pictures: cardiomyopathy

Question 1

Dilated cardiomyopathy definition and pathogensis

Answer 1

1. Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy.
2. Pathogenesis:
   
   1. Thought to be familial in 30-50% of cases (TTN mutations may account for 20% of all cases); usually autosomal dominant.
   2. Alcohol is strongly linked to DCM
   3. Myocarditis
   4. Cardiotoxic drugs/substances: doxorubicin, cobalt, iron overload

Question 2

Dilated cardiomyopathy

Morphology and Presentation

Answer 2

Dilated cardiomyopathy

1. Morphology:
   
   1. dilation of all chambers
   2. mural thrombi are common
   3. functional regurgitation of valves
2. Presentation
   
   1. usually manifests between ages 20-50
   2. progressive CHF → dyspnea, exertional fatigue, ↓ EF arrhythmias
   3. embolism

Question 3

Takotsubo cardiomyopathy

Answer 3

1. Takotsubo cardiomyopathy
   
   1. “Broken heart syndrome”
   2. Excess catecholamines following extreme emotional or psychological stress
      
      1. >90% women, ages 58-75
   3. Symptoms and signs similar to acute myocardial infarction
   4. Apical ballooning of the left ventricle with abnormal wall motion and contractile dysfunction

Question 4

Arrhythmogenic right ventricular cardiomyopathy

Answer 4

1. Arrhythmogenic right ventricular cardiomyopathy
   
   1. Right ventricular failure and arrhythmias
   2. Myocardium of the RIGHT ventricular wall replaced by adipose and fibrosis
   3. Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)

Question 5

Answer 5

1. Arrhythmogenic right ventricular cardiomyopathy
   
   1. Right ventricular failure and arrhythmias
   2. Myocardium of the right ventricular wall replaced by adipose and fibrosis
   3. Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)

Question 6

Answer 6

1. Arrhythmogenic right ventricular cardiomyopathy
   
   1. Right ventricular failure and arrhythmias
   2. Myocardium of the right ventricular wall replaced by adipose and fibrosis
   3. Causes ventricular tachycardia and fibrillation, sudden death Familial (usually autosomal dominant)

Question 7

Answer 7

Hypertrophic cardiomyopathy

1. A genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction
2. Numerous mutations known, involving sarcomeric proteins
3. Most commonly β-myosin heavy chain
4. Morphology: massive myocardial hypertrophy, often with marked septal hypertrophy. Microscopically, myocyte disarray.

White bits in the ventricular wall indicate interstitial fibrosis/scarring

Question 8

Answer 8

1. Hypertrophic cardiomyopathy: image shows interstitial fibrosis (scarring).
   
   1. A genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction
   2. Numerous mutations known, involving sarcomeric proteins
   3. Most commonly β-myosin heavy chain
   4. Morphology: massive myocardial hypertrophy, often with marked septal hypertrophy. Microscopically, myocyte disarray.

Question 9

Answer 9

Amyloid (restrictive cardiomyopathy)

1. Extracellular deposition of proteins which form an insoluble β-pleated sheet.
2. May be systemic (myeloma) or restricted to the heart (usually transthyretin)
3. Certain mutated versions of transthyretin are more amyloidogenic
4. Amyloid can involve different parts of the heart, but when deposits are in the interstitium of the myocardium, a restrictive cardiomyopathy results
5. Image shows pale and pink pattern typical for protein deposition- still needs to be proven with congo red stain (the neon green stain)

Question 10

Answer 10

1. Amyloid (restrictive cardiomyopathy): image shows congo red stain, apple green neon shows protein
   
   1. Extracellular deposition of proteins which form an insoluble β-pleated sheet.
   2. May be systemic (myeloma) or restricted to the heart (usually transthyretin)
   3. Certain mutated versions of transthyretin are more amyloidogenic
   4. Amyloid can involve different parts of the heart, but when deposits are in the interstitium of the myocardium, a restrictive cardiomyopathy results.

Question 12

Restrictive cardiomyopathy

Answer 12

1. Decreased ventricular compliance (increased stiffness), leading to diastolic dysfunction.
   
   1. May be secondary to deposition of material within the wall
   2. (amyloid), or increased fibrosis (radiation).
   3. Ventricles are usually of normal size, but both atria can be enlarged.

Question 13

Answer 13

1. Lymphocytic infiltrate, consistent with viral myocarditis
2. Myocarditis
   
   1. Inflammation of the myocardium, most commonly due to a virus.
   2. Coxsackie A and B viruses are most common
   3. Other infectious causes include
      
      1. rypanosomes (Chagas disease)
      2. Various bacteria and fungi
   4. Noninfectious causes include
      
      1. Immune mediated reactions, including RF, SLE, drug hypersensitivity

Question 14

Question 15

Answer 15

1. Transposition of the great arteries
2. Results in two separate circuits, incompatible with life after birth unless a shunt is present for mixing of blood from the two circuits
   
   1. Approximately one third have a VSD
   2. Two thirds have a patent foramen ovale or PDA
   3. Right ventricle becomes hypertrophic (supports systemic circulation) and the left ventricle atrophies.
   4. Without surgery, patients will die within a few months

