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 extracorporeal membrane oxygenation veno-arteriovenous extracorporeal membrane oxygenation extracorporeal membrane oxygenation complications extracorporeal membrane oxygenation setup double pump setup innovations in extracorporeal membrane oxygenation

Get full access to this article

View all access options for this article.

References

Cakici

Gumus

Ozcinar

, et al. Controlled flow diversion in hybrid venoarterial-venous extracorporeal membrane oxygenation. Interact Cardiovasc Thorac Surg 2018; 26: 112–118.

Squiers

Lima

DiMaio

. Contemporary extracorporeal membrane oxygenation therapy in adults: fundamental principles and systematic review of the evidence. J Thorac Cardiovasc Surg 2016; 152: 20–32.

Lance

Drummond

Gandevia

, et al. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988; 51: 635–642.

Hans-Bittner

Bittner

Filho

. Do you know this syndrome? Harlequin syndrome. An Bras Dermatol 2018; 93: 585–586.

Lindholm

. Cannulation for veno-venous extracorporeal membrane oxygenation. J Thorac Dis 2018; 10: S606–S612.

Avgerinos

DeBois

Voevidko

, et al. Regional variation in arterial saturation and oxygen delivery during venoarterial extracorporeal membrane oxygenation. J Extra Corpor Technol 2013; 45: 183–186.

Rupprecht

Lunz

Philipp

, et al. Pitfalls in percutaneous extracorporeal membrane oxygenation cannulation. Heart Lung Vessel 2005; 7: 320–326.

Haldenwang

Baumann

Elghannam

, et al. Two-stage weaning strategy using veno-veno-arterial perfusion for patients on extracorporeal membrane oxygenation following cardiogenic shock. Perfusion 2019; 34: 689–695.

Napp

Kuhn

Hoeper

, et al. Cannulation strategies for percutaneous extracorporeal membrane oxygenation in adults. Clin Res Cardiol 2016; 105: 283–296.

10.

Cardarelli

Young

Griffith

. Use of extracorporeal membrane oxygenation for adults in cardiac arrest (E-CPR): a meta-analysis of observational studies. ASAIO J 2009; 55: 581–586.

11.

Zangrillo

Landoni

Biondi-Zoccai

, et al. A meta-analysis of complications and mortality of extracorporeal membrane oxygenation. Crit Care Resusc 2013; 15: 172–178.

12.

Raleigh

Hill

. Extracorporeal membrane oxygenation applications in cardiac critical care. Semin Cardiothorac Vasc Anesth 2015; 19: 342–352.