Introduction: Although hypoplastic left heart syndrome (HLHS) is extensively studied this disease still has a high mortality rate compared to other diseases treated as univentricular physiology. In this context, morphological differences between phenotypes within the spectrum of HLHS may be recognized as risk factors, and their identification can assist in choosing treatment between subgroups.Objective: To identify the most prevalent form of coronary artery dominance in HLHS in subgroups with atresia and mitral stenosis.Methods: Analysis of the coronary anatomy according to the distribution of epicardial branches and the dominance patterns classified as right, left, or balanced (co-dominant). Control group was composed of nine specimens of morphologically normal hearts; the HLHS group consisted of nine specimens with MA and 24 specimens with MS. We applied Chi-square test for statistical analysis.Results: There were significant differences between the two groups in relation to coronary artery dominance (÷2 = 9.298; P = 0.01). Left dominance was present in 75% of MS cases and the balanced (co-dominant) dominance was observed only in MS. In the control group, right dominance was observed in all cases (P <0.01).Conclusions: Left dominance is more common in HLHS than in the control group of normal hearts and in HLHS. The left coronary dominance is more frequent in the subgroup with mitral stenosis.Descriptors: Heart/anatomy & histology. Coronary vessels. Hypoplastic left heart syndrome. Heart defects, congenital. Rev Bras Cir Cardiovasc 2011;26(4):604-8
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