Bilateral Ebstein-like Anomaly with Atrial Septal Defect.

Abstract

We report an unusual congenital cardiac anomaly consisting of a downward displacement of all leaflets of both atrio-ventricular valves. In addition to these changes, an atrial septal defect of the secundum type was noted. In light of the extreme rarity of this interesting anomaly complex, we reviewed the literature and compare the morphologic features of the present case with classic Ebstein's anomaly. (Jpn Heart J 36: 119-125, 1995) arise from the annulus fibrosus but instead from the ventricular wall caudal to the atrio-ventricular ring. Ebstein's anomaly accounts for less than 1% of all heart malformations.1) In this case report we describe a lesion which, in addition to the usual Ebstein's anomaly, showed an almost mirror image type of change on the left side of the heart. The heart demonstrated situs solitus using the standard morphological criteria for defining the laterality (sideness) of the heart chamber walls.1) Patent foramen ovale, or atrial septal defect, is associated with classic Ebstein's anomaly in approximately 80 per cent of the cases.1) In our case, an atrial septal defect of the secundum type was demonstrated at time of postmortem examination. This is the third case report in the literature that describes a bilateral anomaly and the first of bilateral anomaly and atrial septal defect. CASE REPORTThis female fetus, delivered prematurely, was the product of a second preg-

In vivo imaging of mGlu5 receptor expression in humans with Fragile X Syndrome towards development of a potential biomarker

“…Regional and voxel-wise estimates of [ 18 F]FPEB non-displaceable binding potential (BP ND ), a measure of mGluR5 availability, were obtained for each subject by multi-linear reference tissue analysis of dynamic PET data 15 , 16 . We found regional brain uptake was consistent with known mGlu5 receptors based on previous studies 1 , 17 . A global reduction of [ 18 F]FPEB BP ND was observed in males with FXS compared to control subjects (Fig.…”

“…Importantly, this difference in BP ND between the subjects with FXS and control group was significant in areas consistent with known distribution of mGluR5, and which have been functionally implicated in FXS symptomatology. Our results of overall reduced mGlu5 receptor expression in relevant regions of the brain in individuals with FXS replicate recent findings 17 and advance ongoing efforts for a much-needed measure of mGluR5 target engagement for drugs and treatment of symptoms in clinical trials of FXS and related disorders 36 , 37 .…”

“…In a recent study, Brašić and colleagues 17 used the same [ 18 F]FPEB tracer in males with FXS, slightly younger than those in the present study, though incorporating different acquisition protocols and data analysis methods for PET across collaborating sites; they found mGluR5 density was significantly reduced in multiple brain regions including the cingulate, cortex, thalamus and striatum, compared to age-matched males with typical development, thereby supporting the use of [ 18 F]FPEB to measure drug occupancy in clinical trials for mGLuR5 in FXS.…”

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