The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: Angiographic and pathological studies

Citation
H. Sugiyama et al., The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: Angiographic and pathological studies, PEDIAT CARD, 20(6), 1999, pp. 422-427
Citations number
12
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Pediatrics
Journal title
PEDIATRIC CARDIOLOGY
ISSN journal
0172-0643 → ACNP
Volume
20
Issue
6
Year of publication
1999
Pages
422 - 427
Database
ISI
SICI code
0172-0643(199911/12)20:6<422:TRBRVF>2.0.ZU;2-M
Abstract
Cases of hypoplastic left heart syndrome (HLHS) were studied angiographical ly in 18 patients and pathologically in 22 patients. They were divided into three subgroups according to the morphological features of the left heart: mitral atresia with aortic atresia (MA/AA), mitral stenosis with aortic at resia (MS/AA) and mitral stenosis with severe aortic stenosis (MS/AS), Pati ents with MS/AA had a significantly lower right ventricular end-diastolic v olume index and more hypokinesis of the right ventricular posterior wall th an those with MA/AA. MS/AA not only increased the thickness or the left Ven tricular posterior wall and interventricular septum but also increased endo cardial thickness compared with MA/AA. Myocardial histology revealed more f requent abnormal findings such as myocardial necrosis, calcification and in terstitial fibrosis in the mitral stenosis groups (i.e., MS/AA and MS/AS) t han in MA/AA. Right ventricular function appeared to be greatly influenced by left heart structure. The presence of larger left ventricular muscle bul k and frequent myocardial damage seen in MS/AA seems disadvantageous to rig ht ventricular end-diastolic volume and right ventricular wall motion.