AN INSTITUTIONAL EXPERIENCE WITH 2ND-STAGE AND 3RD-STAGE PALLIATIVE PROCEDURES FOR HYPOPLASTIC LEFT-HEART SYNDROME - THE IMPACT OF THE BIDIRECTIONAL CAVOPULMONARY SHUNT

Objectives. The aim of this study was to perform a retrospective analy sis of an institutional experience with a consecutive series of patien ts with post-stage I palliation for hypoplastic left heart syndrome (H LHS). Background. In a recent review of 212 consecutive patients who u nderwent stage I operations for HLHS at our institution between 1983 a nd 1993, we identified risk factors related to stage I mortality. We s ought to examine the outcome for these patients at subsequent palliati ve procedures. Methods. All patients who underwent stage I reconstruct ion between January 1983 and June 1993 and also underwent subsequent p alliation at our institution were included. Seventy patients underwent palliative procedures and two underwent heart transplantation. Patien t-specific factors and features of the stage II operation were analyze d for impact on stage II mortality and actuarial survival. Results. Th e only independent risk factor for stage II mortality was the performa nce of a nonfenestrated Fontan operation (p < 0.001). There were nine in-hospital deaths (69%) in the 13 patients undergoing the nonfenestra ted Fontan procedure at stage II. Fifty patients underwent intermediat e superior vena cava to pulmonary artery anastomosis at stage II, with 4 (8%) early deaths. Pulmonary artery augmentation was performed in 1 9 patients (38%) at stage II, without increased operative risk Hypopla stic left heart syndrome anatomic subtype did not influence stage II m ortality. The modified fenestrated Fontan procedure has been performed as a third stage in 32 patients whose median age was 28.7 months, wit h one early death at a median follow-up of 24.5 months. Conclusions. A second-stage bidirectional cavopulmonary anastomosis for HLHS reduces second-stage mortality and improves intermediate survival. The modifi ed fenestrated Fontan operation may then be performed as a final palli ative stage with low operative risk. (C) 1997 by the American College of Cardiology.