Management of Patients with Single Ventricle Circulation

Single ventricle (SV) circulation is a term used to describe a wide variety of complex heart defects which are not suitable for biventricular repair. In other words, these hearts can’t be repaired with surgery such that they are finally divided into four chambers. After palliation, patients with SV will have effectively a circulation with only one ventricle (LV or RV as the “systemic ventricle”) that is pumping oxygen-rich blood to the systemic outflow while the oxygen-poor blood returns passively to the lungs. SV palliation is achieved through three main stages (Figs. 1 and 6). At the final stage of palliation, deoxygenated blood (apart from coronary sinus blood) is drained directly to the pulmonary circulation by bypassing the SV heart. The overall early survival rates for SV palliation have improved to over 90% and 10 years survival rate is about 70%.