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