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Coronary Arterial Abnormalities in Hypoplastic Left Heart Syndrome: Pathologic Characteristics of Archived Specimens

Lookup NU author(s): Professor Bob Anderson


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© 2020 Elsevier Inc. Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.

Publication metadata

Author(s): Stephens EH, Gupta D, Bleiweis M, Backer CL, Anderson RH, Spicer DE

Publication type: Article

Publication status: Published

Journal: Seminars in Thoracic and Cardiovascular Surgery

Year: 2020

Volume: 32

Issue: 3

Pages: 531-538

Print publication date: 01/09/2020

Online publication date: 11/02/2020

Acceptance date: 02/04/2018

ISSN (print): 1043-0679

ISSN (electronic): 1532-9488

Publisher: Elsevier


DOI: 10.1053/j.semtcvs.2020.02.007

PubMed id: 32060012


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