Clinical Update on the Hybrid Comprehensive Stage II Operation

ABSTRACT Objective We previously described the hybrid comprehensive stage II (HCS2) operation as an alternate surgical procedure for a subset of patients with single ventricle (SV) congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the four patients who have undergone this procedure. Methods After undergoing a hybrid approach to the stage I Norwood palliation, the HSC2 procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection. With this approach dissection of the distal arch and creation of a Damus-Kaye-Stansel anastomosis was avoided. A standard Fontan procedure was planned after the usual period of growth. Results The first patient, who had Trisomy 21 and elevated PA pressures, expired post-operatively due to left PA thrombosis. The subsequent three patients survived the procedure and remain clinically well. All have required catheterizations for re-intervention on their stented intrapulmonary baffles and ductal arches, and all have undergone successful completion of their Fontan procedures. Conclusion The HCS2 is a feasible, less complex alternative to the conventional comprehensive stage II operation in a subset of SV patients. Early post-operative anticoagulation to avoid PA thrombosis is recommended, and re-stenting of the ductal arch is anticipated. While the long-term consequences of separate outflow tracts supplying the upper and lower body is unknown, the three surviving patients with this circulation are doing well with their Fontan circulation at midterm follow up.