Eating Cheerios on ECMO: The Use of VA ECMO as a Bridge to Lung Transplant in an Infant

Introduction Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. In PVOD the pulmonary venous vessels are obliterated resulting in obstructive pulmonary vascular disease, increase in pulmonary vascular resistance, right heart failure and death. Pulmonary hypertension is the most common reason for lung transplant in infants. Lung transplant is the only therapy for PVOD. Prolonged intubation, deconditioning and extra corporeal life support are associated with poor outcomes after lung transplant. We present a case of PVOD in an infant who had successful lung transplant using a novel veno-arterial extracorporeal membrane oxygenation (VA ECMO) configuration. Case Report A previously healthy 15-month-old male presented with cough and tachypnea. Cardiomegaly and pulmonary edema were seen on chest x-ray. Echocardiogram revealed severe pulmonary hypertension. Cardiac catheterization study and chest computed tomography revealed PVOD. He was unable to be extubated after cardiac catheterization and was transferred to our institution for lung transplant evaluation. He had severely depressed right ventricular function, requiring vasopressors to maintain hemodynamic stability. Respiratory status was tenuous, requiring sedation and paralysis. He was evaluated and listed for lung transplant. The patient was centrally cannulated for VA ECMO using cannulae for a ventricular assist device (Berlin ExCor) that were exteriorized on the abdomen. “Venous” inflow cannula was placed in the main pulmonary artery to provide afterload reduction for the right ventricle. After starting VA ECMO, vasopressor support, respiratory support, paralysis and sedation were weaned. He was woken up, extubated; and intensive physical and occupational rehabilitation began. He regained strength, was able to sit, stand and take steps. He also regained his milestones, was able to babble, wave and eat simple foods. The patient remained on VA ECMO for 61 days until he successfully underwent lung transplantation. Summary Our case highlights the successful use of a novel VA ECMO configuration as a bridge to lung transplant in an infant with PVOD. To our knowledge this is the youngest patient to undergo awake VA ECMO reported. The use of a unique cannulation allowed for rehabilitation while on VA ECMO. Though challenging, VA ECMO remains an option as a bridge to lung transplant in infants with PVOD.