Brigitte Strizek

High-dose flecainide is the most effective treatment of fetal supraventricular tachycardia

BACKGROUND Fetal tachyarrhythmia can lead to fetal hydrops due to heart failure. Flecainide is often considered as second-line therapy when digoxin monotherapy fails, which is more likely in hydropic fetuses. Time to conversion to sinus rhythm (SR) is critical in cases presenting with hydrops. OBJECTIVE The aim of this study was to evaluate the efficacy and time to conversion to SR of transplacental treatment, especially flecainide. METHODS This is a retrospective observational study of 46 fetuses with fetal tachyarrhythmia. Treatment was either flecainide (n = 28, 60.9%), digoxin+flecainide combination (n = 4, 8.7%), or digoxin (n = 10, 21.7%). In 4 fetuses (8.7%), no treatment was necessary. RESULTS In our study population, 26 of the 32 fetuses (81.2%) that were treated with flecainide as a first-line therapy (flecainide or digoxin+flecainide) converted to SR. The median time to conversion to SR was 3 days (range 1-7 days) with flecainide monotherapy and 11.5 days (range 3-14 days) with a combination therapy. Seventy-two percent (13/18) of hydropic fetuses and 90% (9/10) of nonhydropic fetuses converted to SR when treated with flecainide monotherapy. There was no statistical difference in rates of conversion to SR in hydropic and nonhydropic fetuses (P = .37) or time to conversion to SR in the 2 groups (P = .9). In the majority of the remaining fetuses, there was a partial response with decreased ventricular heart rates that were well tolerated. CONCLUSION Flecainide is highly effective in achieving SR in hydropic and nonhydropic fetuses with supraventricular tachycardia in a median time of 3 days. In our opinion, flecainide should be considered as first-line therapy in fetal supraventricular tachycardia with and without hydrops.

Extracardiac anomalies in prenatally diagnosed heterotaxy syndrome.

To assess the incidence and impact of extracardiac anomalies on the prognosis of fetuses with heterotaxy syndrome.

Prenatal diagnosis of absent pulmonary valve syndrome from first trimester onwards: novel insights into pathophysiology, associated conditions and outcome

To assess the spectrum of associated anomalies, intrauterine course and outcome in fetuses with absent pulmonary valve syndrome (APVS).

First‐trimester intervention in twin reversed arterial perfusion sequence: does size matter?

To evaluate the outcome of first‐trimester intervention (12 + 0 to 14 + 0 weeks of gestation) in pregnancies complicated by twin reversed arterial perfusion (TRAP) sequence.

Prenatal diagnosis of absent pulmonary valve syndrome from the 1(st) trimester onward: novel insights into pathophysiology, associated conditions and outcome of a rare cardiac defect.

To assess the spectrum of associated anomalies, intrauterine course and outcome in fetuses with absent pulmonary valve syndrome (APVS).

Re: Fetal umbilical–portal–systemic venous shunt: in‐utero classification and clinical significance. R. Achiron and Z. Kivilevitch. Ultrasound Obstet Gynecol 2016; 47: 739–747

