Unexpected death after reconstructive surgery for hypoplastic left heart syndrome

Background. Although operative mortality for reconstructive surgery for hyp oplastic left heart syndrome continues to improve, nonoperative mortality, especially in the first year of life, remains relatively high. A number of patients who are thought to be clinically well at hospital discharge die un expectedly. The goal of the present study was to determine the incidence of and risk factors for unexpected death in patients with hypoplastic left he art syndrome. Methods. Retrospectively, we determined the incidence of unexpected death a mong 536 patients with hypoplastic left heart syndrome who were discharged to home after stage I surgical procedure. To identify potential risk factor s, a nested case-control analysis was undertaken. Results. Unexpected death occurred in 22 of 536 patients (4.1%) discharged to home after stage I surgical procedure. The median age at unexpected deat h was 79 days (range, 25 to 227 days). Seizures preceded cardiac arrest in 2 patients, and ventricular arrhythmias were documented in 3 additional pat ients during attempted resuscitation. Autopsy studies were performed in 12 patients and identified residual lesions that may have contributed to death in 2 patients. In multivariate analysis documented perioperative arrhythmi a and earlier year of stage I surgical procedure were associated with an in creased risk for unexpected death (p = 0.03 and p = 0.04, respectively). Th ere were 4 additional patients who had unexpected death after subsequent ca vopulmonary operation at a median age of 1.6 years (range, 0.9 to 3.8 years ). Conclusions. Unexpected death occurred in more than 4% of patients with hyp oplastic left heart syndrome who were discharged to home after stage I surg ical procedure and was most common in the first several months of life. Fac tors that may contribute to unexpected death include residual lesions, arrh ythmias, and neurologic events, although in the majority of eases the cause remains largely unknown. (Ann Thorac Surg 2001;71:61-5) (C) 2001 by The So ciety of Thoracic Surgeons.