Stage 1 Palliation for Hypoplastic Left Heart Syndrome Without the Use of Allogeneic Tissue, with Reduced Allogeneic Blood Product Exposure: A Case Report

Abstract

In the 30 years since Norwood described the palliative procedure for hypoplastic left heart syndrome (HLHS), many modifications have been described which have increased the survival rate of children born with this lesion. We describe further modifications which result in reduced cardiopulmonary bypass time, no cooling or circulatory arrest time, and decreased banked blood exposure. A 16-day-old infant with HLHS undiagnosed during pregnancy presented for stage 1 palliation incorporating the Mee modification, Sano right ventricle to pulmonary artery conduit, dual arterial cannulation of the innominate artery and descending aorta, single venous cannulation of the right atrium, and a bypass prime volume of 130 mL. Anticoagulation and hemostasis were monitored with the Hepcon HMS Plus Hemostasis Management System (Medtronic USA, Minneapolis, MN). Bypass commenced at normothermia. A 5.0 Gore-Tex shunt was placed for the Sano Shunt, and the aortic arch was repaired without use of homologous tissue or synthetic material using a modification of the Mee technique. Bypass time was 92 minutes with a 10 minutes cardiac ischemic time. Modified ultrafiltration (MUF) was performed for 12 minutes and heparinization was reversed with protamine. There was no significant bleeding and no indication to transfuse clotting factors. The patient’s only allogeneic donor exposure was 350 mL of red blood cells during bypass necessary to achieve a post MUF hematocrit of 50% per our current institution policy for cyanotic infants. Using modified surgical and perfusion techniques along with low prime bypass circuits can result in reduced cross clamp and bypass times as well as a decrease in blood donor exposure. Hypothetical benefits include reduced operating room, ventilation, intensive care unit, and hospital times, improved neurodevelopmental outcomes, and an overall reduction in the cost of care for infants with HLHS.