Cardiac amyloidosis for carpal tunnel release71-year-old man, 66 kg, for elective bilateral carpal tunnel release

The patient

The examiner asks

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1. Consider

   What are your considerations?

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   • A 71-year-old with cardiac AL amyloidosis and nephrotic syndrome, for elective bilateral carpal tunnel release. Patient
   • At 71 with an infiltrated heart he has little reserve; I'd discuss that risk openly with him.
   • Restrictive physiology: stiff ventricles, a fixed stroke volume, so he lives on preload and heart rate. Disease
   • Amyloid conduction disease can progress to heart block and sudden death; pacing must be available.
   • He drops 18 mmHg on standing, from autonomic involvement and his diuretic.
   • His big, stiff tongue can obstruct his upper airway under sedation. Airway
   • Amyloid can trap factor X, so he may bleed despite a normal INR. Disease

2. Prepare

   Take me through his cardiac risk assessment.

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   • I'd use the ACC/AHA stepwise approach, applied to him. Disease
   • Urgency: elective, low-risk hand surgery, so there's time to assess him. Stem trigger
   • Active cardiac condition? His new ankle swelling may be decompensated failure; if so, I defer surgery. Disease
   • Risk score: heart failure gives an RCRI of 1; creatinine 1.5 is under 2.
   • Functional capacity: breathless after one flight of stairs puts him under 4 METs.
   • Decision: if his failure is compensated, proceed under regional, given the low surgical risk.

3. Prepare

   How do you prepare him?

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   • Echo: thick walls, large atria, a speckled ventricle and poor relaxation confirm the restrictive picture. Disease
   • ECG for progression of his heart block; transcutaneous pacing pads available.
   • Factor X level and coagulation checked before any deeper block.
   • No further diuresis before surgery: his heart needs its filling.
   • Creatinine 1.5 with nephrotic syndrome: I avoid renally cleared drugs where I can.
   • Cardiology and haematology informed; the risk explained to him and documented. Patient

4. Plan

   What is your anaesthetic plan?

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   • Regional over deep sedation: surgeon's local infiltration or distal blocks, one hand at a time. Surgery
   • Sedation minimal and titrated; no boluses into his fixed-output heart. Disease
   • Sinus rhythm and heart rate protected; I avoid bradycardia.
   • Preload maintained: no vasodilators, fluid and phenylephrine drawn up.
   • Pacing pads on throughout, with the defibrillator next to him.

5. Crisis

   A colleague gives midazolam 2 mg and propofol 40 mg for the second hand. BP falls to 50/30; the monitor then shows sinus rhythm at 60, but there's no pulse. What do you do?

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   • PEA arrest: adult arrest algorithm, non-shockable side. Crisis
   • Call for help; CPR at a rate of 100–120, with oxygen and airway.
   • Adrenaline 1 mg IV straight away, repeated every 3–5 minutes.
   • Rhythm check every 2 minutes; shock only if it becomes shockable.
   • His likely cause: sedative vasodilation in a preload-dependent heart, plus diuretic hypovolaemia, so a fluid bolus.
   • Also hypoxia, if his tongue obstructed his airway under sedation.
   • After ROSC: ICU, and the second hand abandoned. After surgery