Extracorporeal Life Support for Severe Acute Chest Syndrome in Adult Sickle Cell Disease: A Preliminary Report.

Abstract

Extracorporeal life support could be helpful for severe acute chest syndrome in adults sickle cell disease, because of the frequent hemodynamic compromise in this setting, including acute pulmonary vascular dysfunction and right ventricular failure. The aim of this study was to report the extracorporeal life support experience for severe acute chest syndrome in four referral centers in France. The primary endpoint of this multicentric retrospective study was ICU survival of patients with severe acute chest syndrome managed with extracorporeal life support. Secondary endpoints included comparisons between survivors and nonsurvivors. We performed this study between January 2009 and July 2017 in four referral centers in France. We included adult patients (age > 18 yr) with sickle cell disease, admitted for severe acute chest syndrome and who required extracorporeal life support during the ICU stay. The study was observational. Over the 8-year period, 22 patients with sickle cell disease required extracorporeal life support for severe acute chest syndrome, including 10 (45%) veno-venous and 12 (55%) veno-arterial extracorporeal life support. In-ICU mortality was high (73%). Nonsurvivors had a higher severity at extracorporeal life support implantation, as assessed by their Vasoactive-Inotrope Score and number of organ failures. Our study shows that outcome is impaired in sickle cell disease patients receiving extracorporeal life support while in severe multiple organ failure. Further studies are needed to evaluate selection criteria in this setting.