November

Regional Brain Growth Trajectories in Fetuses with Congenital Heart Disease Caitlin K Rollins 1 2, Cynthia M Ortinau 3, Christian Stopp 4, Kevin G Friedman 4 5 6, Wayne Tworetzky 4 5 6, Borjan Gagoski 7 8, Clemente Velasco-Annis 7, Onur Afacan 7 8, Lana Vasung 7 8, Jeanette I Beaute 1, Valerie Rofeberg 4, Judy A Estroff 3 5 7 8, P Ellen Grant 7 8, Janet S Soul 1 2 5, Edward Yang 7 8, David Wypij 4 6 9, Ali Gholipour 7 8, Simon K Warfield 7 8, Jane W Newburger 4 6 Ann Neurol. 2021 Jan;89(1):143-157.  doi: 10.1002/ana.25940. Epub 2020 Nov 4. PMID: 33084086 DOI: 10.1002/ana.25940   Take Home Points: This novel study suggests that fetuses with congenital heart disease (CHD) especially those with lowest cerebral substrate delivery, show a region-specific pattern of small brain volumes and impaired brain growth before 32 weeks gestation. The brains of fetuses with CHD were more similar to those of CHD-related than optimal controls, suggesting genetic or environmental factors also contribute.     Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: In this interesting study the authors investigated the abnormal brain development in utero in association with congenital heart disease, with fetal magnetic resonance imaging (MRI). From 2014 to 2018, the authors enrolled 179 pregnant women into 4 groups: 1. “HLHS/TGA” fetuses with hypoplastic left heart syndrome (HLHS) or transposition of the great arteries (TGA) - diagnoses with lowest fetal cerebral substrate delivery; 2. “CHD-other,” with other CHD diagnoses; 3. “CHD-related,” healthy with a CHD family history; and 4. “optimal control,” healthy without a family history.   Two MRIs were obtained between 18 and 40 weeks gestation. Random effect regression models assessed group differences in brain volumes and relationships to hemodynamic variables.   HLHS/TGA (n = 24), CHD-other (50), and CHD-related (34) groups each had generally smaller brain volumes than the optimal controls (71). Compared with CHD-related, the HLHS/TGA group had smaller subplate (−13.3% [standard error = 4.3%], p < 0.01) and intermediate (−13.7% [4.3%], p < 0.01) zones. These volumetric reductions were associated with lower cerebral substrate delivery.        

Postnatal impact of a prenatally diagnosed double aortic arch Trisha V Vigneswaran 1 2 3, Milou Pm Van Poppel 3, Benedict Griffiths 4, Paul James 4, Haran Jogeesvaran 5, Zehan Rahim 6, John M Simpson 7 2 3, Simone Speggiorin 7, Vita Zidere 7 2, Andrew Nyman 4   Arch Dis Child. 2020 Oct 28;archdischild-2020-318946.  doi: 10.1136/archdischild-2020-318946. Online ahead of print.   PMID: 33115711 DOI: 10.1136/archdischild-2020-318946   Take Home Points: Early signs of tracheal compression are common and therefore delivery where onsite neonatal support is available is recommended. Significant tracheal compression may be present even in the absence of symptoms. Genetic testing may be offered following detection of double aortic arch.     Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: In this interesting study, the authors performed a retrospective review of the postnatal outcome for prenatally diagnosed double aortic arch.   Data was collected from two fetal cardiology units from 2014 to 2019, 50 cases were identified prenatally and 48 had postnatal follow-up. After birth, there was a complete DAA with patency of both arches in 8/48 (17%) and in 40/48 (83%) there was a segment of the left arch which was a non-patent, ligamentous connection.   Stridor was present in 6/48 (13%) on the day of birth. Tracheo-oesophageal compressive symptoms/signs were present in 31/48 (65%) patients at median age of 59 days (IQR 9–182 days). Seven of 17 (41%) asymptomatic cases demonstrated moderate–severe tracheal compression.   All morphologies of DAA caused symptoms and morphology type was not predictive of significant tracheal compression (p=0.3).