Harlequin Syndrome during Venoarterial Extracorporeal Membrane Oxygenation
Chetan Pasrija , Kareem Bedeir, Jean Jeudy, Zachary N. Kon
Author Affiliations
From the Division of Cardiac Surgery (C.P.) and Department of Radiology (J.J.), University of Maryland, 110 S Paca St, 7th Floor, Baltimore, MD 21201; Division of Cardiac Surgery, Brigham and Women’s Hospital, Boston, Mass (K.B.); and New York University, Department of Cardiothoracic Surgery, New York, NY (Z.N.K.).
Address correspondence to C.P. (e-mail: cpasrija@som.umaryland.edu).
Published Online:Jun 20 2019https://doi.org/10.1148/ryct.2019190031
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).
a: (a) Axial and (b) 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 (blue arrow) and retrograde flow in the descending aorta via the femoral extracorporeal membrane oxygenation cannula with preferential flow to the left subclavian artery (red arrow).
b: (a) Axial and (b) 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 (blue arrow) and retrograde flow in the descending aorta via the femoral extracorporeal membrane oxygenation cannula with preferential flow to the left subclavian artery (red arrow).
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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.
Disclosures of Conflicts of Interest: C.P. disclosed no relevant relationships. K.B. disclosed no relevant relationships. J.J. disclosed no relevant relationships. Z.N.K. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: consultant for Breethe; receives payment for lectures from Medtronic; has stock in Breethe; travel, accommodations, meeting expenses paid by Breethe, Medtronic, and Lung Bioengineering; and pending patent for graded stiffness wire/bidirectional flow cannula (specialty cannula and wire intended for ECMO cannulation). Other relationships: disclosed no relevant relationships.
Chetan Pasrija , Kareem Bedeir, Jean Jeudy, Zachary N. Kon
Author Affiliations
From the Division of Cardiac Surgery (C.P.) and Department of Radiology (J.J.), University of Maryland, 110 S Paca St, 7th Floor, Baltimore, MD 21201; Division of Cardiac Surgery, Brigham and Women’s Hospital, Boston, Mass (K.B.); and New York University, Department of Cardiothoracic Surgery, New York, NY (Z.N.K.).
Address correspondence to C.P. (e-mail: cpasrija@som.umaryland.edu).
Published Online:Jun 20 2019https://doi.org/10.1148/ryct.2019190031
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).
a: (a) Axial and (b) 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 (blue arrow) and retrograde flow in the descending aorta via the femoral extracorporeal membrane oxygenation cannula with preferential flow to the left subclavian artery (red arrow).
b: (a) Axial and (b) 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 (blue arrow) and retrograde flow in the descending aorta via the femoral extracorporeal membrane oxygenation cannula with preferential flow to the left subclavian artery (red arrow).
Download as PowerPointOpen in Image Viewer
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.
Disclosures of Conflicts of Interest: C.P. disclosed no relevant relationships. K.B. disclosed no relevant relationships. J.J. disclosed no relevant relationships. Z.N.K. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: consultant for Breethe; receives payment for lectures from Medtronic; has stock in Breethe; travel, accommodations, meeting expenses paid by Breethe, Medtronic, and Lung Bioengineering; and pending patent for graded stiffness wire/bidirectional flow cannula (specialty cannula and wire intended for ECMO cannulation). Other relationships: disclosed no relevant relationships.
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