A novel veno-arteriovenous extracorporeal membrane oxygenation with double pump for the treatment of Harlequin syndrome

Abstract

Objectives: The Harlequin syndrome is a complication observed in patients receiving peripheral venoarterial extracorporeal membrane oxygenation. This condition is defined as a critical variation in the oxygen saturation between the upper and the lower part of the body deriving from a poor lung function.

Methods: Between July 2018 and November 2019, a total of 60 patients (42 men and 18 women; mean age 57.4 ± 10.0 years; range = 28-71 years) underwent peripheral venoarterial extracorporeal membrane oxygenation in our center. Harlequin syndrome was identified in eight cases (six men and two women; 13.3%) of the 60 venoarterial extracorporeal membrane oxygenation-supported patients. As a result of the Harlequin syndrome, all these patients required conversion to veno-arteriovenous extracorporeal membrane oxygenation. Control and monitoring of the blood flows of the return cannulae were performed using two centrifugal pumps, one for each inlet line, according to the patient requirements to achieve optimum hemodynamic and oxygenation.

Results: Mean duration of veno-arteriovenous extracorporeal membrane oxygenation support was 5.3 ± 1.4 days. Seven patients (87.5%) were switched to venovenous extracorporeal membrane oxygenation, and after 13.5 ± 2.7 days, those patients were totally weaned from extracorporeal membrane oxygenation support. One patient (12.5%) had an improvement in the pulmonary function, but the cardiac function was poor. This patient was switched to venoarterial extracorporeal membrane oxygenation, and after 10 days, the patient was completely weaned from extracorporeal membrane oxygenation support.

Conclusion: The use of a secondary centrifugal pump to manage the blood flow directed to the internal jugular vein, in the veno-arteriovenous extracorporeal membrane oxygenation setup, allows the reduction in the risk of blood clot formation, clotting factor consumption, and pulmonary embolism when compared to the use of an external clamp.

Keywords: Harlequin syndrome; double pump setup; extracorporeal membrane oxygenation; extracorporeal membrane oxygenation complications; extracorporeal membrane oxygenation setup; innovations in extracorporeal membrane oxygenation; veno-arteriovenous extracorporeal membrane oxygenation.