Coronary Blood Supply of Patients with Hypoplastic Left Heart Syndrome

Background: Hypoplastic left heart syndrome (HLHS) is a complex cardiac underdevelopment of the left sided part of the heart resulting in a very small left ventricle causing hemodynamic insufficiency. Patients suffering from this condition are treated with a staged surgery (Norwood I or hybrid, PCPC or Comprehensive Stage II, TCPC). The right ventricle serves as the systemic ventricle and the pulmonary trunk is used as the neo-aorta. The native aorta is connected to the neo-aorta by creating the Damus-Kaye-Stansel (DKS) anastomosis. Despite the success of this staged approach, patients are limited in their performance throughout their lives and are at high risk of hemodynamic failure. We studied various conceivable anatomic aspects of the DKS anastomosis that may influence the coronary blood supply. Coronary blood flow depends solely on retrograde flow in the native aorta, when antegrade flow through the hypoplastic left ventricle and aortic valve is not present.