy
1) Medical management of Systolic Heart Failure (fluid overload / emboli / arrhythmias)
2) Heart Transplant
A: Hypertrophic cardiomyopathy is…
Caused by ______ mutation in 1 of at least __ genes encoding cardiac ______ protein. Mutations in the ______ chain are most common and Most cases are familial
B: Mode of Inheritance
C: Signs (2)
D: Histology (2)
A: 100% CAUSED BY BAD GENETICS!
Caused by missense mutation in 1 of at least 12 genes encoding for [cardiac sarcomere protein]. Mutations in myosin heavy chain are most common and Most cases are familial
B: Autosomal dominant with variable expression
C:
- Septum Bulging into L ventricle –> [Banana shaped L ventricle]
- Left ventricle outflow tract obstruction 2º to systolic anterior motion of mitral valve and mitral-septal contact
D:
*Disorganized, haphazardly arranged myocytes
*myocytes are extensively hypertrophied
Hypertrophic cardiomyopathy is AKA what 2 other names?
iHSS - Idiopathic hypertrophic subaortic stenosis
and
HOCM - Hypertrophic obstructive cardiomyopathy
Clinical Features of Hypertrophic Cardiomyopathy (7)
1) Cardiac Insufficiency 2º to [Diastolic Heart Failure]
2) [Systolic Ejection murmor] tht INC in loudness during maneuvers tht DEC preload (i.e. squatting to standing / valsalva / vasoDilators)
3) [Systolic Ejection murmor] tht DEC in loudness during maneuvers tht INC preload (i.e. passive elevation of legs / [standing–>squating])
4) Angina and SOB
5) Arrhythmias
6) Mural Thrombi
7) Sudden Death
3 Tx for Hypertrophic Cardiomyopathy (2)
Tx
- [Pharmacologic ventricular relaxation] to enhance diastolic filling (ie beta blockers, [non-dihydroyridine calcium channel blockers])
- Surgical septal excision (myectomy)
A: Define Restrictive Cardiomyopathy
B: Gross findings (2)
C: Etiologies (8)
A: Primary decrease in ventricular compliance –> impaired ventricular filling during diastole–> Diastolic CHF.
**Systolic function is unaffected**
B: Histology:
- Ventricle and left ventricle cavity are generally normal in size
- myocardium is firm and stiff impeding expansion of left
ventricle cavity
C: Etiologies: RAMILIES
- Radiation Fibrosis
- Amyloidosis (heterogenous misfolded proteins)
- Sarcoidosis= [Noncaseating granuloma formation] in multiple organs 2º to [CD4 Helper T] attack on unidentified antigen
- Metstatic Tumor
- Inborn metabolism errors
- Endomyocardial fibrosis= [African/Tropic children] develop dense endocardial fibrosis
- Loeffler Endomyocarditis= endomyocardial fibrosis w/ [Peripheral blood eosinophilia and infiltrate]
- Idiopathic
A: Amyloidosis is 1 of the etiologies for _________ and results from pathologic ______ substance deposited in the myocardial interstitium (__ forms of Amyloid protein)
B: Structural Similiarities between Amyloid Proteins (3)
C: Sx
A: Amyloidosis is 1 etiology for Restrictive Cardiomyopathy and results from pathologic proteinaceous substance deposited in the myocardial interstitium (15 forms of Amyloid protein)
B: Structural Similiarities:
- interlacing bundles of parallel arrays of fibrils;
- Protein in amyloid fibrils contains large amt of crosspleated sheet structure
- All have affinity for congo-red stain resulting in apple-green birefringence under polarized light
C: Sx depends on organ affected by protein deposits
Most Common causes of Myocarditis are ______!
List the specific etiologies within each group
A: Viruses:(3)
B: Bacteria:(2)
C: Parasites: (3)
D: Immune Mediated: (3)
E: Unknown: (2)
Most Common causes of Myocarditis are Infection!
