Development Of Hydrops Fetalis Research Articles

Maternal Red Blood Cell Alloimmunization Managed with Intrauterine Blood Transfusion: Predictors of Poor Outcome

Background: The rhesus (Rh) and Kell blood group systems are the most common of over 50 different antigens capable of causing maternal red blood cell (RBC) alloimmunization and severe fetal hemolytic disease. Anti-K and anti-D are responsible for a significant proportion of fetal anemia requiring intrauterine transfusion (IUT). Whilst IUT of packed RBCs improves neonatal survival and morbidity, clinical prognostic indicators are lacking. Our primary objective was to identify predictors of adverse outcome. Methods: We conducted a retrospective single-center study at Mount Sinai Hospital (MSH), Toronto, Canada. All pregnant patients alloimmunized with anti-K and anti-D as a single antibody, between 1991 and 2018 were included. Data were obtained from patient medical records, ultrasound reports and information from the transfusion medicine laboratory. Data included maternal demographics, antibody titers, pregnancy history, number of IUTs, hemoglobin (HB) concentration at the beginning and end of all IUTs. Neonatal outcomes included survival, mode of delivery, gestational age at delivery, birth weight, HB at birth and need for neonatal transfusion, phototherapy or intravenous immunoglobulins (IVIG). Our primary outcome was the composite outcome of stillbirth or neonatal death (SB/NND). We also constructed a secondary outcome consisting of top-up neonatal transfusion, exchange transfusion, phototherapy, or use of IVIG. Medians and interquartile ranges (IQR) or mean±SD were used as summary statistics and compared by Mann-Whitney or t-test; p<0.05 was statistically significant. Outcome predictors were identified using multivariable logistic or linear regression analysis; accounting for patient level clustering by marginal Generalized Estimating Equation did not significantly affect variables. Data were analyzed using SPSS. Results: 116 women with 128 pregnancies and 425 IUTs with anti-K or anti-D as a single antibody were identified. Median maternal age was 31 years (27.0-35.0) for anti-K and 32 years (23.6-40.6) for anti-D. The gestational age at 1st IUT differed significantly between anti-K and anti-D (24.3 vs 28.7 weeks respectively, p=0.004). Women with anti-K antibodies required more IUTs than women with anti-D (3.84 vs 3.12 IUTs, p=0.036) and HB at 1st IUT was significantly lower in the anti-K group (5.10 vs 7.05 g/dL, p=0.001) (Table 1). Following initiation of IUT, the time from 1st IUT to delivery was 69.6 days in the anti-K group and 54.6 in the anti-D group (p=0.06). The daily decrease of HB between 1st and 2nd IUT (as a marker of disease severity), development of fetal hydrops and severe preterm birth did not differ significantly between the two groups. Mean gestation age at delivery was 35.0 weeks in the anti-K and 36.0 weeks in the anti-D group (p=0.28), with 87.1% and 93.9% survival (p=0.37), respectively. The proportion of neonates requiring phototherapy, IVIG and exchange/top-up transfusion was comparable across the two antibody groups (Table 1). Regression analysis showed that delivery occurred sooner if HB dropped more rapidly between the first two IUTs (p=0.01). Each additional transfusion gained on average 22.5 days in utero (Table 2). In multivariable analysis, gestational age at 1st IUT was the only predictor of a SB/NND outcome (adjusted OR 0.79 [95%CI 0.67-0.93]; p=0.006). With 1st IUT at 23 weeks, the risk of SB/NND was 8%, but only 2.5% at 28 weeks and <0.01% at 36 weeks (Table 3). Gestational age at delivery (adjusted OR 1.11 [95%CI 1.00 to 1.23]; p=0.046) and HB at 1st IUT (adjusted OR 0.84 [95%CI 0.72 to 0.99]; p=0.038) were the only predictors of a composite adverse blood product requirement/intervention outcome (Table 4). The odds of blood product requirement/intervention postnatally at 1st IUT (median HB at 1st IUT of 6.6g/dL) at 28 weeks were 43% and increased by a further 38% for delivery at 34 weeks. Conclusion: The earlier in gestation that IUTs are implemented, the higher the odds of a SB/NND; however the later the gestation at delivery, the greater the odds of the neonate requiring blood products post-partum. The greater the HB drop between the 1st and 2nd IUT, the shorter the 'time between the first IUT and delivery', which increases the odds of a SB/NND outcome. Disclosures Garbowski: Vifor Pharma: Consultancy, Membership on an entity's Board of Directors or advisory committees; Imara: Consultancy. Shehata:Ferring: Honoraria.

