A one-and-a-half repair in a pulmonary atresia intact ventricular septum: A more appropriate final repair strategy

Background: Pulmonary atresia with intact ventricular septum (PAIVS) may be unable to compensate for right heart failure in the remote period due to inadequate right ventricular function, even if both ventricular circulations are maintained in the short term. One-and-a-half repairs may have a lower risk of remote right heart failure and severe complications than biventricular repair (BVR). We compared complications and hemodynamics during the medium term for each type of circulatory repair, estimated the risk of right heart failure, and discussed the PAIVS strategy, considering the remote prognosis. Materials and Methods: Of 105 PAIVS patients who underwent surgical intervention at our hospital, we excluded patients who died ( n = 8) and those with Glenn circulation awaiting Fontan repair ( n = 3). Ninety-four patients were analyzed (BVR, n = 16; one-and-a-half, n = 6; Fontan, n = 72). Hemodynamics and complications in the medium term were compared among groups according to the final repair. Results: The mean age (range) at the last evaluation was as follows: BVR, 17.5 (2–39) years; one-and-a-half, 28.5 (6–34) years; and Fontan, 14 (3–36) years. The New York Heart Association (NYHA) classification was lowest in the one-and-a-half group (P = 0.06) (NYHA I/II/III: BVR, 11/5/0; one-and-a-half, 6/0/0; Fontan, 65/7/0). The central venous pressure was the lowest in the one-and-a-half group: one-and-a-half, 4 (1–14) mmHg; BVR, 4.5 (1–10) mmHg; and Fontan, 8 5–15 mmHg. Conclusions: A one-and-a-half repair is a final repair because it offers a safer hemodynamic margin and may also carry a lower risk of long-term right ventricle failure than BVR.