CASE REPORTA 38-year-old woman, gravida 2 para 1, was referred for fetal echocardiography for evaluation of abnormal cardiac axes in a set of twins. The woman had previously delivered a term male infant who died at 1 month of age from severe respiratory distress despite aggressive mechanical support. Autopsy revealed bilateral lung hypoplasia with arrested acinar development, small pulmonary arteries and a radial alveolar count of four. The couple was not consanguineous.The mother presented to our program at 18 weeks' gestation with dizygotic twins (female and male), products of in-vitro fertilization. General fetal ultrasound examination revealed a right-sided subependymal hemorrhage in the female, and mildly decreased thoracic circumference and normal amniotic fluid levels in both twins. Fetal echocardiography in both demonstrated normal cardiac anatomy and function. Both had a leftward cardiac axis (female, 90 • ; male, 80 • ) and an increased cardiothoracic diameter ratio (female, 60%; male, 50%), and the female had a small pericardial effusion (Figure 1). Both fetuses had hypoplastic branch pulmonary arteries (0.9-1.2 mm in female and 1.3-1.5 mm in male vs. 1.8 mm expected). Flow could not be demonstrated in the pulmonary arteries or pulmonary veins by color or pulsed Doppler imaging in either twin. The findings of hypoplastic branch pulmonary arteries with decreased flow and increased cardiothoracic diameter ratios suggested pulmonary hypoplasia. The parents requested elective termination of the pregnancy.The twins were delivered at 19 weeks' gestation, weighing 169 g (male) and 127 g (female). In both, the diaphragms were intact and there were no cardiac malformations, skeletal dysplasia or renal malformations. The lungs were normally configured but extremely small. In the male, the lungs weighed 1.6 g (expected 5.6 g). In the female, the lungs weighed 1.4 g. Both had markedly hypoplastic extrapulmonary branch pulmonary arteries.The lungs of each fetus showed similar histopathology: there was a lobular architecture with thickened connective tissue septa and prominent lymphatics. In contrast to cuboidal epithelium expected at this developmental stage, the distal sac epithelium of the twins appeared intermediate between columnar and cuboidal epithelium, with low columnar epithelium containing dark nuclei. There was a striking paucity of terminal airspaces within each lobule, and the mesenchymal interstitium was thicker than normal. Peripheral airways were larger than those observed in controls. The findings suggested persistent pseudoglandular stage. The cartilaginous airways were found closer to the pleura than normal, suggesting failure

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Introduction: The prenatal diagnosis of conotruncal defects (CTDs) has improved over the past decade, particularly with inclusion of outflow tract imaging at routine ultrasound. Appropriate prenatal counseling for CTDs demands an accurate diagnosis, as even more subtle cardiac pathology could complicate clinical outcomes. We sought to determine the anatomical accuracy of fetal echocardiography in evaluating common CTDs and factors that contribute to accuracy. Methods: All cases of tetralogy of Fallot(TOF), double outlet right ventricle(DORV) and truncus arteriosus(TA) encountered in our institution from 2007-2018 were reviewed. Discrepancies in anatomical findings between prenatal (most accurate exam) and postnatal (echo/surgery) or autopsy exams were categorized as: C1) no difference C2) minor difference with no impact on outcome (e.g.aberrant right subclavian artery), C3) minor difference that could make a minor difference to the delivery plan or surgery (e.g.branch pulmonary artery stenosis), C4) major difference that changes the course of the pregnancy, delivery or surgical planning (e.g.ductal dependency). Results: Of the 255 CTD cases, 162 had prenatal and postnatal and/or autopsy data available. Of the 162, 107(65.6%) fit C1, 35(21.5%) C2, 12(7.4%) C3, and 8(5.5%) C4. The greatest accuracy was observed in TOF, with 69/71(97.2%) in C1 and C2 versus 56/69(81.2%) in DORV and 16/22(72.7%) in TA(p=0.003). Excluding 5 cases at 10-16weeks, there was a tendency towards a greater proportion in C1 and C2 when examined at 17-23 weeks (60/64, 93.4%) versus 24-32 weeks (50/57, 87.7%) and >32 weeks (28/36, 77.8%) (p=0.06). Era of assessment also revealed a difference with 43/55(78.2%) of studies performed from 2007-2011 in C1 and C2 versus 99/107(92.5%) from 2012-2018 (p=0.01).When we compared pregnancies with one versus serial exams, we observed a lower proportion of C4 cases from 9.6% to 2.7%, respectively. In those with serial exams, 20% had achieved C1 or C2 only at serial exam. Conclusions: The diagnostic accuracy of fetal echocardiography in CTDs is generally high, especially for TOF and when performed at 17-24weeks. There has been significant improvement in accuracy since 2011. Serial exams potentially improve diagnostic accuracy.