Harlequin Syndrome during Venoarterial Extracorporeal Membrane Oxygenation

Introduction

A 32-year-old woman had intraoperative cardiac arrest secondary to a pulmonary embolism. After 30 minutes of cardiopulmonary resuscitation, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) via the femoral artery and vein. Within 72 hours, her native cardiac function improved with increasing pulse pressure, but she paradoxically developed progressive hypoxia isolated to the right face and upper extremity. Axial and oblique maximum intensity projection images from chest CT angiography demonstrate antegrade flow in the ascending aorta and right-sided great vessels via native cardiac ejection and retrograde flow in the descending aorta via the femoral ECMO cannula with preferential flow to the left subclavian artery (Figure).

Download as PowerPoint

Download as PowerPoint

When cardiac recovery occurs before the resolution of lung injury, insufficiently oxygenated pulmonary venous return can be ejected, leading to selective hypoxia of the coronary arteries, upper extremities, and brain. This phenomenon, Harlequin syndrome, was named after the 16th-century character Arlecchino (Harlequin), who wore a two-faced mask in his plays.