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Open in new tab Download slide Open in new tab Download slide OBJECTIVES Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. METHODS We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. RESULTS A total of 132 children were operated on for a DAA (n  = 77) or a RAA (n  = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7–13.3) months and for RAA was 13.9 (6.4–20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0–15.6] vs 5.4 months [1.8–17.7]; P  = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P  = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [ P  = 0.006, odds ratio = 3.9 (95% confidence interval 1.5–9.4)]. CONCLUSIONS Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes. [ABSTRACT FROM AUTHOR]

Prenatal diagnosis of congenital heart disease is now well established for a wide range of cardiac anomalies. Diagnosis of congenital heart disease during fetal life not only identifies the cardiac lesion but may also lead to detection of associated abnormalities. This information allows a detailed discussion of the prognosis with parents. For continuing pregnancies, appropriate preparation can be made to optimize the postnatal outcome. Reduced morbidity and mortality, following antenatal diagnosis, has been reported for coarctation of the aorta, hypoplastic left heart syndrome, and transposition of the great arteries. With regard to screening policy, most affected fetuses are in the "low risk" population, emphasizing the importance of appropriate training for those who undertake such obstetric anomaly scans. As a minimum, the four chamber view of the fetal heart should be incorporated into midtrimester anomaly scans, and where feasible, views of the outflow tracts should also be included, to increase the diagnostic yield. Newer screening techniques, such as measurement of nuchal translucency, may contribute to identification of fetuses at high risk for congenital heart disease and prompt referral for detailed cardiac assessment. [ABSTRACT FROM AUTHOR]

Aim: The impact of prenatal diagnosis on morbidity and mortality for certain types of congenital heart disease (obstructive left heart lesions and transposition of the great arteries) is well established. No data are available for lesions with duct dependent pulmonary flow. We aimed to assess the impact of prenatal diagnosis of pulmonary atresia on clinical presentation and neonatal outcome. Method: Fifty-eight newborns with pulmonary atresia presenting to our centre were identified retrospectively between 1997 and 2004 (prenatal diagnosis n = 37, postnatal n = 21). Anatomical sub-types included intact ventricular septum (PAIVS, n = 33) and ventricular septal defect (PAVSD, n = 25); those with more complex anatomy were excluded. Results: After adjusting for anatomical sub-type, postnatally diagnosed infants were significantly more hypoxic at presentation (mean oxygen saturation 65% vs 84%). However, they presented early (median age 1 day) and prostaglandin E was initiated promptly (median 3 hours) with rapid improvement of oxygen saturations (interaction p<0.001). This resulted in no appreciable differences in terms of pH, base deficit, blood pressure or heart rate between the groups by the time of the first catheter/surgical intervention. Postnatal infants did not differ in terms of length of intensive care unit (p = 0.18) or hospital stay (p = 0.86), incidence of complications (p=0.72), or mortality (p=0.77). Multivariable analysis revealed a positive association between occurrence of complications and both degree of cyanosis at presentation (rather than postnatal diagnosis per se) and anatomy (PAIVS). Conclusion: Postnatal diagnosis of pulmonary atresia is associated with greater cyanosis at presentation. However this does not translate into greater neonatal morbidity or mortality provided that early recognition and prompt initiation of prostaglandin E therapy occur. [ABSTRACT FROM AUTHOR]

Circulating maternal anti-Ro antibodies can cross the placenta after 16 weeks of gestation and damage the cardiomyocytes and the conducting system leading to myocarditis and fetal arrhythmias. The commonest arrhythmia seen with anti-Ro antibodies is complete heart block, although varying degrees of atrioventricular block and rarely, fetal tachycardias have been described. We describe the echocardiographic diagnosis and management of a fetus with anti-Ro mediated complete heart block who developed atrial flutter in the third trimester. In utero management of atrial flutter on a background of complete heart block is challenging as anti-arrhythmic agents may cause further bradycardia, but with a non-aggressive approach to treatment of atrial flutter a good outcome was achieved. [ABSTRACT FROM AUTHOR]