Cardiomyopathy

Question 0

Risk factors for dilative cardiomyopathy

Answer 0

MAP GenetHemo

Myocarditis
Acromegaly - myxoedema heart + severe HYPOthyro
Postpartum state

Solvents GLUE SNIFFER
Alcohol
Idiopathic
Drugs – cocaine doxorubicin

Genetic (25–35%) + Haemochromatosis

Question 1

What are the acronyms for the CARDIOMYOPATHies

Which ones are systolic dysfunctions and which ones are diastolic dysfunctions?

Answer 1

Dilated cardiomyopathy - MAP SAID GenetHemo!!!
Hypertrophic CM - NOTCH2
Restrictive Cardiomyopathy - PALESH

DRS DiHy

Systolic dysf = Dilated + Restrictive cardiomyopathy
Diastolic dysfunction = Hypertrophic cardiomyopathy

Question 2

Pathophysiology of cardiomyopathy

Treatment?

Answer 2

Global enlargement = stretch heart –>

Regurgitation - Mitral/tricuspid - stretch valves
Arrhythmia - stretch conduction system
Dysfunction systolic = congestive biventricular HF

Heart transplant, it’s inhibitors, beta-blockers

Question 3

Who does hypertrophic cardiomyopathy happen in?

Types of HCM?

Answer 3

Young athletes

Familial – 60–70% > sporadic

Question 4

Explain the genetics behind familial form HCM

Answer 4

Missense mutation

AA substitute in contractile proteins of SARCOMERE -

Beta myosin binding Prot C

Question 5

Explain the pathophysiology of HCM

Answer 5

Hypertrophy of myocardium – >

Stretch conduction system = arrhythmia fatal death
+ IVS growth >LV growth –>

• LV = non-compliant = diastolic dysfunction
• IVS hypertrophy obstructs LV outflow tract below aortic valve – >functional aortic stenosis
  Syncope
  Angina
  Microangiopathic haemolytic anemia

Question 6

What do you see histologically for HCM

What’s the moment?

Answer 6

Myofibrillar disarray

Crescendo decrescendo systolic injection murmur

Decrease with handgrip + squat

Increase with Valsalva

Question 7

Risk factors for restrictive cardiomyopathy

Answer 7

Restricted from filling – cannot expand = decreased compliance

Postradiation fibrosis
Amyloidosis phil’s heart – >difficult to expand

Loeffler syndrome Endomyocardial fibrosis + eosinophilic infiltrate – >can’t expand

Endocardial fibroelastosis – thick fibroelastic tissue @endocardium of kids – >can’t stretch

Sarcoidosis – granuloma inside heart wall – >can’t expand

Haemochromatosis – Iron @ heart wall

Question 8

Explain how the risk factors cause the pathophysiology for restrictive cardiomyopathy

Answer 8

Can’t fill – >RCM –> blood back up behind heart – >

Decreased ECG voltage despite thick myocardium
Decreased QRS amplitudes

Question 9

Explain concentric hypertrophy

Explain eccentric hypertrophy

Answer 9

Increased afterload = concentric thickening
Sarcomeres duplicate parallel to long axis of cells
– >individual fibres thicken

Increased preload = eccentric thickening
Sarcomeres duplicate in series
Individual fibers increasing length + with

Question 10

Explain how concentrically thickened heart can cause angina

Answer 10

Endocardium receive the least amount of blood

If concentrically thick - subendocardial tissue receives low oxygen levels