Successful Management of Severe Idiopathic Fetal Hydrops With Thoracoamniotic Shunts [34P

INTRODUCTION: This case report describes successful management of severe idiopathic fetal hydrops with thoracoamniotic shunts. METHODS: Patient is a 38 y/o G2P0010 who initiated care at 6 weeks. She was evaluated by perinatology for AMA and a fibroid uterus. First trimester and anatomy ultrasounds were unremarkable. She had low-risk genetic screening and normal MSAFP. A growth ultrasound at 23 weeks demonstrated bilateral pleural effusions. Evaluation included: normal fetal echocardiogram, Rh positive with no antibodies, negative serum TORCH titers, normal microarray, and negative amniotic fluid TORCH PCR. Due to worsening bilateral pleural effusions and development of fetal hydrops, patient underwent left-sided thoracoamniotic shunt placement at 25 and 26 weeks after receiving antenatal corticosteroids with resolution of left-sided pleural effusion and hydrops. She was admitted at 31 weeks for re-accumulation of left-sided pleural effusion and administration of rescue corticosteroids. At 32 weeks the fetus was again noted to have fetal hydrops and delivery was recommended. A primary LTCS was performed due to nonreassuring fetal heart tones. Patient delivered a female fetus weighing 1845 gram with APGARS of 2/7. The neonate spent 25 days in NICU with no evidence of pulmonary hypoplasia and was discharged home in stable condition. There was no identified etiology for hydrops and neonate was thriving at 6 week postpartum visit. CONCLUSION: Fetal intervention in a severe case of fetal hydrops can lead to an excellent neonatal outcome. Placement of thoracoamniotic shunts allowed for fetal lung development in setting of severe pleural effusions and allowed an additional 6 weeks of intrauterine growth.

368. Current practices in maternal monitoring during antiarrhythmic treatment for fetal SVT: A scoping review

Background In pharmacologic management of fetal supraventricular tachycardia (SVT), antiarrhythmic agents are administered to the pregnant patient serving as a vehicle of transplacental delivery to the fetus. Given that antiarrhythmic therapy can have profound maternal adverse effects, administration of initial loading and dose adjustments should involve deliberate attention to pharmacologic principles with close maternal monitoring for adverse effects. We reviewed current monitoring practices and maternal complications of transplacental antiarrhythmic therapy for fetal SVT. Methods A query of the Embase database for search terms ”Fetal” or ”Foetal” and ”Supraventricular Tachycardia” or ”SVT” yielded 93 titles, of which 19 articles detailing the modalities of therapy for Fetal SVT were reviewed. Results The 19 articles represented 565 cases of fetal SVT, 111 of which had developed hydrops fetalis. Of these cases, 184 received a course of digoxin alone and 33, 56, and 5 cases received a combination therapy of digoxin with flecainide, sotalol, or amiodarone. Flecainide, Sotalol, and amiodarone were given as monotherapy in 89, 77, and 6 cases, respectively. Maternal monitoring information was available in 15 studies. Investigators performed electrocardiograms (EKGs) at baseline and during follow-up in 12 studies, and monitored electrolytes in five. Two studies reported a mandatory cardiologist consultation for all mothers and two studies considered performing echocardiography in select mothers. Digoxin and flecainide levels were measured in eight and four studies, respectively. Toxicity was reported in 5 patients: two on digoxin monotherapy, two on flecainide monotherapy, and one on combination of digoxin and flecainide. Conclusion Pharmacologic management of fetal SVT is generally safe for mothers with normal baseline cardiac assessments and close monitoring of symptoms, EKG, electrolytes, and/or drug levels. Future prospective studies on the treatment of fetal SVT should also include a systematic monitoring strategy for mothers, with consistent reporting of adverse reactions.

