Objectives. To determine the most common fetal ultrasound markers of total anomalous pulmonary venous return (TAPVR) during mid-trimester ultrasound using standardly obtained images and evaluate the performance of diagnostic algorithms for improving prenatal diagnosis. Methods. This was a matched case-control study at a regional referral centre (2005 to 2019). Cases of TAPVR were matched to controls 1 : 4 by date of birth and biologic sex. Postprocessing review of stored fetal ultrasound images was performed by two blinded and independent observers in a standardized fashion using nine sonographic markers: (i) left/right heart disproportion; (ii) abnormal distribution of great vessels; (iii) pulmonary vein entry into the left atrium (LA); (iv) confluence behind the LA; (v) abnormal coronary sinus; (vi) absence of the Coumadin ridge; (vii) aortic diameter; (viii) distance between LA and aorta; and (ix) post-LA space index >1.27. Descriptive and inferential statistics were used to present results and compare cases and controls. Diagnostic algorithms were compared by sensitivity/specificity. Results. 21 cases of isolated TAPVR were matched to 84 controls (n = 105). The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). Cases of TAPVR had significantly larger post-LA spaces than controls ( p < 0.0001 ) and wider aortic diameters ( p = 0.006 ). A diagnostic algorithm stratifying on absence of pulmonary veins followed by an abnormal Coumadin ridge, can correctly identify cases of TAPVR with high specificity (90.5%) and moderate sensitivity (61.9%). Conversely, a diagnostic algorithm using the presence of any 3 abnormal markers had improved specificity (94.1%) but poorer sensitivity (23.8%). Conclusions. Using standardly obtained images from routine fetal ultrasound, improved prenatal detection of isolated TAPVR is possible. A standardized diagnostic approach can be highly specific for fetal TAPVR, however, algorithms that are sufficiently sensitive for screening in the general population are still needed.

Objective. To describe the early neurodevelopmental outcomes following fetal exposure to previable preterm prelabour rupture of membranes (pPPROM). Methods. This was a secondary analysis of a subgroup of neonates born following pPPROM from a retrospective cohort study (2009–2015). Surviving infants who underwent standardized neurodevelopmental evaluation at 18–24 months corrected age (CA) between 2017 and 2019 were eligible for inclusion. Data abstracted from hospital charts were linked to prospectively collected developmental outcomes stored in an electronic database from a regional neonatal follow-up clinic. The primary outcome was Bayley-III composite scores (compared to the population mean 100, standard deviation (SD) 15). Secondary outcomes included presence of cerebral palsy, vision loss, hearing impairment, and requirement of rehabilitation therapy. Descriptive statistics were used to present results. Results. 25.7% (19/74) of neonates born after pPPROM survived to hospital discharge, but only 21.6% (16/74) survived to 18–24 months CA. Of these, 9 infants were eligible for follow-up at the regional clinic and 7 had developmental outcomes stored in the electronic database. Infants exposed to pPPROM exhibited Bayley-III scores more than 1 SD below the population mean across all three domains: cognitive 84.9 (SD 12.2); motor 82.3 (SD 11.5); and language 66.4 (SD 18.9). There were particular deficiencies in language development with 71% (5/7) scoring more than 2 SDs below the population mean. There were no cases of cerebral palsy. Conclusions. Only 1 in 5 infants born following expectantly managed pPPROM survived to 18–24 months CA. These infants born after pPPROM had significantly lower Bayley-III scores and particular deficiencies in language development. Better understanding of early neurodevelopmental challenges following pPPROM will help refine counselling of families contemplating expectant management and provide insights into the postnatal educational resources required to improve long-term developmental outcomes for these children.