List the specific etiologies within each group
A: Viruses: [(Coxsakie A and B) detected via PCR in myocardium biopsy] / Influenza / HIV
B: Bacteria: Borrelia / [Corynebacterium Diphtheriae]
C: Parasites:
-Trypanasoma cruzi - Chagas Dz (Endemic South America)
- Trichinosis
- Toxoplasmosis
D: Immune Mediated:
- Hypersensitivty to drugs
- Rheumatic Fever
- Systemic Lupus Erythematosus
E: Unknown: Sarcoidosis / [Giant Cell myocarditis]
Myocarditis
A: Gross Findings (2)
B: Microscopic Findings (2)
C: Sx (5)
A: Gross – Normal or flabby heart with mottled myocardium
B: Microscopic:
*Interstitial inflammatory infiltrate (often lymphocytic but depends on etiology) +/- progressive fibrosis
*focal myocyte necrosis
C: Sx
- Asymptomatic
- Acute CHF vs. Progressed Dilated Cardiomyopathy
- Nonspecific sx (fatigue / feve)
- Sudden Cardiac Death 2º to Arrhythmias
- Can “mimic” sx of MI
A: Normally there is ______ of pericardial fluid in pericardial sac
B: What type of Fluid accumulates (3)? Is this acute or chronic condition?
C: Etiologies of moderate to large pericardial effusions (9)
D: Diagnostic
A: Normally there is 30-50cc of pericardial fluid in pericardial sac
B: Fluid (serous, purulent, or bloody) may accumulate in pericardial sac for many reasons and can be acute or chronic.
C: “Pericardial EffUsions require CATNIP IV”
Etiologies of moderate to large pericardial effusions include:
- neoplasms,
- uremia 2º to renal failure
- iatrogenic,
- post-acute MI
- viral,
- collagen vascular diseases,
- tuberculosis,
- idiopathic
- asymptomatic If there’s slow accumulation less than 500cc
D: Diagnostic = [globular heart shadow] on CXR
In Pericardial EffUsions, if fluid ______ accumulates greater than ____ cc —> Symptoms:
What are they (5)
In Pericardial EffUsions, if fluid rapidly accumulates greater than 500 cc —> Symptoms:
- “Cardiac tamponade” may develop
(fluid in pericardial sac compresses atria/ vena cavae, ventricles, and restricts cardiac filling) - hypotension,
- increased JVD
- distant heart sounds,
- pulsus paradoxus
A: Describe Pericarditis
B: Etiologies (7)
C: Which autoimmune syndrome is it associated with?
A: Pericarditis is pericardial inflammation that occurs in response to cardiac/thoracic/systemic process.
B: “Pericarditis gave HIM A UTI”
- Infection/Viruses ([Coxsackie Virus A and B] / echovirus/adenovirus are most common cause of pericarditis)
- Acute MI
- Immune mediated
- [HMLB Neoplasm] - (Lung/Breast/Hodgkin’s/Mesothelioma)
- Trauma
- Mediastinal Radiation
- Uremia
C: Associated with Dressler Syndrome = autoimmune phenomenon –> pericarditis SEVERAL WEEKS post MI
A: What specifically causes Fibrinous/Serofibrinous Pericarditis? (5)
B: Findings (3)
A: “Pericarditis gave HIM A UTI”
- Fibrinous/Serofibrinous =* I / A / U / T / I
- Acute MI
Uremia
Infection/Rhematiac Fever
Immune mediated (Lupus and [Dressler’s syndrome])
Trauma
B: Findings
- Dry/rough Pericardial surface with thick yellow fluid on surface
- Presence of inflammatory cells / blood / fibrin
- Friction rub
A: What is this
B: What Dz is this seen in?
Disorganized, haphazardly arranged myocytes in [Interstitial Fibrosis]
B: Seen in Hypertrophic Cardiomyopathy
Cardiac amyloid in a Congo red stain
(will result in apple-green birefringence under polarized light)