Second-trimester maternal serum screening for fetal Down syndrome: As a screening test for hemoglobin Bart's disease: A prospective population-based study.

The purpose of this study is to determine the effectiveness of second-trimester maternal serum screening for Down syndrome as a screening test for fetal hemoglobin (Hb) Bart's disease among an unselected population. A secondary analysis of a large prospective database (20254 pregnancies) was conducted to compare the levels of maternal serum screening, alpha-fetoprotein (AFP), free beta-human chorionic gonadotropin, and unconjugated estriol between pregnancies with Hb Bart's disease and unaffected pregnancies. The median AFP levels were much higher among affected fetuses (1.96 vs 1.12 multiple of the median; P<.001), yielding a sensitivity of 81.6% and specificity of 86.4%. Thus, AFP measurement is effective in predicting fetal Hb Bart's disease among an unselected population when using a cutoff value of 1.5 multiple of the median. The serum free beta-human chorionic gonadotropin levels were slightly, but significantly, higher in the affected pregnancies, while the serum unconjugated estriol levels were minimally, but significantly, lower among the affected pregnancies. Second-trimester maternal serum AFP levels were significantly elevated in cases of fetal Hb Bart's disease. Pregnancies with unexplained elevated serum AFP levels in areas of high prevalence of Hb Bart's disease should always undergo a detailed ultrasound examination to detect any early signs of fetal anemia before development of hydrops fetalis.

NON-IMMUNE HYDROPS FETUS IN THE THIRD TRIMESTER: THE EXPERIENCE OF DIAGNOSIS AND TREATMENT TACTICS DELIVERY INFANT

Presented clinical cases non-immune fetal hydrops diagnosed in the third trimester of pregnancy. Analyzed etiological faktory non-immune fetal hydrops, diagnosis and tactics of pregnancy and childbirth, infant treatment non-immune fetal hydrops. Established that cases of non-immune fetal hydrops met in 0.05% of cases, the total number of births. In 50% of etiological factor in the development of fetal hydrops was intrauterine infection. The successful outcome of infants with non-immune fetal hydrops was 33.3%.

An Unusual Hydrops Fetalis Associated with Compound Heterozygosity for Krüppel-like Factor 1 mutations

Hydrops fetalis is commonly due to Hb Bart’s (γ4) disease in South East Asia. Here, we report an unusual case of hydrops fetalis due to congenital dyserythropoietic anemia (CDA) associated with compound heterozygosity for Krüppel-like factor 1 (KLF1) gene mutations. Fetal cardiomegaly was first detected on routine mid-trimester scan in a pregnant woman with normal mean corpuscular volume (MCV) and Rhesus positive status. The fetus subsequently developed hydrops fetalis, and cordocentesis showed severe fetal anemia with a hemoglobin (Hb) level of 3.4 g/dL. Common causes of fetal anemia including Hb Bart’s disease, parvovirus infection, and red cell antibodies were excluded. In view of the marked increase in erythroblasts at various stages of erythropoiesis, the diagnosis of CDA was suspected. We screened the couple for previously reported KLF1 gene mutations, showing that the mother was heterozygous for the c.525_526insCGGCGCC, p.Gly176Argfs*179 mutation, and her husband heterozygous for c.1012C>A, p.Pro338Thr mutation. The fetus was a compound heterozygote for these two KLF1 mutations. After counseling, repeated intrauterine transfusions were given at 27, 29, and 34 weeks’ gestation; the hydrops fetalis was resolved. The baby was delivered at 34 weeks’ gestation and required monthly blood transfusions but was otherwise thriving. Bone marrow aspiration at 10 months of age showed the features of ineffective erythropoiesis, compatible with CDA. In conclusion, hydrops fetalis can rarely be due to CDA associated with a compound heterozygous mutation for KLF1 gene mutations, and be managed by repeated intrauterine transfusions. Our present report adds to the wide clinical spectrum of KLF1 mutations.

Importance of Endogenous Atrial and Brain Natriuretic Peptides in Murine Embryonic Vascular and Organ Development.

Atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) bind to the receptor guanylyl cyclase (GC)-A, leading to diuresis, natriuresis, and blood vessel dilation. In addition, ANP and BNP have various angiogenic properties in ischemic tissue. When breeding mice devoid of GC-A, we noted significant skewing of the Mendelian ratio in the offspring, suggesting embryonic lethality due to knockout of GC-A. Consequently, we here investigated the roles of endogenous ANP and BNP in embryonic neovascularization and organ morphogenesis. Embryos resulting from GC-A(-/-) × GC-A(+/-) crosses developed hydrops fetalis (HF) beginning at embryonic day (E)14.5. All embryos with HF had the genotype GC-A(-/-). At E17.5, 33.3% (12 of 36) of GC-A(-/-) embryos had HF, and all GC-A(-/-) embryos with HF were dead. Beginning at E16.0, HF-GC-A(-/-) embryos demonstrated poorly developed superficial vascular vessels and sc hemorrhage, the fetal side of the placenta appeared ischemic, and vitelline vessels on the yolk sac were poorly developed. Furthermore, HF-GC-A(-/-) embryos also showed abnormal constriction of umbilical cord vascular vessels, few cardiac trabeculae and a thin compact zone, hepatic hemorrhage, and poor bone development. Electron microscopy of E16.5 HF-GC-A(-/-) embryos revealed severe vacuolar degeneration in endothelial cells, and the expected 3-layer structure of the smooth muscle wall of the umbilical artery was indistinct. These data demonstrate the importance of the endogenous ANP/BNP-GC-A system not only in the neovascularization of ischemic tissues but also in embryonic vascular development and organ morphogenesis.

OP18.03: Neurological outcome of children who were treated for fetal tachycardia complicated by hydrops

Fetal tachycardia is a condition associated with congestive heart failure and development of fetal hydrops which may result in morbidity or death. The aim of this study was to investigate the long-term outcome of hydropic fetuses. Retrospective cohort study. We studied all cases with fetal hydrops after tachycardia treated in our clinic. We identified 27 cases. At the time of this abstract data on 18 cases were available. Six fetuses had atrial flutter and twelve supraventricular tachycardia. Twelve children with normal neurological outcome, three with neurological morbidity and three perinatal death. In this study, neurological outcome was good in 12 out of 18 infants.

Prenatal management of fetal supravevtricular tachycardia

Introduction: Fetal cardiac arrhythmias are discovered in about 1% of fetuses and most of them are supraventricular tachycardia (SVT). Persistent tachycardia with a fetal heart rate (FHR) more than 220 bpm could lead into congestive cardiac failure and can manifests as non-immune fetal hydrops. With the rapid advancement in fetal echocardiographic skills, such arrhythmias can be diagnosed accurately during prenatal life by using M-mode echocardiogram and Doppler. Case 1: A 28 year old primigravida was referred from Jaffna due to fetal tachycardia and ascites detected at routine scan at 27 weeks. Detailed anomaly scan including cardiac evaluation was performed after admission to our unit and SVT with a heart rate of 278 bpm with hydrops fetalis found with no other gross anomalies. Mother was started on digoxin 0.25mg twice a day and changed in to sotalol 80mgtwice a day after 48 hours. Sotalol omitted and flicanide 100mg twice a day started after 4days. Because of persistently high FHR Cordocentesis done and 30µg of digoxin injected to umbilical vein and 4 days later amioderone 5mg injected. Subsequently FHR decreased to 137 bpm. Case 2: A 38 year old, mother of one was transferred from Badulla due to fetal tachycardia at 36 weeks. Scan at our unit revealed Ebstein anomaly of fetus lead to re-entral tachycardia. Atrial rate was 338 bpm and ventricular rate was 168 bpm. Baby delivered electively and admitted to SCBU. Discussion: Fetal SVTs may be associated with adverse perinatal outcome. Development of fetal hydrops is a poor prognostic factor and placental oedema reduces transfer of drugs to fetus. Early identification and intervention (before development of hydrops) will lead to better perinatal outcome.

