Joanna Dangel

Isolated Atrioventricular Block in the Fetus A Retrospective, Multinational, Multicenter Study of 175 Patients

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 <20 weeks, ventricular rate ≤50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 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 (23.4±2.9 versus 24.9±4.9 weeks; P =0.02), 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 <20 weeks, ventricular rate ≤50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen. # Clinical Perspective {#article-title-22}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 <20 weeks, ventricular rate ⩽50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 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 (23.4±2.9 versus 24.9±4.9 weeks; P=0.02), 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 <20 weeks, ventricular rate ⩽50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Ablation of severe drug-resistant tachyarrhythmia during pregnancy

Ablation of Tachyarrhythmia During Pregnancy. Aims: The goal of this study was to describe mapping and ablation of severe arrhythmias during pregnancy, with minimum or no X‐ray exposure. Treatment of tachyarrhythmia in pregnancy is a clinical problem. Pharmacotherapy entails a risk of adverse effects and is unsuccessful in some patients. Radiofrequency ablation has been performed rarely, because of fetal X‐ray exposure and potential maternal and fetus complications.

Recommendations for the practice of fetal cardiology in Europe.

F ETAL CARDlOLOGY IS CURRENTLY PRACTISED IN of rhythm. An early accurate diagnosis will make most European countries, blit even within possible parental choice, as well as providing the countries there is a great variation in the service opportunity to plan the delivery and postnatal provided. The recommendations provided in this management so as to optimize the outcome, document are intended to be guide for all paediatric Support can also be provided to specialists in fetal cardiologists undertaking fetal echocardiography with medicine, and to obstetricians, in the management the view of providing a service in fetal cardiology. It is of fetuses with functional disturbances, as in tWinclear that the health and legal systems vary from counto-tWin transfusion syndrome try to country, so that not all aspects of these recomb. To provide appropriate counselling and support mendations Can be implemented in all countries, The for parents and families following prenatal recommendations, nonetheless, provide a framework diagnosis l that can be adapted to fit in with local situations, c, To communicate results to the referring obstetri-

Natural history of 107 cases of fetal aortic stenosis from a European multicenter retrospective study

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 remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV.

Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum: A Report From the International Fetal Cardiac Intervention Registry

Infants with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum (HLHS-IAS) represent a high-risk subset. Fetal cardiac intervention (FCI) for fetuses with HLHS-IAS has been reported in single-institution series as a therapy that may improve outcomes. This study uses the International Fetal Cardiac Intervention Registry (IFCIR) to examine fetal and maternal characteristics and pregnancy and neonatal outcome data for FCI in this population. For this descriptive analysis, the IFCIR was queried for fetuses with HLHS-IAS evaluated between 2001 and March 2015 for possible FCI and felt to be candidates at the referral center by a variety of published criteria including foramen ovale (FO) size, shunt flow direction across the FO, and pulmonary venous Doppler flow patterns. It includes those who were determined not to be candidates because of other fetal noncardiac conditions, maternal conditions, or maternal preferences, or because FCI was not performed at the participating institution. Further details on the methodology of the IFCIR registry are found in the initial report.1 Participating IFCIR member sites obtained local institutional review board and ethics board approval or a waiver, as governed …

Fetal aortic valvuloplasty: investigating institutional bias in surgical decision-making

Fetal aortic valvuloplasty may prevent the progression of aortic stenosis to hypoplastic left heart syndrome and allow biventricular rather than univentricular postnatal treatment. This study aimed to investigate whether blinded simulation of a multidisciplinary team approach aids interpretation of multicenter data to uncover institutional bias in postnatal decision‐making following fetal cardiac intervention for aortic stenosis.

Successful surgical treatment of a neonate with prenatal diagnosis of severe Ebstein's anomaly.

We present a case of a patient with a severe form of Ebstein’s anomaly diagnosed in a 36th week of gestation through prenatal echocardiographic evaluation. The emergency surgery consisted of closure of the tricuspid valve and creation of a residual atriventricular communication, enlargement of interatrial communication, closure of the pulmonary artery, and construction of an aortopulmonary shunt. Subsequently, the patient underwent the hemiFontan and Fontan operation. We conclude that prenatal echocardiographic diagnosis is crucial for the survival of children with a severe form of Ebstein’s anomaly, which is a neonatal emergency and requires prompt decision mating to initiate surgical management.

Isolated Atrioventricular Block in the Fetus

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 <20 weeks, ventricular rate ≤50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 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 (23.4±2.9 versus 24.9±4.9 weeks; P =0.02), 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 <20 weeks, ventricular rate ≤50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen. # Clinical Perspective {#article-title-22}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 <20 weeks, ventricular rate ⩽50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 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 (23.4±2.9 versus 24.9±4.9 weeks; P=0.02), 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 <20 weeks, ventricular rate ⩽50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Fetal hemodynamic response to aortic valvuloplasty and postnatal outcome: a European multicenter study

Fetal aortic stenosis may progress to hypoplastic left heart syndrome. Fetal valvuloplasty (FV) has been proposed to improve left heart hemodynamics and maintain biventricular (BV) circulation. The aim of this study was to assess FV efficacy by comparing survival and postnatal circulation between fetuses that underwent FV and those that did not.

OC216: Evaluation of 36 fetuses with hypoplastic left heart syndrome (HLHS) and comparison between classical and modified Norwood procedure

possible benefit we evaluated the value of different indications, the complication rate and the time span of drains in situ. Methods: We made a survey of all level III ultrasound centres of German-speaking countries from 1993 to 2001. Six level III centres returned the questionnaire: forty-seven feto-amniotic shuntings were performed in 30 fetuses [megacystis in 18 fetuses (three of these with urinary ascites), Hydrothorax in eight fetuses, hydronephrosis in two fetuses, cystic adenomatoid malformation of the lung in one fetus, ovarian cyst in one fetus]. Results: The median gestational age at time of shunting was 23.5 (range 16–33) weeks, at time of delivery 35 (range 23–41) weeks. The median time span of drains in situ was 19 (range 0–170) days. Altogether 18 of 30 fetuses (60%) had a benefit of feto-amniotic shunting. Conclusions: The best possible selection of pregnancies who might profit of feto-amniotic shunting is required. The deciding criteria are the underlying defect, the severity and progression of the disorder.