A 4-year-old boy with atrioventricular discordance,
double-outlet right ventricle,
pulmonary stenosis, and
mitral regurgitation, was undergoing anatomical repair consisting of Senning, Rastelli, Damus-Kaye-Stansel procedures, and a mitral valve repair, complained of post-operative excessive airway tract secretion, which ultimately developed into
acute respiratory distress syndrome (ARDS) 28 days after the operation. The cause of the ARDS was thought to be frequent manual positive pressure recruitment and prolonged inhalation of pure
oxygen. At 45 days after the operation,
hypercapnia and
respiratory acidosis turned out to be irreversible, and therefore, veno-arterial
extracorporeal membrane oxygenation (ECMO) was established utilizing the Endumo(®)4000 system. Pulmonic interstitial
inflammation gradually improved while resting the lung under ECMO support; however, effective ventilation volume decreased critically because a massive pulmonary
hemorrhage occurred at 2 and 9 days after the initiation of ECMO. To maximize the effectiveness of respiratory
physical therapy, "Awake ECMO" was started and tidal volume dramatically increased with a regained
cough reflex. Five days later, he was successfully weaned off from ECMO, and discharged 7 months after the operation without any neurological and physiological sequelae.