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22 Cards in this Set

  • Front
  • Back

What is MHS?

Malignant Hyperthermia Susceptibility.


An Autosominal dominant genetic disorder of the ryanodine receptor (RYR1) calcium channels with crises triggered in susceptibility individuals.

Mode of Transmission: Autosomial Dominant Inheritance

Dominant: only one mutated gene of a pair is required to posses a trait.


Therefore, off spring of patient with MH have 50% chance of inheriting the gene.

MHS: Pathophysiology

Trigger agents cause calcium gated channels to open more easily and stay open longer (leaky channel syndrome)




■ Elevated intracellular calcium levels cause sustained muscle activity, muscle cell metabolism leading to enhanced O2 consumption and heat production.

MHS: Early Signs

■ Prolonged masseter spasm


■ Hypercarbia (80mmHg)


■ Unexplained tachypnoea


■ Unexplained tachycardia


■ Cardiac Arrhythmias (ectopic beats)

MHS: Developing Signs

■ Rapid rise in temp - 0.5 degree/15mins


■ Metabolic acidosis


■ Hyperkalaemia


■ Cardiovascular instability


■ Desaturation


■ Generalised muscular rigidity

MHS: Later Signs

■ Coloured urine - due to myoglobinuria


■ Generalised muscle ache (in awake patient)


■ Coagulopathy


■ Cardiac Arrest

What is prolonged Masseter Spasm?

■ True masseter muscle spasm occurs when "jaws of steel" make mouth opening impossible (for at least 2mins)


■ over 50% of patients will subsequently prove to be MH susceptible.

MHS: Non anaesthetic triggers

■ Stenuous exercise


■ Stress


■ Heat Stroke


■ Muscular Dystrophy

MHS: Anaesthetic Triggers

■ Volatile inhalational agents


■ Halo ■ Iso ■ Des ■ Sevo


■ Depolarising muscle relaxants


■ Sux

MH Complications

■ Disseminated Intravascular coagulation (DIC)- widespread activation of the clotting cascade.


■ Pulmonary Oedema


■ Acute Renal Failure


■ Blindness


■ Seizures


■ Paralysis


■ Cardiac Arrest

MHS: Confirmation testing

■ Blood clotting studies - prothrombin time


■ Urine Analysis: presence of myoglobin (protein)

MHS: Diagnosing MHS

■ Muscle Biopsy: caffein hallothane contracture test (CHCT)


■ Genetic Testing - DNA Analysis - only detects 30%

What is the caffien halothane contracture test?

■ Standard for MH diagnostic testing


■ Used to determine patients MH susceptibility

CHCT: Procedure and result

■ Biopsy of Vastus lateralis 3.5cm long, 2cm wide, 1.5cm deep. (under LA)


■ Biopsy is exposed to caffeine of Halothane separately and the rate and force of contraction recorded electronically.


Result:


MH susceptible muscle is more sensitive and contracts with greater sustained force than normal muscle.

MH trolley contents

∆ 24x 20mg vials of Dantrolene


∆ drawing up needles


∆ 2000ml sterile water


∆ 50ml syringes (5-10)


∆ task cards


∆ urinary catheter

MH trolley contents: drugs

8.4% sodium bicarbonate


50% dextrose 50ml


Lignocaine 1%


Amiodarone 300mg

MH trolley contents: cool box in fridge

∆ 2L normal saline


∆ actrapid insulin

MH trolley contents: blood sample tubes

Haematology


•Coagulation profile


•Electrolytes


•Cross match


•Blood gas syringes

MH trolley contents: monitoring

Art line equipment


Central line equipment


Temperature probe

MH resource kit contents

MH introduction


MH crisis initial management


MH task cards


MH education poster (you've only got a few mins)


Recommendations for contents


Guidelines for management

Crisis cards

1) coordinator


2) anaesthetist 1: resus


3) Dantrolene


4) anaesthetic tech


5) anaesthetist 2: lines & investigation


6) cooling


7) surgical team


8) logistics


Anaesthetic tech role

1) layout trolley contents


2) prepare and assist with art line


3) assist with Dantrolene mixing


4) ensure adequate stocks


5) prepare & assist with central line.


6) ensure volatile agent is removed


7) change sida lime when required