Objective To evaluate the use of pulse oximetry to screen for early detection of life threatening congenital heart disease. Design Prospective screening study with a new generation pulse oximeter before discharge from well baby nurseries in West Götaland. Cohort study comparing the detection rate of duct dependent circulation in West Götaland with that in other regions not using pulse oximetry screening. Deaths at home with undetected duct dependent circulation were included. Setting All 5 maternity units in West Götaland and the supraregional referral centre for neonatal cardiac surgery. Participants 39 821 screened babies born between 1 July 2004 and 31 March 2007. Total duct dependent circulation cohorts: West Götaland n=60, other referring regions n=100. Main outcome measures Sensitivity, specificity, positive and negative predictive values, and likelihood ratio for pulse oximetry screening and for neonatal physical examination alone. Results In West Götaland 29 babies in well baby nurseries had duct dependent circulation undetected before neonatal discharge examination. In 13 cases, pulse oximetry showed oxygen saturations ≤90%, and (in accordance with protocol) clinical staff were immediately told of the results. Of the remaining 16 cases, physical examination alone detected 10 (63%). Combining physical examination with pulse oximetry screening had a sensitivity of 24/29 (82.8% (95% CI 64.2% to 95.2%)) and detected 100% of the babies with duct dependent lung circulation. Five cases were missed (all with aortic arch obstruction). False positive rate with pulse oximetry was substantially lower than that with physical examination alone (69/39 821 (0.17%) v 729/38 413 (1.90%), P<0.0001), and 31/69 of the “false positive” cases with pulse oximetry had other pathology. Thus, referral of all cases with positive oximetry results for echocardiography resulted in only 2.3 echocardiograms with normal cardiac findings for every true positive case of duct dependent circulation. In the cohort study, the risk of leaving hospital with undiagnosed duct dependent circulation was 28/100 (28%) in other referring regions versus 5/60 (8%) in West Götaland (P=0.0025, relative risk 3.36 (95% CI 1.37 to 8.24)). In the other referring regions 11/25 (44%) of babies with transposition of the great arteries left hospital undiagnosed versus 0/18 in West Götaland (P=0.0010), and severe acidosis at diagnosis was more common (33/100 (33%) v 7/60 (12%), P=0.0025, relative risk 2.8 (1.3 to 6.0)). Excluding premature babies and Norwood surgery, babies discharged without diagnosis had higher mortality than those diagnosed in hospital (4/27 (18%) v 1/110 (0.9%), P=0.0054). No baby died from undiagnosed duct dependent circulation in West Götaland versus five babies from the other referring regions. Conclusion Introduc...

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

BackgroundPrenatal screening for foetal cardiac abnormalities has been increasingly practiced in Sweden during the last 25 years. A prenatal diagnosis may have medical benefits but may also cause sustained parental psychological distress. The aim of this study was to explore pregnant women’s, and their partner’s, experiences of counselling and need for support during continued pregnancy following a prenatal diagnosis of a cardiac defect. A second aim was to use this information to propose a structured follow-up programme for continued support after the first counselling.MethodDesign: Qualitative study, using interviews performed 5–9 weeks after a prenatal diagnosis of congenital heart disease.Setting: A tertiary foetal cardiology unit in SwedenSample: Six pregnant women and their 6 partners, consecutively recruited after a prenatal diagnosis of an isolated and significant cardiac defect.Data analysis: Qualitative content analysis.ResultsThe analysis resulted in three themes. 1/ Counselling and making a decision - the importance of knowledge and understanding: Short waiting time for specialist evaluation together with clear and straightforward information was essential. Parents called for written information together with a high-quality website with relevant information about congenital heart disease. 2/ Continued support during pregnancy: Continued and easy access to health care professionals, including a paediatric specialist nurse, throughout pregnancy, was important. Contact with couples with similar experiences and social media were also considered valuable sources of support. 3/ Next step – the near future: Practical and economical issues during the postnatal hospital stay and the initial period following the hospital stay were common concerns.ConclusionsThe following aspects should be considered in a structured follow up program during pregnancy after a prenatal diagnosis of CHD; written information, access to a safe web-site with information of high quality in their native language, support from parents with similar experiences and continued contact with a specialist liaison nurse with experience of paediatric cardiology.

Objective

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 To investigate whether prenatal screening is effective in the detection of total anomalous pulmonary venous connection (TAPVC) and to identify common prenatal features. Methods

Many infants with critical congenital heart defects are not diagnosed before discharge from the neonatal maternity ward. The proportion discharged undiagnosed has increased. Neonatal screening fails mainly in children with duct-dependent systemic circulation.

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