Repair of hypoplastic left heart syndrome of a 4.25-kg Jehovah’s witness

Abstract

The care of patients who refuse homologous transfusions has challenged cardiac surgery teams to refine blood conservation techniques and question standard trans-fusion practices. We cared for a newborn child with hypoplastic left heart syndrome (HLHS) whose parents refused to give consent to care for the child that involved the transfusion of homologous blood. A Norwood Stage I procedure was planned with the understanding that transfusions would be avoided, if possible. A court order was obtained that specified the conditions under which the attending physicians would transfuse the newborn. The birth weight of the patient was 4.25 kg. A low prime cardiopulmonary bypass (CPB) circuit and aggressive blood conservation techniques that included modified ultrafiltration (MUF) allowed the completion of the repair and CPB portion of the operation without the use of blood. The lowest hematocrit during CPB was 20%. After an unsuccessful attempt to separate from CPB, blood was transfused. Recovery was consistent for HLHS patients following Norwood Stage I. However, at 1 month postoperatively, the patient did require a shunt reduction for pulmonary overcirculation. Norwood Stage II repair was completed at age 4 months without donor blood. The key to a successful outcome is a well-thoughtout plan by the surgeon, anesthesiologist and perfusionist. This plan should include careful monitoring of the patient’s oxygenation and cardiovascular status.