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Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome
J M Baffa1, S L Chen, M E Guttenberg
1Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine 19104.
Insights
Right ventricular preservation is not compromised in hypoplastic left heart syndrome (HLHS) subgroups, despite varying coronary artery abnormalities. This study found no significant differences in right ventricular perfusion across HLHS anatomic variations.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Coronary artery abnormalities are more frequent in specific hypoplastic left heart syndrome (HLHS) subgroups.
- Previous research suggested a link between coronary abnormalities and HLHS, but right ventricular perfusion impact remained unclear.
Purpose of the Study:
- To investigate if right ventricular preservation is affected in different anatomic subgroups of hypoplastic left heart syndrome (HLHS).
- To assess coronary artery anatomy and myocardial histology in HLHS specimens.
Main Methods:
- Examined 151 postmortem HLHS specimens, categorized by mitral and aortic valve status.
- Histologic analysis of the right ventricle, left ventricle, and coronary arteries was performed on 64 hearts.
- Compared findings across HLHS subgroups and with 5 control hearts.
Main Results:
- Identified gross coronary anomalies including coronary-cameral communications and tortuosity, significantly associated with mitral hypoplasia and aortic atresia.
- No differences in coronary artery wall thickness or evidence of stenosis/interruption were found among subgroups.
- Right ventricular histologic findings were similar across all HLHS subgroups, unaffected by age.
Conclusions:
- While coronary abnormalities are more common in the mitral hypoplasia and aortic atresia subgroup of HLHS, right ventricular perfusion does not appear to be compromised.
- No significant differences in right ventricular perfusion were observed among the distinct anatomic subgroups of hypoplastic left heart syndrome.
Objectives:
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.
Background:
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.
Methods:
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.
Results:
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.
Conclusions:
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.