Acute intermittent porphyria (AIP) is a disorder of the heme biosynthesis pathway of a specific inherited enzymatic defect. Many different environmental factors such as drugs, calorie restriction, infection etc., often play a key role in triggering a porphiric acute attack. We describe a 50-year-old woman with AIP who underwent aortic valve replacement due to acute infective endocarditis and review the perioperative management of these patients.

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