Double inlet right ventricle versus other types of double or common inlet ventricle: its clinical characteristics with reference to the Fontan procedure

Objective: To determine the clinical features in patients with double inlet right ventricle (DIRV) and separate atrioventricular (AV) valves in terms of the Fontan procedure. Methods: Surgical procedures were carried out in 3 1 patients with this ra-re, malformation. Their clinical characteristics we re compared with similar features in 45 patients with double inlet left ven tricle (DILV), 93 with common inlet right ventricle (CIRV), and 20 with com mon inlet left ventricle (CILV). Results: Pulmonary atresia with the right and the left pulmonary arteries being confluent was seen in 58% of patients with DIRV, and pulmonary atresia with non-confluent pulmonary arteries in 15% (P = 0.0001). The incidence of these findings was significantly higher when compared with DILV (P = 0.0001). Reflecting these morphologic features , constructions of systemic-to-pulmonary shunts and extensive plasty to the pulmonary arteries were more frequently needed in patients with DIRV than in the others (P = 0.04, 0.0001). The AV valves have become moderately or s everely regurgitant in three with DIRV (10%). The Fontan procedure has thus been employed in 21 with DIRV (64%), this incidence being significantly hi gher than that seen in the CIRV (37%) group (P = 0.01). Survival rate of 95 % at 10 years in this group was as excellent as that in the DILV group, and obviously better than that in either CIRV or CILV (P = 0.002). Conclusions : Although multiple palliative procedures rnight be frequently needed to ma intain pulmonary perfusion, the Fontan circulation can be justifiably estab lished in patients with DERV, and regurgitation across the AV valves was no t very common. (C) 2001 Elsevier Science B.V. All rights reserved.