Objective Patients with heterotaxy and complex congenital heart disease (CHD) undergo cardiac surgery with high mortality and morbidity. Recent studies have revealed an association among heterotaxy, CHD, and primary ciliary dyskinesia (PCD). We undertook a retrospective review of patients undergoing cardiac surgery at Children’s National Medical Center between 2004 to 2008 to explore the hypothesis that in heterotaxy patients there is increased mortality and respiratory complications. Methods and Results Retrospective review was performed on post-surgical outcomes of 87 patients with heterotaxy and CHD exhibiting the full spectrum of situs abnormalities associated with heterotaxy. As controls, 634 cardiac surgical patients with CHD but without laterality defects were selected, and surgical complexities were similar with a median RACHS-1 score of 3.0 for both groups. We found the mean length of postoperative hospital stay (17 vs 11 days) and mechanical ventilation (11 vs 4 days) were significantly increased in the heterotaxy patients. Also elevated were rates of tracheostomies (6.9% vs. 1.6%; Odds Ratio 4.6), extracorporeal membrane oxygenation (ECMO) support (12.6% vs. 4.9%: Odds Ratio 2.8), prolonged ventilatory courses (23% vs. 12.3%; Odds Ratio 2.1) and post-surgical deaths (16.1% vs. 4.7%; Odds Ratio 3.9). Conclusions Our findings show heterotaxy patients had more post-surgical events with increased post-surgical mortality and risk for respiratory complications as compared to controls with similar RACHS-1 surgical complexity scores. We speculate that increased respiratory complications maybe due to ciliary dysfunction. Further, studies are needed to explore the basis for the increased surgical risks for heterotaxy patients undergoing cardiac surgery.

*Rapid advances in medical imaging are facilitating the clinical assessment of first-trimester human embryos at increasingly earlier stages. To obtain data on early human development, we used magnetic resonance (MR) imaging and episcopic fluorescence capture (EFIC) to acquire digital images of human embryos spanning the time of dynamic tissue remodeling and organogenesis (Carnegie stages 13 to 23). These imaging data sets are readily resectioned digitally in arbitrary planes, suitable for rapid high-resolution three-dimensional (3D) observation. Using these imaging datasets, a web-accessible digital Human Embryo Atlas (http://apps.devbio.pitt.edu/humanatlas/) was created containing serial 2D images of human embryos in three standard histological planes: sagittal, frontal, and transverse. In addition, annotations and 3D reconstructions were generated for visualizing different anatomical structures. Overall, this Human Embryo Atlas is a unique resource that provides morphologic data of human developmental anatomy that can accelerate basic research investigations into developmental mechanisms that underlie human congenital anomalies. Developmental Dynamics 239:1585-1595,

Embryos undergo complicated morphogenetic changes during organogenesis. Detailed characterization of the remodeling will help in elucidating the etiology of the congenital anomalies. The analysis of such changes require the visualization of embryonic structures in three‐dimensions (3D). To generate high resolution 3D images through the period of organogenesis, human embryos from the Kyoto Collection at Carnegie stages 13 to 23 were imaged by magnetic resonance imaging (MRI) and with episcopic fluorescence image capture (EFIC) ‐ a novel high resolution histological technique that provides perfectly registered serial 2D images suitable for high fidelity 3D rendering. Embryos were first MRI scanned using a 7T super‐conducting magnet, followed by EFIC imaging. Obtained high‐resolution images were resectioned digitally in multiple planes, allowing detailed visualization of anatomic structures in the developing embryo. Using these data sets, we constructed a digital atlas, with each stage represented by serial 2D image stacks shown in three orthogonal planes. The Atlas also includes annotations delineating important anatomic structures and developmental milestones, as well as 3D Quicktime movies in different perspectives. This Atlas of the Human Embryo should serve as an invaluable resource for morphologic information of human development, and for studying the etiology of the congenital anomalies.