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

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 144; no. 6; pp. 1301 - 1306
• Main Authors: Nathan, Meena, MD, Williamson, Alex K., MD, Mayer, John E., MD, Bacha, Emile A., MD, Juraszek, Amy L., MD
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.12.2012; Elsevier
• Subjects: Abnormalities, Multiple; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Aortic Valve - abnormalities; Aortic Valve - pathology; Autopsy; Biological and medical sciences; Boston; Cardiology. Vascular system; Cardiothoracic Surgery; Chi-Square Distribution; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Coronary heart disease; Coronary Vessel Anomalies - mortality; Coronary Vessel Anomalies - pathology; Fistula - mortality; Fistula - pathology; Heart; Heart Ventricles - abnormalities; Heart Ventricles - pathology; Humans; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - pathology; Medical sciences; Mitral Valve - abnormalities; Mitral Valve - pathology; Mitral Valve Stenosis - mortality; Mitral Valve Stenosis - pathology; Pneumology; Registries; Retrospective Studies; Risk Assessment; Risk Factors; Boston; AA; hypoplastic left heart syndrome; RV; EFE; left ventricle (ventricular); MS; LV; mitral atresia; RVEFE; LVEFE; HLHS; left ventricular endocardial fibroelastosis; endocardial fibroelastosis; MA; mitral stenosis; right ventricular endocardial fibroelastosis; aortic atresia; 20; right ventricle (ventricular); Prognosis; Mortality; Cardiovascular disease; Left Heart Hypoplasia; Review; Congenital disease; Coronary heart disease; Syndrome; Case study; Autopsy; Atresia; Anesthesia; Aorta; Circulatory system; Cardiology; Bibliographic review
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/j.jtcvs.2012.03.013

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.