In their manuscript, Achiron and Kivilevitch describe a relatively large cohort of fetuses with anomalies of the hepatic venous circulation. The study deals only with shunts involving the umbilicoportal venous system as their origin and the systemic veins as their point of drainage. In contrast to their previous publications and those of other authors, a new classification system is used, based on the anatomical origin of the shunt. In Type I, umbilical–systemic shunt (USS), the umbilical vein (UV) connects directly to the systemic circulation without connection to the portal system; the ductus venosus (DV) is always absent and the intrahepatic portal venous system (IHPVS) is absent in most cases. Previously, this type has been referred to as DV agenesis with extrahepatic UV drainage1. In Type II, DV–systemic shunt (DVSS): the UV–portal–DV complex is normal, but the DV connection is slightly aberrant, being either to the inferior vena cava at a distance from the heart or to the hepatic veins; the DV and the IHPVS are always present. This type is a variation of the normal anatomy but has also been referred to as DV agenesis by some authors, who define the DV based on its exact connection site at the prediaphragmatic infundibulum, separate from the IVC and hepatic veins1,2. In Type IIIa, intrahepatic portal–systemic shunt (IHPSS), the UV–portal–DV complex and the IHPVS are normally developed, but there is a connection between portal and hepatic veins. The DV is missing in some cases, which previously have been referred to as DV agenesis with intrahepatic UV drainage1,2. In Type IIIb, extrahepatic portal–systemic shunt (EHPSS), parts of the main portal vein (splenic vein and/or mesenteric vein) drain directly into the systemic circulation. The IHPVS is either partly present or absent, as is the UV–portal–DV complex. The outcome in this study was determined mainly by the frequently associated additional anomalies and the absence of the IHPVS, although the rate of terminations was high. This new in-utero classification is an approach that takes into account that there are many more anomalies of the hepatic/UV/portal circulation than DV agenesis alone, which has previously been the starting point (and often the sole defining criterion) for the assessment of anomalies of the portal and hepatic venous systems. This point of view is certainly more detailed and anatomically correct, and therefore has its merits. It is about time to reappraise DV agenesis as a consequence of an altered hepatic/UV/portal circulation and not as a disease in itself, as the alterations of the hepatic circulation are more complex than has been perceived previously. These authors, who pioneered this field with numerous publications, have now taken an important step after their meticulous description of the normal and abnormal fetal venous system in the prenatal period3,4. However, the proposed classification is rather complex and probably difficult to understand for most prenatal diagnosticians (including these reviewers), who start evaluation of the hepatic circulation with assessment of the DV. The strength of this classification is the good discrimination of the postnatal course. Isolated cases of DVSS and IHPSS have a good prognosis as the IHPVS is present and the shunts have a high rate of spontaneous closure after birth. USS and EHPSS are strongly associated with absence of the IHPVS and, consequently, with significant morbidity. There are two main issues that will have an impact on the universal acceptance of this new classification. First, there is no uniformly accepted definition of DV. If the DV is defined as a vessel that connects the UV with the systemic circulation at any anatomical point, then there is no such thing as DV agenesis or USS. If the DV is defined as a connection of the hepatic/UV/portal complex to the prediaphragmatic infundibulum, separate from the IVC and hepatic veins, then there is no such thing as DVSS. Simcha Yagel, who authored or co-authored a major part of the previous publications of this group, just recently looked at it in another way: USS and DVSS were classified as DV agenesis and subdivided into types with broad or narrow connection to the systemic veins2,5. He hypothesized that only the narrow type of connection enables normal development of the portal circulation, as is confirmed in the cohort in this manuscript. Second, even the complex type of classification proposed has overlapping cases. Three of the cases in the EHPSS group had a direct connection of the UV to the systemic circulation and could also have been classified as USS. This underlines the complexity of anomalies concerning the hepatic/UV/portal circulation and the difficulty of creating a universal classification. The future will show whether the readers of Ultrasound in Obstetrics and Gynecology adopt this classification or instead propose new ones. C. Berg*†‡ and B. Strizek‡ †Division of Prenatal Medicine and Gynecologic Sonography, Department of Obstetrics and Gynecology, University of Cologne, Cologne, Germany; ‡Division of Fetal Surgery, Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany *Correspondence. (e-mail: christoph.berg@ukb.uni-bonn.de)

OC14.05: Comparison of fetal outcome in lower urinary tract obstruction treated with the Harrison® vs Somatex® vesicoamniotic shunt <17+0 weeks

