Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism.

Right atrial isomerism is associated with complex cardiac malformations, particularly single-ventricle lesions; right atrial isomerism is rarely associated with aorto-pulmonary collateral arteries. We report a foetal diagnosis of right atrial isomerism, with an unbalanced atrioventricular septal defect, pulmonary stenosis, total anomalous venous drainage, and significant aorto-pulmonary collaterals diagnosed at 22 weeks' gestation.

Impact of prenatal diagnosis on the management and early outcome of critical duct-dependent cardiac lesions.

OBJECTIVE: The objective of this study was to compare the preoperative management and outcome of neonates with duct-dependent critical CHD with fetal versus postnatal diagnosis. METHODS: Patients referred with CHD to our centre from January 1, 2009 to December 31, 2010 were enrolled prospectively. Live births with a critical form of CHD, a gestational age ⩾36 weeks and a weight ⩾2 kg at birth, and the intention-to-treat were included in this sub-study. Excluded were neonates with lethal non-cardiac and/or genetic anomalies. RESULTS: In total, 129, 63 fetal and 66 postnatal, cases met the study inclusion criteria. All had received appropriate antenatal care, including a routine fetal anatomy scan. Both cohorts were comparable in weight, gestational age, and APGAR scores at birth. Unlike the postnatal cases, there were no deaths (0/63 versus 5/66; p=0.06) and no cardiac arrests (0/63 versus 9/63; p=0.003) before surgery or catheter intervention in those cases with a prenatal diagnosis of critical CHD. Moreover, newborns with fetal diagnoses were admitted earlier (median 0 (range 0-3) versus 2 (0-25) days; p<0.001) and were less likely to require preoperative ventilation (19/63 versus 31/61, p=0.03) and vasoactive medication (4/63 versus 15/61, p=0.006) than the postnatal cases. CONCLUSIONS: Prenatal diagnosis of critical CHD in this study was associated with significantly shorter time intervals from birth to neonatal admission and the absence of life-threatening or fatal preoperative cardiac events. Increased efforts should be made to improve rates of prenatal diagnosis.

Serial echocardiography for immune-mediated heart disease in the fetus: results of a risk-based prospective surveillance strategy.

OBJECTIVE: Mothers carrying anti-Ro antibodies are frequently referred for weekly echocardiograms to early detect and treat antibody-mediated fetal heart disease. We tested a surveillance strategy based on anti-Ro antibody titers. METHODS: From 2009 to 2014, 232 pregnancies were referred for maternal anti-Ro antibodies. At the baseline echocardiogram, anti-Ro titers were measured by enzyme-linked immunosorbent essay and results categorized as negative (<8 U/mL; n = 43; excluded), low-moderate positive (8-49 U/mL; n = 62; group 1) or high positive (50 - >100 U/mL; n = 127; group 2). Serial echocardiograms to ≥24 weeks were only recommended for group 2 mothers. RESULTS: Group 1 patients underwent significantly less fetal echocardiograms when compared with group 2 mothers (median 2 vs. 4; p < 0.001). Isolated endocardial fibroelastosis (n = 1) and incomplete (n = 4) or complete (n = 4) heart block were diagnosed in 9 (8%) pregnancies with anti-Ro titers >100 U/mL but none with lower titers (odds ratio 17.78; p = 0.004). Incomplete block and endocardial fibroelastosis regressed with transplacental corticosteroid and immune globulin therapy. CONCLUSIONS: Limiting serial fetal echocardiograms to women with high anti-Ro antibody levels is safe and more cost effective. While numbers of echocardiograms were significantly reduced in referrals with anti-Ro titers <50 U/mL, reversible abnormalities with prenatal treatment were detected by serial echocardiography in group 2 patients. © 2017 John Wiley & Sons, Ltd.