Upper-body extracorporeal membrane oxygenation as a strategy in decompensated pulmonary arterial hypertension
A 23-year-old woman with PAH associated with an unrepaired 15-mm secundum atrial septal defect (ASD) with bidirectional shunting and WHO functional Class III symptoms on intravenous and oral PAH therapies presented with severe hypoxemia from decompensated PAH that was refractory to inhaled nitric oxide and inotropic agents (). ECMO was initiated as BTT. A bicaval dual-lumen cannula, designed to drain deoxygenated blood from the vena cavae and reinfuse oxygenated blood across the tricuspid valve, was inserted via the right internal jugular vein (RIJV; ).[] By directing reinfused blood across the ASD toward the left atrium, an oxygenated right-to-left shunt was created, effectively providing venoarterial ECMO with a single venous access site.[] The patient was extubated in the operating room. Pulmonary vasodilators were down-titrated both to divert oxygenated reinfusion flow away from the pulmonary vasculature and through the ASD, and to avoid systemic vasodilation which may occur with shunting of pulmonary vasodilators into the systemic circulation. On ECMO Day 7, the patient underwent successful lung transplantation and ASD closure, and was discharged home 20 days post-transplant.