Background-Prenatal diagnosis of aortic coarctation suffers from high false-negative rates at screening and poor specificity. Methods and Results-This retrospective study tested the applicability of published aortic arch and ductal Z scores (measured just before the descending aorta in the 3-vessel and tracheal view) and their ratio on 200 consecutive normal controls at a median of 22Ϯ0 gestational weeks (range, 15Ϯ4 to 38Ϯ4 weeks). Second, this study tested the ability of serial Z scores to distinguish fetuses with coarctation within a cohort with ventricular and/or great arterial disproportion detected at screening or fetal echocardiography. Third, it evaluated the diagnostic significance of associated cardiac lesions, coarctation shelf, and isthmal flow disturbance. We studied 44 fetuses with suspected coarctation at 24Ϯ0 weeks (range, 17Ϯ3 to 37Ϯ4 weeks). Receiver-operating characteristic curves were created. Logistic regression tested the association between z scores, additional cardiac diagnoses, and coarctation. Good separation was found of isthmal Z scores for cases requiring surgery from controls and false-positive cases, and receiver-operating characteristic curves showed an excellent area under the curve for isthmal Z score (0.963) and isthmal-to-ductal ratio (0.969). Serial isthmal Z scores improved to ϾϪ2 in suspected cases with normal outcomes; those requiring surveillance or surgery remained ϽϪ2. Minor lesions did not increase the diagnostic specificity of coarctation, but isthmal flow disturbance increased the odds ratio of true coarctation versus arch hypoplasia 16-fold. Conclusions-Isthmal

Background-Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. Methods and Results-We studied 175 fetuses diagnosed with second-or third-degree atrioventricular block (2000 -2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age Ͻ20 weeks, ventricular rate Յ50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of Ն1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4Ϯ2.9 versus 24.9Ϯ4.9 weeks; Pϭ0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. Conclusions-Risk factors associated with a poor outcome were gestation Ͻ20 weeks, ventricular rate Յ50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen. (Circulation.

Objectives 22.4 (range. 16.6-7.0) weeks and the median number of scans per fetus was three (range, one to five). I < −2 at final scan was the most powerful predictor (odds ratio, 3.6 (95% CI,). Shelf was identified in 66% and Flow in 50% of fetuses with CoA.

Objectives Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life