Question 16

Answer 16

1. Tetrology of Fallot (classically R–> L shunting)
2. Four cardinal features:
   
   1. VSD
   2. Obstruction of RV outflow tract
   3. Aorta overrides the VSD
   4. RV hypertrophy
3. Heart is enlarged and “boot shaped” because of the right ventricular hypertrophy
4. Clinical severity depends on the degree of subpulmonary stenosis
   
   1. Mild stenosis: L to R shunt.
5. Classic TOF is R to L shunting with cyanosis

Question 17

ASD

Answer 17

1. Atrial septal defect
   
   1. Usually asymptomatic until adulthood
   2. The left-to-right shunting causes volume overload on the right side, which may lead t
      
      1. Pulmonary hypertension
      2. Right heart failure
      3. Paradoxical embolization
      4. May be closed surgically, with normal survival

Question 18

VSD

Answer 18

Ventricular septal defect (heart becomes “dusky red”)

1. Most common form of congenital heart disease
2. Effects depend on size, and presence of other heart defects Many small VSDs close spontaneously
3. Large VSDs may cause significant shunting, leading to
   
   1. Right ventricular hypertrophy
   2. Pulmonary hypertension, which can ultimately reverse flow through the shunt, leading to cyanosis

Question 19

Patent ductus arteriosis

Answer 19

1. Patent ductus arteriosis
   
   1. May fail to close when infants are hypoxic, and/or have defects associated with increased pulmonary vascular pressure (VSD)
      
      1. PDA produces a harsh machinery-like murmur Effect is determined by the shunt’s diameter
      2. Large shunts can increase pulmonary pressure and eventually shunt reversal and cyanosis

Question 20

what are the L–R shunts mentioned in class?

Answer 20

VSDs, ASDs, PDA

Question 21

what are the R–> L shunts mentioned in class?

Answer 21

w and w/out VSD, TOF ??

Question 22

in a congenital R–> L shunt compared to a congenital L–> R shunt

Answer 22

Right-to-left shunts. Cyanosis is seen soon postnatally

Question 23

Answer 23

1. Coarctation of the aorta: infantile form
   
   1. Narrowing of the aorta, generally seen with a PDA (infantile form), or without a PDA (adult form).
   2. Degree of narrowing is variable, with variable clinical effect
   3. Coarctation with PDA manifests at birth: may produce cyanosis in the lower half of the body
2. Coarctation without PDA – usually asymptomatic
   
   1. Hypertension in upper extremities, hypotension in lower extremities
   2. Claudication and cold lower extremities
3. May eventually see concentric LV hypertrophy

Question 24

Answer 24

1. Coarctation of the aorta: adult form
2. Narrowing of the aorta, generally seen with a PDA (infantile form), or without a PDA (adult form).
3. Degree of narrowing is variable, with variable clinical effect
4. Coarctation with PDA manifests at birth: may produce cyanosis in the lower half of the body
5. Coarctation without PDA – usually asymptomatic
   
   1. Hypertension in upper extremities, hypotension in lower extremities
   2. Claudication and cold lower extremities
   3. May eventually see concentric LV hypertrophy

Question 25

RAE vs LAE

Answer 25

* **_RIGHT ATRIAL ENLARGEMENT (RAE)_** * – P wave * – pulmonale peaked P wave with * amplitude **greater than .25 (2.5 mm) mv** in leads **II, III AVF** * amplitude **greater than .1 mv in leads V1 & V2** * – P wave has a _slight rightward axis_ _​_ * **_Left atrial enlargement (LAE)_** * P wave * mitrale “M” signs to P wave * broad, notched P wave duration .11 sec * amplitude of terminal negatively directed portion in V1 to greater than .1 mV or 1 mm deep and .04 sec wide * slight axis of P wave

Question 26

causes of LAE vs RAE

Answer 26

LAE: **MS, MR** RAE: Associated with **TV disease or pul hypertension** 1. – COPD, PE, MS or MR are causes of pul hypertension

Question 27

Answer 27

LAE

Question 28

Romhilt‐Estes Scoring System for LVH

Answer 28

1a. R or S in limb lead: **20mm or more** 1b. S in V1, V2, or V3: **25 mm or more** 1c. R in V5, V6: **30 mm or more** 2. Any ST shift (without digitalis) 3. Typical “strain” ST‐T (with digitalis) 4. LAD ‐ 30◦ or more

Question 29

Answer 29

Left ventricular hypertrophy, arrow points to a "strain pattern"

Question 30

Sokolow Lyon Criteria

Answer 30

Sokolow Lyon Criteria for LVH R in I + S in III \> 25 mm R in AVL \> 11 mm RinV6 \>26mm

Question 31

which leads are used to determine LVH?

Answer 31

1. limb leads (I, II, III) + precordal leads (V1-V6) 2. Limb leads need @ least 1. **R** or **S** wave @ **least 20 mm** 3. PreCo leads need @ least 1. **25 mm** _S wave_ in **V1-2 or V3** 2. **30 mm** _R wave_ in **V5-V6**

Question 32

Answer 32

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

Question 33

Answer 33

LVH with straining pattern, inverted T wave

Question 34

causes of dominant R waves in V1

Answer 34

RVH • Posterior or lateral MI • WPW • Hypertrophic cardiomyopathy • Muscular dystrophy • Normal variant