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Cardiology > Cholesterol, congenital, channelopathies > Flashcards

Cholesterol, congenital, channelopathies Flashcards

1
Q

High intensity statins & dose

A
  • Atorvastatin 80mg / 40mg
  • Rosuvastatin 40mg / 20mg
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2
Q

LDL target

A

<1.8 mmolL

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3
Q

Familial hypercholesterolaemia - gene mutations

A
  • LDLR
  • apoB
  • PCSK9
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4
Q

Familial hypercholesterolaemia - most specific fx

A
  • Tendon xanthoma (#1)
  • corneal arcus

Kid <18 but >95th percentile

LDL >8.5

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5
Q

PSCK9 - names & effect when combined w/ Ezetimibe & statin (in FH)

A
  • Alirocumab
  • Evolucumab
  • 85% reduction in LDL w/ combo
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6
Q

HCM - most common genetic mutations & what do they code

A

MYH7 (MYBPC3)

Encode myosin production & activity

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7
Q

Aortopathy - common genetic mutation

A

FBN-1 in Marfans

(also Ehlers Danlos (COL), Loeys Dietz (TGF & SMAD)

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8
Q

Long QT syndrome - types & channelopathies & mutations

A

AD - 80% have identifiable mutations (some crossover in 450-500msc range)

  • Type 1 & 2: K channels (90%) - loss fxn (short QT is gain of fxn in gene)
  • Type 3: Na channel (SCN5A) - gain of fxn (Brugada is gain of fxn in gene)
  1. LQT1 (50%): swimming
  2. LQT2 (40%): other exercise/emotion (eg fright)
  3. LQT3 (10%): asleep/at rest - bradycardic
    * Auditory/post partum - catecholamines are triggers*
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9
Q

Long QT - Rx

A
  • *B blockers**
  • start straight away (most effective in QT1)

ICD: only if VT/VF, syncope or high risk

Screen FHx or high suspicion

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10
Q

CPVT: Catecholamine polymorphic VT - Fx, pathophys

A

Exercise induced (catecholamine) polymorphic VT (w/ normal QT)

Ryanodine receptor (mediates Ca release from SR)

AD inherited (RyR or CASQ2)

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11
Q

CPVT: Catecholamine polymorphic VT - Rx

A
  1. Beta blockers
  2. ICD if high risk
  • 2nd line:
  • Flecainide
  • Verapamil
  • (sympathetic denervation)
  • No strenuous exercise unless ext defib available
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12
Q

Brugada - Fx

A

AD - SCN5a mutation (loss of fxn - Na channel)

V1-V3 downsloping ST w/ TWI
+ QRS can be wide like RBBB

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13
Q

Brugada - Rx

A

ICD (if sx/high risk, asx ECG - just monitor if low risk)

Avoid drugs

  • Na channel blockers - Flecainide, Amiodarone, Propranolol
  • CCB - Verapamil, Diltiazem
  • ETOH/Cocaine
  • Phenytoin, Lidocaine
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14
Q

Most common cardiac abnormality in Trisomy 21

A

Atrioventricular septal defect (AVSD)

VSD

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15
Q

Cardiac abN in Di George (22q) deletion

A

Tetralogy of Fallot

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16
Q

Which cardiac chamber develops vol OL in VSD

A

LV

(as each contraction, some flows through VSD, so needs to dilate/increase volume to maintain CO)

17
Q

Which is the only shunt causing R) side volume OL

A

ASD

18
Q

Associations w/ bicuspid AV

A 
Aortic coarctation (interrupted aortic arch - claudication etc)
50% have bicuspid AV

Berry aneurysms (10%) - screen CTA

19
Q

Most common cyanotic congenital heart defect

A

Tetralogy of Fallot

Anterior deviation on infundicular septum - leads to

  1. Unrestrictive VSD
  2. PS (usu RVOTO)
  3. Overriding aorta (moves to right)
  4. RVH

Rx: surgery (immediate if sx) - often have PR after

20
Q

Most common ASD type

A

Ostium secundum (Fossa ovalis)

(Ostirum primum is partial AVSD - down syndrome etc)

21
Q

Most common congenital HD

A

PFO doesn’t close in 25% (in RA)

But - congenital bicuspid AV is most common (1% popn)

Assoc w/ premature AS + AR ?ASD.VSD

22
Q

AF & stent - what Rx

A

DAPT is fine (and should be P2Y12 like Clopidogrel, not aspirin)

After 12 months - single agent AC

If needing triple therapy initially (w/ aspirin) - don’t continue beyond first month

23
Q

Cardiac amyloid - types

A

1) AL (light chains)
2) Wild type ATTR (transthyretin) - “senile”

  • AA is rare & doesn’t cause cardiac amyloid
  • Inherited ATTR mutation amyloid DOESN’T CAUSE
  • AL is worst pronosis
  • Wild type has Tafamidus Rx to stabilies the transthyretin & slow the deposition which improves ACM & QOL
24
Q

Ix cardiac amyloid

A

Bloods: SFLC, SPEP
BMAT
Mass spec - gold std
Nuclear HDL amyloid scan helpful

25
Q

Amyloidosis - type & agent

A

Tafamidus - for wildtype Transthyretin
Others - chemo, SCTx (Bortezomib)
HF: A, B, C poorly tolerated- sml cavity LV, needs HR fxn - can try fruse & spiro (not if post hypoTN)

26
Q

Amyloid - appearance micro/histo

A

Apple-green bifringence w/ congo red staining on polarized light microscopy
Highly ordered fibrils of low molecular wt - circulate as plasma

27
Q

Statins - MoA

A

↓ hepatocyte production of cholesterol by inhibiting HMG-COA reductase

Thereby ↑ LDL receptor synthesis → ↑ LDL clearance

28
Q

PCSK9 - MoA

A

PCSK9 regulate LDL receptors in the cell surface (ie. Increase degradation)

PCSK9 inhibitors block this and increase LDL receptor levels more LDL taken up from the circulation

29
Q

Ezetimibe - MoA

Mipomersen - MoA

A

Ezetimibe: Azetidinone cholesterol absorption inhibitor

  • acts by blocking NPC1L1
  • a key brush border transport protein

Mipomersen: Inihbits synthesis of apoB100 & LDL

30
Q

Type 3, 4, 5 MI

A

3: SCD w/ sx suggestive of ischaemia or MI found on autopsy (no biomarkers)
4: (a) PCI, (b) stent thrombosis

cTn >5x ULN + evidence new ischaemia (ECG ∆, q wave, imaging, CAG)

5: CABG

cTn >10xULN + new evidence of ischaemia/flow limiting complication

Cardiology (7 decks)

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