55: Novel Techniques to Treat Harlequin Syndrome and Determine Brain Death on Peripheral VA ECMO

Introduction: We present the case of an obese (BMI=46) 58-year-old woman who underwent ECPR for witnessed out-of-hospital PEA arrest. Case Presentation: After peripheral cannulation for VA ECMO and return of spontaneous circulation, she developed severe pulmonary edema and Harlequin Syndrome. A 17-French single-stage cannula was then inserted via the left subclavian vein into the main pulmonary artery and attached to the arterial limb of the ECMO circuit, which resolved the Harlequin Syndrome. In the Intensive Care Unit, she remained unresponsive, and imaging suggested severe anoxic brain injury. However, due to morbid obesity and pulmonary edema, apnea testing could not be used to determine brain death, as the patient rapidly desaturated when disconnected from the ventilator. Thus, she underwent nuclear medicine brain flow/brain death imaging. No evidence of intracranial radiotracer flow was seen on flow or 30-minute delayed images, and the patient was pronounced brain dead. Conclusions: Our case illustrates a novel treatment of Harlequin Syndrome using a cannula positioned in the main pulmonary artery and attached to the arterial limb. This technique provides both oxygenation and hemodynamic support for the right ventricle. This cannulation strategy is also very positionally secure. Successful determination of brain death in a patient on ECMO who could not undergo apnea testing due to rapid development of hypoxia when disconnected from the ventilator. Our case also illustrates that brain flow nuclear medicine scanning is a robust approach to brain death determination in patients where hypoxia precludes apnea testing.

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Publication Details

Journal
ASAIO Journal
Published
2025-10-01
DOI
https://doi.org/10.1097/01.mat.0001171516.68659.70
Primary Topic
Sympathectomy and Hyperhidrosis Treatments
Type
article
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article

55: Novel Techniques to Treat Harlequin Syndrome and Determine Brain Death on Peripheral VA ECMO

Nathan D. Peffley, Jeremy Grate
ASAIO Journal
Sympathectomy and Hyperhidrosis Treatments
article

55: Novel Techniques to Treat Harlequin Syndrome and Determine Brain Death on Peripheral VA ECMO

Nathan D. Peffley, Jeremy Grate
article en

Abstract

Introduction: We present the case of an obese (BMI=46) 58-year-old woman who underwent ECPR for witnessed out-of-hospital PEA arrest. Case Presentation: After peripheral cannulation for VA ECMO and return of spontaneous circulation, she developed severe pulmonary edema and Harlequin Syndrome. A 17-French single-stage cannula was then inserted via the left subclavian vein into the main pulmonary artery and attached to the arterial limb of the ECMO circuit, which resolved the Harlequin Syndrome. In the Intensive Care Unit, she remained unresponsive, and imaging suggested severe anoxic brain injury. However, due to morbid obesity and pulmonary edema, apnea testing could not be used to determine brain death, as the patient rapidly desaturated when disconnected from the ventilator. Thus, she underwent nuclear medicine brain flow/brain death imaging. No evidence of intracranial radiotracer flow was seen on flow or 30-minute delayed images, and the patient was pronounced brain dead. Conclusions: Our case illustrates a novel treatment of Harlequin Syndrome using a cannula positioned in the main pulmonary artery and attached to the arterial limb. This technique provides both oxygenation and hemodynamic support for the right ventricle. This cannulation strategy is also very positionally secure. Successful determination of brain death in a patient on ECMO who could not undergo apnea testing due to rapid development of hypoxia when disconnected from the ventilator. Our case also illustrates that brain flow nuclear medicine scanning is a robust approach to brain death determination in patients where hypoxia precludes apnea testing.

ASAIO JournalVol. 71(Supplement 6)
Mayo Clinic in Arizona (US)
Openalex Percentile: Top 61%
Sympathectomy and Hyperhidrosis Treatments
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55: Novel Techniques to Treat Harlequin Syndrome and Determine Brain Death on Peripheral VA ECMO — Nathan D. Peffley, Jeremy Grate · ASAIO Journal (2025) | TGRS Research Map | TGRS