Upper-body extracorporeal membrane oxygenation as a strategy in decompensated pulmonary arterial hypertension
A 22-year-old woman with PAH following an arterial switch operation as a newborn for transposition of the great vessels, stenting for left pulmonary vein stenosis, and WHO functional Class II symptoms, who had been well-controlled on oral PAH therapy presented with hypoxemic respiratory failure due to pneumonia. Despite noninvasive ventilation, inotropes, diuretics, and antibiotics, she had progressive right heart, respiratory, and renal failure, with supra-systemic pulmonary artery pressures on echocardiogram. She was intubated and placed on venoarterial ECMO as BTR. A drainage cannula was inserted through the RIJV and a reinfusion cannula was grafted to the right subclavian artery.[] She was started on intravenous epoprostenol and was extubated within 24 hours. She participated in physical therapy within 48 hours of extubation, ambulating 30 feet by ECMO Day 6. With up-titration of epoprostenol and volume removal with dialysis, ECMO was discontinued after eight days. Within two weeks, she was optimized on oral PAH therapy and iloprost, and epoprostenol was discontinued. She was discharged home 20 days post-ECMO, remaining off oxygen and on a stable PAH regimen.