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Eisenmenger syndrome for urgent wrist fixation31-year-old woman, 54 kg, for urgent fixation of a displaced distal radius fracture

From memory: the diagnosis and the notes are hidden. Answer each question aloud, then press Show.

Know this first

  • Her PVR is fixed; a fall in SVR drives more right-to-left flow.
  • Hold her systemic resistance at, or a little above, its preoperative level.
  • A sudden saturation drop with unchanged ventilation can be the first sign of falling SVR.
  • Any air in a line can cross to her systemic circulation.

The patient

31-year-old woman, 54 kg, for urgent fixation of a displaced distal radius fracture. Eisenmenger syndrome from an unrepaired large VSD; cyanosed and clubbed; haematocrit 62%; breathless after one flight of stairs. Drugs: sildenafil, ferrous sulfate. Vitals: BP 112/70, HR 84, SpO2 84% on air (her usual), RR 18.

The examiner asks

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  1. Consider

    What are your concerns?

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    • A 31-year-old with Eisenmenger from an unrepaired VSD, for urgent wrist fixation. Patient
    • Her PVR is fixed, so any fall in systemic resistance sends more blood right to left. Disease
    • Her SpO2 of 84% is her baseline; I judge every change against that.
    • Haematocrit 62% means erythrocytosis, with hyperviscosity and thrombosis risk.
    • Hypoxaemia or high airway pressures would raise her PVR further.
    • Wrist surgery can be done under a plexus block, sparing her airway and ventilation. Surgery
  2. Prepare

    Take me through her risk assessment and preparation.

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    • Urgency: urgent fracture, so hours for the right team, not weeks. Stem trigger
    • Active cardiac condition? Yes: Eisenmenger is severe pulmonary hypertension, and it can't be optimised. Disease
    • Risk score: no RCRI items, so RCRI 0.
    • Functional capacity: breathless after one flight, under 4 METs.
    • Decision: proceed, but in a pulmonary hypertension centre with her PH team.
    • Sildenafil continues; venesection only if she has hyperviscosity symptoms.
    • Air filters on every line; even small bubbles can reach her brain.
  3. Plan

    What is your anaesthetic plan?

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    • Axillary block: it covers forearm and hand, and spares her phrenic nerve. Disease
    • Light sedation only, with capnography, so her CO2 doesn't climb.
    • Oxygen on throughout; it can help her hypoxaemia.
    • Arterial line; SVR kept at or just above her starting level.
    • Phenylephrine drawn up and noradrenaline ready before the block goes in. Crisis
    • Monitored bed overnight with her PH team; never a same-day discharge. After surgery
  4. Crisis

    After midazolam and fentanyl, SpO2 falls to 68%, BP to 84/50, RR 10. What do you do?

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    • Two problems together: vasodilation widening her shunt, and hypoventilation raising her PVR. (Crisis)
    • Stop sedation, 100% oxygen, jaw thrust, and assist her breathing to clear CO2.
    • Phenylephrine 50–100 mcg IV to restore her systemic resistance.
    • Pressure still falling: start noradrenaline, 0.05–0.3 mcg/kg/min.
    • Fentanyl slowing her breathing: naloxone 40 mcg IV every 2 minutes.
    • My target is her baseline saturation of 84%, not 100%.
  5. Crisis

    In recovery she cannot lift her left arm and her speech is slurred. What do you do?

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    • Stroke until proven otherwise: paradoxical embolism or a clot from thick blood. Crisis
    • Stroke team now, and emergent brain imaging.
    • Recent surgery doesn't absolutely bar thrombolysis; clot retrieval if bleeding risk is high.
    • Meanwhile keep her pressure and saturation at her baseline.
    • Stroke unit or ICU, with her PH team involved. After surgery

Fatal mistakes

  • Leaving systemic vasodilation untreated.
  • Air in any of her intravenous lines.
  • Deep sedation with hypercarbia.
  • Sending her home the same day.

Checked against: Stoelting 8e · AHA/ACC 2024 · Faust · SNACC consensus statement: perioperative care of patients at high risk for stroke during or after non-cardiac, non-neurologic surgery (2014)

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