Severe PAH on epoprostenol for hysteroscopy46-year-old woman, 58 kg, booked as a day case for hysteroscopy under sedation for heavy menstrual bleeding

The patient

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1. Consider

   What are your concerns?

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   • A 46-year-old with severe PAH on IV epoprostenol, booked for day-case hysteroscopy under sedation. Patient
   • Class III, mean PA pressure 52 and PVR 9: firmly in the severe group. Disease
   • Moderate RV dysfunction leaves her no reserve for any rise in afterload.
   • A short procedure, but sedation alone can cause hypoventilation and hypercarbia in her. Surgery

2. Prepare

   Is this booking appropriate, and how will you prepare?

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   • Urgency: elective day-case hysteroscopy, so there is time to move her somewhere safer. Stem trigger
   • Active cardiac condition? Yes: severe PAH, mean PA pressure 52 and PVR 9, with RV dysfunction. Disease
   • Risk score: no RCRI items, so RCRI 0; the score misses her pulmonary hypertension.
   • Functional capacity: class III, so poor.
   • Decision: not a day case; a specialist PH centre with an ICU bed.
   • Epoprostenol runs on its own line; it is never paused, flushed or disconnected.
   • Sildenafil and ambrisentan continue; arterial line before sedation; ECMO cover agreed with her PH team.

3. Plan

   What is your anaesthetic plan?

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   • Carefully titrated sedation with capnography, oxygen on throughout, so her CO2 never climbs. Disease
   • Good analgesia and adequate depth; pain and light anaesthesia raise her PVR.
   • Analgesia with fentanyl in small 25 mcg steps, watching her breathing closely.
   • Her systemic pressure is kept up, because her RV is perfused from it.
   • Every line de-aired; even small bubbles are dangerous for her.
   • Noradrenaline, vasopressin, milrinone and inhaled nitric oxide prepared before she arrives. Crisis
   • Overnight in a monitored bed with the PH team reviewing her. After surgery

4. Crisis

   Ten minutes in, after propofol and 50 mcg fentanyl, SpO2 is 84%, RR 8 with a shallow capnograph trace, BP 96/60. What do you do?

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   • She is hypoventilating: stop the propofol, jaw thrust, 100% oxygen. (Crisis)
   • Assist her breathing by bag and mask to bring the CO2 down fast.
   • Naloxone 40 mcg IV every 2 minutes, titrated to her breathing.
   • Check that the epoprostenol pump is still running into her line.
   • Rising CO2 and acidosis now can precipitate RV failure.

5. Crisis

   Despite ventilation, BP falls to 70/45, HR 118, and echo shows a dilated RV bowing the septum. What do you do?

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   • Acute RV failure: call for help, the PH team and ICU. (Crisis)
   • Noradrenaline at 0.1–1 mcg/kg/min to restore her systemic pressure.
   • Or vasopressin at 0.01–0.05 units/min; it barely raises PVR.
   • Inhaled nitric oxide at 10–40 ppm to cut her PVR; it spares systemic pressure.
   • Milrinone for RV contractility once systemic pressure is held.
   • Fluid only if the RV looks underfilled; hers is distended and pushing on the LV.
   • If she keeps deteriorating, VA ECMO is the rescue.