Baffa J M, Chen S L, Guttenberg M E, Norwood W I, Weinberg P M
Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine 19104.
J Am Coll Cardiol. 1992 Aug;20(2):350-8. doi: 10.1016/0735-1097(92)90101-r.
To determine whether right ventricular preservation is compromised in any anatomic subgroup of hypoplastic left heart syndrome, we assessed the coronary artery anatomy and myocardial histologic features of 151 postmortem specimens with hypoplastic left heart syndrome.
Although previous studies have shown that coronary artery abnormalities are more prominent in the subgroup of patients who have a patent mitral valve and obstructed aortic valve, it has not been established that these abnormalities compromise right ventricular perfusion.
Eighty-nine specimens with a patent mitral valve and aortic atresia, 52 with mitral and aortic atresia and 10 with patent mitral and aortic valves were examined. Histologic sections of the right ventricle, left ventricle and coronary arteries were obtained from 64 study hearts and 5 control hearts.
Gross coronary anomalies included coronary-cameral communications (n = 29), single left coronary artery (n = 2), single right coronary artery (n = 1) and tortuosity (n = 19). Coronary-cameral communications and tortuosity were significantly associated with the subgroup that had mitral hypoplasia and aortic atresia. Coronary artery wall thickness relative to lumen diameter was not different among the subgroups. No areas of coronary artery stenosis or interruption were discovered. Although endocardial fibroelastosis of the left ventricle was strongly associated with the mitral hypoplasia and aortic atresia group, the right ventricular histologic findings were similar in all subgroups and were not differentially affected by older age.
Although the incidence of coronary abnormalities is greater in patients with mitral hypoplasia and aortic atresia, in this study there was no apparent difference in perfusion of the right ventricle among the anatomic subgroups of hearts with hypoplastic left heart syndrome.
为了确定左心发育不全综合征的任何解剖亚组中右心室的保存是否受到损害，我们评估了151例左心发育不全综合征尸检标本的冠状动脉解剖结构和心肌组织学特征。
尽管先前的研究表明，在二尖瓣开放而主动脉瓣闭锁的患者亚组中冠状动脉异常更为突出，但尚未证实这些异常会损害右心室灌注。
检查了89例二尖瓣开放且主动脉闭锁、52例二尖瓣和主动脉均闭锁以及10例二尖瓣和主动脉均开放的标本。从64个研究心脏和5个对照心脏获取右心室、左心室和冠状动脉的组织学切片。
大体冠状动脉异常包括冠状动脉-心腔交通（n = 29）、单一左冠状动脉（n = 2）、单一右冠状动脉（n = 1）和迂曲（n = 19）。冠状动脉-心腔交通和迂曲与二尖瓣发育不全和主动脉闭锁亚组显著相关。各亚组之间冠状动脉壁厚度与管腔直径的比值无差异。未发现冠状动脉狭窄或中断区域。虽然左心室心内膜弹力纤维增生与二尖瓣发育不全和主动脉闭锁组密切相关，但所有亚组的右心室组织学结果相似，且不受年龄增长的差异影响。
尽管二尖瓣发育不全和主动脉闭锁患者冠状动脉异常的发生率较高，但在本研究中，左心发育不全综合征心脏的解剖亚组之间右心室灌注没有明显差异。
