Mortality in hypoplastic left heart syndrome: Review of 216 autopsy cases of aortic atresia with attention to coronary artery disease

Objectives: Aortic atresia (AA) in hypoplastic left heart syndrome (HLHS) has been associated with increased mortality in several prior studies. We reviewed our autopsy series to explore the relationship of coronary abnormalities to anatomic subsets of HLHS with AA. Methods: We retrospectively reviewed all pathology specimens with AA/MS (mitral stenosis) and AA/MA (mitral atresia) in the Cardiac Registry of Children's Hospital Boston between 1955 and 2009 including autopsy reports, operative notes, and imaging studies. Formalin-fixed hearts were examined, and cases found to have macroscopic 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 histologic analysis of coronary arteries using tissue sections stained with hematoxylin and eosin. Results: A total of 216 autopsy cases were identified with AA/MS (134) and AA/MA (82). Coronary anomalies were found in 49 cases, left ventricle-coronary fistula in 39, all in AA/MS, and 10 other coronary abnormalities, all in AA/MA. Histologic study confirmed fistulas only in the AA/MS group with no evidence of fistulas in the AA/MA group. Conclusions: The occurrence of left ventricle-coronary fistulas appears limited to the AA/MS group, and coronary fistula specimens were disproportionately more prevalent in postoperative specimens. Further clinical studies are required to validate this finding and to identify subgroups that carry a higher mortality risk.