Propofol infusion syndrome (PRIS) is a rare entity that could lead to profound cardiogenic shock (CS). Mitochondrial toxicity and sympathetic blockade are the mechanisms leading to CS in PRIS.

We present a 22-year-old woman who developed refractory CS due to PRIS after aortic valve replacement surgery secondary to Coxiella infective endocarditis. She was rescued with VA-ECMO (veno-arterial extracorporeal membrane oxygenation) and was discharged 2 months later with no cardiac dysfunction.

PRIS diagnosis is difficult even though propofol is frequently used in critical care units. Abrupt refractory CS in patients with recent use of high doses of propofol (> 4 mg/Kg/h) together with rhabdomyolysis should raise the suspicion. Diagnostic confirmation is based on muscle biopsy and fat enzyme analysis.

Propofol withdrawal and support therapies—including VA-ECMO—are the treatment of choice in severe PRIS. VA-ECMO could increase survival as a bridge to recovery due to reversibility of PRIS.