Successful management of harlequin syndrome due to pulmonary hemorrhage and atelectasis with VAV- ECMO

Perfusion. 2023 Jun 5:2676591231181847. doi: 10.1177/02676591231181847. Online ahead of print.

Abstract

Introduction: Pulmonary hemorrhage is a life-threatening complication of VA-ECMO occasionally presenting with Harlequin syndrome.

Case report: We present a case of a VA-ECMO patient complicated with pulmonary hemorrhage, complete right lung atelectasis and differential hypoxia refractory to conventional treatment including optimal mechanical ventilation and bronchoscopy interventions. Patient was successfully managed by conversion of VA to VAV-ECMO.

Discussion: Pulmonary hemorrhage and atelectasis treatment in a VA-ECMO patient includes transfusion, hold and reversal of anticoagulation, bronchoscopy interventions and optimization of VA-ECMO and ventilator support. Differential hypoxia may ensue due to residual native cardiac function. If refractory to conservative treatment, a VAV-ECMO configuration may be utilized to improve upper body oxygenation by inserting an additional cannula to the superior vena cava.

Conclusion: VAV-ECMO is an ECMO configuration support in patients at risk of Harlequin syndrome presenting with pulmonary hemorrhage.

Keywords: VAV ECMO; atelectasis; cardiogenic shock; differential hypoxia; pulmonary hemorrhage.