Coronary Arterial Abnormalities in Hypoplastic Left Heart Syndrome: Pathologic Characteristics of Archived Specimens.
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.
['Stephens|Elizabeth H|EH|', 'Gupta|Dipankar|D|', 'Bleiweis|Mark|M|', 'Backer|Carl L|CL|', 'Anderson|Robert H|RH|', 'Spicer|Diane E|DE|']
D000015:Abnormalities, Multiple D001021:Aortic Valve D001024:Aortic Valve Stenosis D002641:Chicago D003330:Coronary Vessel Anomalies D003331:Coronary Vessels D005431:Florida D006801:Humans D018636:Hypoplastic Left Heart Syndrome D008943:Mitral Valve D008946:Mitral Valve Stenosis D017202:Myocardial Ischemia D016157:Vascular Fistula
Coronary artery Hypoplastic left heart syndrome Aortic atresia Mitral stenosis Congenital heart disease