A comparison of treatment strategies for hypoplastic left heart syndrome using decision analysis

OBJECTIVES We sought to identify the optimal treatment strategy for hypopla stic left heart syndrome (HLHS). BACKGROUND Surgical treatment of HLHS involves either transplantation (Tx) or staged palliation of the native heart. Identifying the best treatment fo r HLHS requires integrating individual patient risk factors and center-spec ific data. METHODS Decision analysis is a modeling technique used to compare six strat egies: staged surgery; Tx; stage 1 surgery as an interim to Tx; and listing for transplant for one, two, or three months before performing staged surg ery if a donor is unavailable. Probabilities were derived from current lite rature and a dataset of 231 patients with HLHS born between 1989 and 1994. The goal was to maximize first-year survival. RESULTS If a donor is available within one month, Tx is the optimal choice, given baseline probabilities; if no donor is found by the end of one month , stage 1 surgery should be performed. When survival and organ donation pro babilities were varied, staged surgery was the optimal choice for centers w ith organ donation rates < 10% in three months and with stage I mortality < 20%. Waiting one month on the transplant Est optimized survival when the t hree-month organ donation rate was less than or equal to 30%. Performing st age 1 surgery before listing, or performing stage 1 surgery after an unsucc essful two- or three-month wait for transplant, were almost never optimal c hoices. CONCLUSIONS The best strategy for centers that treat patients with HLHS sho uld be guided by local organ availability, stage 1 surgical mortality and p atient risk factors. (C) 2001 by the American College of Cardiology.