Search

Venovenous extracorporeal membrane oxygenation (VV ECMO) can successfully support patients with refractory respiratory failure and is widely accepted as a bridge to recovery or bridge to transplantation. However, some problems hinder success. Recirculation, an innate complication of VV ECMO, hamper efficient oxygenation. Right ventricular (RV) failure secondary to respiratory failure is not uncommon and can be reversed by VV ECMO. But there are often times when RV failure gets worse, and since VV ECMO is no longer effective, additional measures are needed. Moreover, peripheral cannulation restricts active rehabilitation leading to weakness and weaning failure. Oxygenator-right ventricular assist device (OxyRVAD) refers any configuration that combines oxygenator and centrifugal pump. Compared to VV ECMO, it has advantages of hemodynamic support, elimination of recirculation, and facilitation of rehabilitation. In the present case, we overcame recirculation and impending RV failure by applying OxyRVAD to patient who was initially managed with VV ECMO. He underwent lung transplantation after about 6 months of OxyRVAD support with active rehabilitation, the longest maintenance period ever known.

Plasminogen plays an important role in fibrinolysis and is encoded by the PLG gene. The missense variant PLG Ala620Thr is the major cause of dysplasminogenemia in East Asian countries, including Korea. Although dysplasminogenemia was first reported in a Japanese patient with recurrent venous thromboembolism (VTE), subsequent studies have not demonstrated any clear association between the PLG Ala620Thr variant and the risk of VTE. To the best of our knowledge, this is the first report of a homozygous PLG Ala620Thr variant case from Korea.Here, we report a Korean family with PLG Ala620Thr mutation. The proband was a 34-year-old man who presented with multiple thrombotic arterial embolism and cardiac myxoma.Laboratory workup, including coagulation profile and PLG gene sequencing, was carried out for the affected family.The proband carried a heterozygous PLG Ala620Thr variant with decreased plasminogen activity of 65%. His 53-year-old mother, who had no reported history of VTE, was homozygous for the PLG Ala620Thr variant with decreased plasminogen activity of just 25%. Decreased plasminogen activity indicates dysplasminogenemia.We believe that this clinically silent homozygous case supports the previous findings that isolated PLG Ala620Thr variant does not confer a significant risk of VTE.