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The atrial septum in fetal Hypoplastic Left Heart Syndrome

Cook, AC; Sharland, GK; (1997) The atrial septum in fetal Hypoplastic Left Heart Syndrome. Heart , 77 (SUPPL. 1)

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Premature closure of the foramen ovale has long been implicated in the etiology of the Hypoplastic Left Heart Syndrome (HLHS). A restrictive atrial septum is also an indication for early intervention in HLHS after birth and has recently been cited as the cause of pulmonary vascular disease resulting in early death after stage I of the Norwood operation. In order to establish the state of the foramen ovale in the developing human fetus with HLHS, the cardiac specimens of 117 fetuses with this syndrome were examined. The size and patency of the foramen ovale together with the attachments and thickness of the flap valve were documented. The same parameters were examined in 27 fetuses with severe aortic stenosis as the progression of this condition to HLHS during fetal life has been documented. 246 fetuses with structurally normal hearts were used as age matched controls. In fetuses with mitral and aortic atresia (group 1:72 of 117) the morphology of the foramen ovale showed wide variation. Premature closure was rarely encountered (4/72). The state of the foramen ovale ranged from severe hypoplasia (29/72) to a normal (22/72) or dilated foramen ovale (17/72). Deviation of the flap valve towards the left atrium and hypoplasia of the limbus were also commonly observed. In contrast those hearts with severe aortic stenosis (group 3: n=27) had a normally sized foramen, no deviation of the flap valve attachments and a normally formed limbus. In this group premature closure of the flap valve was common (16/27). Fetuses with aortic atresia and a patent, hypoplastic mitral valve (group 2:45 of 117) showed a combination of the features found in groups 1 & 2. In all groups, the lungs of fetuses with a prematurely closed foramen showed evidence of pulmonary vascular disease. These findings indicate that a closed atrial septum and pulmonary vascular abnormalities should be expected in 14% of fetuses with HLHS. The atrial septum will be restrictive in a further 45% and may well become closed by term. Inter-atrial flow patterns should be documented when diagnosis of HLHS is made in the fetus. Premature closure of the foramen ovale is more likely to be a result of HLHS whereas severe hypoplasia of the foramen cannot be excluded as a cause for reduced flow into the left heart leading to the syndrome in some fetuses.

Type: Article
Title: The atrial septum in fetal Hypoplastic Left Heart Syndrome
UCL classification: UCL > Office of the President and Provost
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: http://discovery.ucl.ac.uk/id/eprint/1306897
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