Improved Pretransplant Management of Infants with Hypoplastic Left Heart Syndrome Enables Discharge to Home While Waiting for Transplantation

For infants whose families select primary transplantation for hypoplastic left heart syndrome (HLHS), the waiting time averages 3 months. Given the relative shortage of organs, the morbidity and mortality of these patients have been high. Therefore, pretransplant management is critical to improve the number of patients who survive to transplantation. This series shows our evolving management for these children, with an emphasis on nonintensive care. Fifty-two infants with HLHS were listed for primary transplantation at our institution during a 6-year period. The management was aimed at manipulating the pulmonary and systemic blood flows by low-dose continuous infusion of prostaglandin E1 (PGE1), early use of inhaled nitrogen, delayed opening of the atrial septum, and discharge to home with PGE1 infusion for continuing care when the child was on room air and growing. Almost all of the children (46/ 52) required nitrogen therapy with initial FiO2 of 0.16-0.17. Patients were weaned off nitrogen by 5 to 6 weeks of age. One fourth of the children needed atrial septal opening, typically at 2 or 3 months of age. Seventeen (32.7%) of the infants were able to spend at least some of their waiting time at home. Forty-five of the 52 children (86.5%) survived to receipt of a donor heart. Newborns with HLHS whose families select primary transplantation as their surgical option can be managed with a minimally invasive approach until receipt of a donor heart with an improvement in mortality rate.