Abstract 13203: Mortality in Hypoplastic Left Heart Syndrome: Review of 216 Autopsy Cases of Aortic Atresia With Attention to Coronary Artery Pathology
Objectives: Some centers have shown that Mitral Stenosis/Aortic Atresia (MS/AA) subtype of Hypoplastic Left Heart Syndrome (HLHS) has higher mortality; however, the underlying patho-physiology of this is unclear. We reviewed our autopsy series to determine possible etiologies for increased mortality
Methods: We retrospectively reviewed all cases of AA/MS and AA/MA (Mitral Atresia) in the Cardiac Registry of Children's Hospital Boston from 1955-2009. Chart review of autopsy reports, operative notes, imaging studies was performed. Formalin-fixed hearts were examined, and cases found to have coronary artery abnormalities were sectioned at mid-left ventricular level in the transverse plane and at mid right ventricular level in the longitudinal plane for histological analysis of coronary arteries using standard H&E stains.
Results: 1337 patients with HLHS were seen in our institution from 1955-2009. There were 479 deaths in this time period. A total of 216 autopsy cases were identified with AA/MS and AA/MA. 61.5% (133) had AA/MS. 48 coronary anomalies were found: 36 ventriculo-coronary connections i.e. fistula (all in AA/MS), 12 coronary abnormalities (2 in AA/MS, 10 in AA/MA). Histology confirmed fistula (Fig 1) only in the AA/MS group with no evidence of fistula in the AA/MA group. CONCLUSIONS The occurrence of ventricle-coronary connections appears limited to the AA/MS group. Further prospective study is required to validate this finding and to identify subgroups (such as LV dependent coronary circulation) that carry a higher mortality risk Figure 1: Left ventricular myocardium at 40x demonstrating multiple coronary fistulae with abnormally thickened vessel walls.
- © 2010 by American Heart Association, Inc.