pictures: cardiomyopathy Flashcards
1
Q
Dilated cardiomyopathy definition and pathogensis
A
- Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy.
- Pathogenesis:
- Thought to be familial in 30-50% of cases (TTN mutations may account for 20% of all cases); usually autosomal dominant.
- Alcohol is strongly linked to DCM
- Myocarditis
- Cardiotoxic drugs/substances: doxorubicin, cobalt, iron overload
2
Q
Dilated cardiomyopathy
Morphology and Presentation
A
Dilated cardiomyopathy
- Morphology:
- dilation of all chambers
- mural thrombi are common
- functional regurgitation of valves
- Presentation
- usually manifests between ages 20-50
- progressive CHF → dyspnea, exertional fatigue, ↓ EF arrhythmias
- embolism
3
Q
Takotsubo cardiomyopathy
A
- Takotsubo cardiomyopathy
- “Broken heart syndrome”
- Excess catecholamines following extreme emotional or psychological stress
- >90% women, ages 58-75
- Symptoms and signs similar to acute myocardial infarction
- Apical ballooning of the left ventricle with abnormal wall motion and contractile dysfunction
4
Q
Arrhythmogenic right ventricular cardiomyopathy
A
- Arrhythmogenic right ventricular cardiomyopathy
- Right ventricular failure and arrhythmias
- Myocardium of the RIGHT ventricular wall replaced by adipose and fibrosis
- Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)
5
Q
A
- Arrhythmogenic right ventricular cardiomyopathy
- Right ventricular failure and arrhythmias
- Myocardium of the right ventricular wall replaced by adipose and fibrosis
- Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)
6
Q
A
- Arrhythmogenic right ventricular cardiomyopathy
- Right ventricular failure and arrhythmias
- Myocardium of the right ventricular wall replaced by adipose and fibrosis
- Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)
7
Q
A
Hypertrophic cardiomyopathy
- A genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction
- Numerous mutations known, involving sarcomeric proteins
- Most commonly β-myosin heavy chain
- Morphology: massive myocardial hypertrophy, often with marked septal hypertrophy. Microscopically, myocyte disarray.
White bits in the ventricular wall indicate interstitial fibrosis/scarring
8
Q
A
- Hypertrophic cardiomyopathy: image shows interstitial fibrosis (scarring).
- A genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction
- Numerous mutations known, involving sarcomeric proteins
- Most commonly β-myosin heavy chain
- Morphology: massive myocardial hypertrophy, often with marked septal hypertrophy. Microscopically, myocyte disarray.
9
Q
A
Amyloid (restrictive cardiomyopathy)
- Extracellular deposition of proteins which form an insoluble β-pleated sheet.
- May be systemic (myeloma) or restricted to the heart (usually transthyretin)
- Certain mutated versions of transthyretin are more amyloidogenic
- Amyloid can involve different parts of the heart, but when deposits are in the interstitium of the myocardium, a restrictive cardiomyopathy results
- Image shows pale and pink pattern typical for protein deposition- still needs to be proven with congo red stain (the neon green stain)
10
Q
A
- Amyloid (restrictive cardiomyopathy): image shows congo red stain, apple green neon shows protein
- Extracellular deposition of proteins which form an insoluble β-pleated sheet.
- May be systemic (myeloma) or restricted to the heart (usually transthyretin)
- Certain mutated versions of transthyretin are more amyloidogenic
- Amyloid can involve different parts of the heart, but when deposits are in the interstitium of the myocardium, a restrictive cardiomyopathy results.
11
Q
A
12
Q
Restrictive cardiomyopathy
A
- Decreased ventricular compliance (increased stiffness), leading to diastolic dysfunction.
- May be secondary to deposition of material within the wall
- (amyloid), or increased fibrosis (radiation).
- Ventricles are usually of normal size, but both atria can be enlarged.
13
Q
A
- Lymphocytic infiltrate, consistent with viral myocarditis
- Myocarditis
- Inflammation of the myocardium, most commonly due to a virus.
- Coxsackie A and B viruses are most common
- Other infectious causes include
- rypanosomes (Chagas disease)
- Various bacteria and fungi
- Noninfectious causes include
- Immune mediated reactions, including RF, SLE, drug hypersensitivity
14
Q
A
15
Q
A
- Transposition of the great arteries
- Results in two separate circuits, incompatible with life after birth unless a shunt is present for mixing of blood from the two circuits
- Approximately one third have a VSD
- Two thirds have a patent foramen ovale or PDA
- Right ventricle becomes hypertrophic (supports systemic circulation) and the left ventricle atrophies.
- Without surgery, patients will die within a few months
16
Q
A
- Tetrology of Fallot (classically R–> L shunting)
- Four cardinal features:
- VSD
- Obstruction of RV outflow tract
- Aorta overrides the VSD
- RV hypertrophy
- Heart is enlarged and “boot shaped” because of the right ventricular hypertrophy
- Clinical severity depends on the degree of subpulmonary stenosis
- Mild stenosis: L to R shunt.
- Classic TOF is R to L shunting with cyanosis
23
Q
A
- Coarctation of the aorta: infantile form
- Narrowing of the aorta, generally seen with a PDA (infantile form), or without a PDA (adult form).
- Degree of narrowing is variable, with variable clinical effect
- Coarctation with PDA manifests at birth: may produce cyanosis in the lower half of the body
- Coarctation without PDA – usually asymptomatic
- Hypertension in upper extremities, hypotension in lower extremities
- Claudication and cold lower extremities
- May eventually see concentric LV hypertrophy
24
Q
A
- Coarctation of the aorta: adult form
- Narrowing of the aorta, generally seen with a PDA (infantile form), or without a PDA (adult form).
- Degree of narrowing is variable, with variable clinical effect
- Coarctation with PDA manifests at birth: may produce cyanosis in the lower half of the body
-
Coarctation without PDA – usually asymptomatic
- Hypertension in upper extremities, hypotension in lower extremities
- Claudication and cold lower extremities
- May eventually see concentric LV hypertrophy
27
Q
A
LAE
29
Q
A
Left ventricular hypertrophy, arrow points to a “strain pattern”
32
Q
A
LVH: leads 1-3 R or S wave 20 mm or more, precordal leads V1-3 S wave 25 mm or more, V5-6 R wave 30 mm or more
33
Q
A
LVH with straining pattern, inverted T wave
