Cardiovascular Perfusion Unit, Department of Cardiac Surgery and Heart Transplantation, S. Camillo Hospital, Rome, Italy.
Department of Heart and Vessels, Cardiac Surgery Unit and Heart Transplantation Center, S. Camillo-Forlanini Hospital, Rome, Italy.
Perfusion. 2020 May;35(1_suppl):65-72. doi: 10.1177/0267659120908409.
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.
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.
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.
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.
哈雷奎因(Harlequin)综合征是接受外周动静脉体外膜肺氧合(VA-ECMO)治疗的患者中观察到的一种并发症。这种情况定义为上半身和下半身之间的氧饱和度发生严重变化,源自肺功能不佳。
在 2018 年 7 月至 2019 年 11 月期间,共有 60 名患者(42 名男性和 18 名女性;平均年龄 57.4±10.0 岁;范围 28-71 岁)在我院接受了外周动静脉体外膜肺氧合治疗。在 60 名接受 VA-ECMO 支持的患者中,有 8 例(6 名男性和 2 名女性;13.3%)被诊断为哈雷奎因综合征。由于哈雷奎因综合征,所有这些患者都需要转为静脉-动脉-静脉体外膜肺氧合。根据患者的需求,使用两个离心泵分别为每个入口线控制和监测返回插管的血流,以实现最佳的血液动力学和氧合。
静脉-动脉-静脉体外膜肺氧合支持的平均时间为 5.3±1.4 天。7 名患者(87.5%)被转为静脉-静脉体外膜肺氧合,13.5±2.7 天后,这些患者完全脱离体外膜肺氧合支持。1 名患者(12.5%)的肺功能有所改善,但心功能较差。该患者被转为 VA-ECMO,10 天后完全脱离体外膜肺氧合支持。
在静脉-动脉-静脉体外膜肺氧合设置中,使用辅助离心泵来管理流向颈内静脉的血流,与使用外部夹相比,可以降低血栓形成、凝血因子消耗和肺栓塞的风险。