Objectives: Reports on the effects of CO2 gas on human development are lacking. This information is relevant considering the advantages of fetoscopic NTD repair (Fetosc.) technique. Aims: 1) Assess the incidence of brain abnormalities after prenatal NTD repair; 2) Compare the incidence of brain abnormalities between open vs. fetoscopic repair. Methods: Longitudinal retrospective cohort study of 57 fetuses that underwent prenatal NTD repair (27 Fetosc. and 30 Open). Presurgery MRIs were obtained in all cases and 6 weeks postsurgery in 54 cases (26 Fetosc. vs. 28 Open). At 1 year, MRI scans from 22 open and 16 fetoscopic repaired infants were reviewed. A detailed assessment of all scans was performed. Results: GA at surgery, GA and age at MR scans were similar between groups. Hindbrain herniation (HBH) (all cases), ventricular dilation (Open:11±3 mm vs. Fetosc:11.4±3mm p=0.28), 4th ventricle effacement (Open:93% vs. Fetosc:93% p=0.1), callosal anomalies (Open:50% vs. Fetosc:41% p=0.5) and tectal beaking (Open:67% vs. Fetosc:70% p=0.8) were the most common findings detected prior to surgery. On follow-up scans, increase in ventricular dilation (6 weeks’ post-op: Open:15±6mm vs. Fetosc:16±4mm p=0.5; postnatal: Open:23±8 vs. Fetosc:27±11mm p=0.3), detection of callosal anomalies (6 weeks post-op: Open:57% vs. Fetosc:54% p=0.59; postnatal: Open:82% vs. Fetosc:75% p=0.69) and nodular heterotopia (presurgery: Open:3% vs. Fetosc:0% p=1.0; 6 weeks post-op: Open:11% vs. Fetosc:12% p=1.0; postnatal: Open:32% vs. Fetosc:19% p=0.36) were more common. Reversal of HBH was seen in 71% of the open and 58% of the fetoscopic cases after surgery. No signs of ischemia, parenchymal calcifications or cysts were detected in any scans. No differences in the incidence of brain anomalies were seen between groups. Conclusions: NTD is associated with brain abnormalities that are detected before and after prenatal repair, independently of the repair technique used. These results support the safety of CO2 exposure for prenatal NTD repair as it is not associated with detectable structural changes after surgery.

Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome

PURPOSE  To assess the anatomic variants, associated anomalies and postnatal outcome of fetuses with a prenatally diagnosed agenesis of ductus venosus (ADV). MATERIALS AND METHODS  Retrospective study of 119 cases with agenesis of ductus venosus diagnosed by prenatal ultrasound in two tertiary referral centers from 2006 to 2014. The type and location of the umbilical venous drainage site was noted. Charts were reviewed for associated structural or chromosomal anomalies, pregnancy outcome and postnatal course. RESULTS  In 24 cases (20.2 %) ADV was an isolated finding, while 95 cases (79.8 %) had associated anomalies. We identified 84 cases (70.6 %) with intrahepatic and 35 cases (29.4 %) with extrahepatic drainage of the umbilical vein. 58.8 % of neonates were alive at follow-up. There was no statistical association between drainage site and associated anomalies or outcome. Postnatal outcome was determined by the presence and severity of associated anomalies. There was no adverse outcome in the isolated group related to ADV. Overall, there were 6 persistent portosystemic shunts, 3 of them with a spontaneous closure, and one total agenesis of the portal venous system with lethal outcome. CONCLUSION  Postnatal outcome in cases with ADV mainly depends on the presence of associated anomalies. In isolated cases the prognosis is generally good, but neonates with a prenatally diagnosed portosystemic shunt should be followed until its occlusion. Portal venous system agenesis is rare but should be ruled out on prenatal ultrasound.

Outcome of Bronchopulmonary Sequestration with Massive Pleural Effusion after Intrafetal Vascular Laser Ablation

Objective: To assess the outcome of 12 fetuses with bronchopulmonary sequestration (BPS) and massive pleural effusion after intrafetal vascular laser ablation (VLA). Methods: All fetuses with BPS and massive pleural effusion that were treated with intrafetal VLA during a 5-year period (2012-2016) were reviewed for safety, intrauterine course, and postnatal outcome. Results: In the study period, 12 fetuses with BPS were treated with VLA. In 7 (58.3%) fetuses, complete cessation of blood flow was achieved after the first VLA, while in 5 (41.7%) fetuses, residual perfusion of the feeding vessel was demonstrated at follow-up. A second intervention was successful in 4 of 5 (80%) fetuses. Overall, in 11 of 12 (91.7%) fetuses, complete coagulation of the feeding vessel could be achieved, followed by a reduction in size or complete resolution of the BPS. All 11 fetuses with successful prenatal intervention were live-born at a median gestational age of 39+1 (range, 37+5-41+2) weeks. Postnatally, 2 (18.2%) of the 11 newborns underwent sequestrectomy, as well as the preterm newborn on which a second fetal intervention was not feasible. Conclusion: VLA is an effective and safe treatment of BPS that appears to be of benefit in improving prognosis and decreasing the need for postnatal sequestrectomy.