Successful use of veno-arterial extracorporeal membrane oxygenation in a patient with high-risk pulmonary embolism and cardiogenic shock during sickle-cell crisis: a case report

Background Sickle cell disease, the most common hereditary hemolytic disorder worldwide, is characterized by recurrent hemolytic and vaso-occlusive crises that are responsible for much of its morbidity and mortality. Data on the management of cardiogenic shock in the setting of sickle cell crisis is limited, especially with regards to mechanical circulatory support. Case summary We present the case of a 23-year-old male with sickle cell disease and a history of allogeneic hematopoietic stem cell transplantation, who initially presented to the hospital due to recurrent pain exacerbation. The clinical course was complicated by a high-risk pulmonary embolism during a sickle cell crisis, resulting in rapid cardiopulmonary deterioration and refractory shock, for which veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was successfully employed as rescue therapy. VA-ECMO support was successfully weaned after two days, follow-up assessment six months later demonstrated substantial recovery of both left and right ventricular systolic function. Despite multiple intracranial hemorrhages and markedly elevated neuron-specific enolase levels during VA-ECMO support, both strong predictors of poor neurological outcome, the patient was discharged without neurological deficits. Discussion This case demonstrates the potential role of VA-ECMO as a feasible rescue strategy in carefully selected patients with sickle cell disease who develop refractory shock secondary to pulmonary embolism. Furthermore, markedly elevated neuron-specific enolase levels should be interpreted with caution in the setting of chronic hemolysis, as they may overestimate the extent of hypoxic-ischemic brain injury and thereby limit their prognostic value for neurological outcome.