Marfan aortic regurgitation for urgent appendicectomy24-year-old man, 82 kg, 1.96 m, for urgent laparoscopic appendicectomy

The patient

The examiner asks

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1. Consider

   What are your concerns?

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   • A 24-year-old with Marfan, a 4.8 cm root and severe AR, for urgent laparoscopic appendicectomy. Patient
   • His regurgitant volume rises with a slow heart rate and with any jump in systemic resistance. Disease
   • A diastolic of 48 is his coronary perfusion pressure; if it falls, he gets angina.
   • His root is past 4.5 cm with severe AR, so root surgery is already due.
   • High-arched palate and a jaw that dislocates easily: video laryngoscope ready. Airway
   • Marfan also brings spontaneous pneumothorax, so I keep airway pressures modest. Disease

2. Prepare

   Take me through his risk assessment and preparation.

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   • Urgency: urgent appendicitis, so I optimise in hours; valve surgery can't come first. Stem trigger
   • Active cardiac condition? Yes, severe AR, but compensated: good LV function, no failure. Disease
   • Risk score: intraperitoneal surgery is his only RCRI item, so RCRI 1.
   • Functional capacity: he runs 5 km, well over 4 METs.
   • Decision: proceed now with an AR- and aorta-protecting plan; cardiothoracic team told.
   • Bisoprolol continues, as it slows root growth; losartan is withheld for 24 hours.
   • Arterial line before induction; nitroprusside, clevidipine and noradrenaline drawn up. Crisis

3. Plan

   What are your haemodynamic goals?

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   • Heart rate around 90 or more, so diastole and regurgitation stay short. Disease
   • Systemic resistance a little low; no sudden rises from light anaesthesia or pain.
   • Preload maintained with judicious fluid for his dilated ventricle.
   • Root wall stress kept low: no pressure surges at laryngoscopy, incision or emergence.
   • For hypotension, noradrenaline rather than phenylephrine, which can slow him by reflex.
   • HDU afterwards; cardiothoracic review for root replacement before he leaves hospital. After surgery

4. Crisis

   At laryngoscopy his BP rises to 186/58 and HR to 118. What do you do?

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   • A pressor surge on a 4.8 cm root: wall stress is the danger, more than his rate. (Crisis)
   • Deepen him and pause stimulation until he settles.
   • Nitroprusside 5–25 mcg or clevidipine 100–250 mcg IV to drop the pressure fast.
   • Esmolol 10–25 mg only to trim the rate, never to make him slow.
   • Then a slower re-approach, deeper, with the pressure controlled before the next attempt.

5. Crisis

   In recovery he has tearing chest and back pain; BP 172/40 in the right arm, 128/38 in the left, HR 104. What do you do?

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   • Aortic dissection until proven otherwise: call for help and the cardiothoracic surgeon now. (Crisis)
   • Unequal arm pressures suggest a flap; root involvement makes it type A, a surgical emergency.
   • Urgent CT angiography; TEE in theatre if he is too unstable to move.
   • Lower the force on his aorta: esmolol infusion with clevidipine 2–21 mg/h, titrated.
   • Slow his rate with care; his severe AR worsens if the heart becomes slow.
   • No intra-aortic balloon pump; severe AR is a contraindication.
   • Echo for tamponade and worsening AR on the way to theatre.