Amicrobial Pustulosis of the Folds

<b>Background:</b> Maternofetal parvovirus B19 infection may result in fetal hydrops or abortion. Chronic infection has been associated with long term complications (polyarthritis, persistent aplastic anaemia, hepatitis). In pregnancy maternal immunosuppression caused by a TH2 dominant response to viral antigens has been observed. There is little information on long term reactivity to intrauterine infection. <b>Aims:</b> To assess the serological status in children and their mothers after maternofetal parvovirus B19 infection and development of fetal hydrops. <b>Methods:</b> A total of 18 children and their mothers, and 54 age matched control infants were studied. Main outcome measures were parvovirus B19 DNA, specific IgM and IgG against the virus proteins VP1/VP2, and NS-1 in venous blood. <b>Results:</b> Parvovirus B19 DNA and antiparvovirus B19 (IgM) were undetectable in all sera. A significant larger proportion of maternal sera compared to study children’s sera contained IgG against the non-structural protein NS-1. Mean levels of VP1/VP2 IgG antibodies were significantly lower in the children than in their mothers (48 (36) <i>v</i> 197 (95) IU/ml). There was no history of chronic arthritis in mothers and children. Five women had subsequent acute but transient arthritis postpartum, which was not correlated with antibodies against NS-1. <b>Conclusions:</b> Serological evidence of persistent infection after maternofetal parvovirus B19 disease could not be detected. Increased maternal prevalence of anti NS-1 (IgG) and increased levels of antiparvovirus B19 (IgG) may reflect prolonged viraemia compared to fetal disease.

Prenatal diagnosis and outcome of fetal lung masses

Aim The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses. Methods The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome. Data were analyzed for predicting development of hydrops or the need for fetal therapy using receiver operating characteristic curves. Results Of 82 fetuses (41 male) evaluated for a lung mass, 53 (65%) were left-sided (1 bilateral), and the mean (SD) GA at diagnosis was 21.5 (4.3) weeks. Seventy-three fetuses underwent fetal magnetic resonance imaging at a mean (SD) GA of 26.1 (4.6) weeks. Thirteen fetuses (16%) had fetal treatment. Four fetuses with hydrops underwent open fetal surgical resection, and 3 survived. Six fetuses with large lung masses and persistent mediastinal compression near term underwent ex-utero intrapartum therapy-to-resection procedures, and 3 fetuses with hydrops underwent serial thoracentesis. Congenital cystic adenomatoid malformation volume ratio correlated strongly with the development of hydrops and the need for fetal therapy with an area under the receiver operating characteristic curve of 0.96 ( P < .0001) and 0.88 ( P < .0001), respectively. Of 18 fetuses with a CVR greater than 2.0 compared with 2 (3%) of 60 with a CVR of 2.0 or less, 10 (56%) required fetal intervention ( P < .0001). Conclusion Congenital cystic adenomatoid malformation volume ratio correlates strongly with the development of fetal hydrops and the need for fetal intervention. A threshold value of 2.0 yields the most powerful statistical results.

Parvovirus B19 Infection

Primary parvovirus B19 infection is not uncommon during pregnancy, and is often asymptomatic in the mother (de Jong et al, J Clin Virol. 2006;36:1–7). Vertical transmission to the fetus occurs in roughly 30% of patients with primary infection (Koch et al, Pediatr Infect Dis J. 1998;17:489–494). The risk of an adverse fetal outcome following parvovirus B19 infection is low; however, severe infections can lead to fetal hydrops and intrauterine fetal demise (de Jong et al, J Clin Virol. 2006;36:1–7; Koch et al, Pediatr Infect Dis J. 1998;17:489–494; Young et al, N Engl J Med. 2004;350:586–597). Diagnosis of parvovirus B19 infection can be made by identification of the characteristic findings within the placenta, including a grossly hydropic placental disc and the presence of intranuclear inclusions within fetal red blood cells (Koduri et al, Am J Hematol. 1998;58:95–99; Quemelo et al, Inst Med Trop Sao Paulo. 2007;49:103–107). Similar inclusions can be seen in various tissues of fetuses who experience an intrauterine demise and present for autopsy (Rodriguez et al, Pediatr Dev Pathol. 2002;5:365–374; O'Malley et al, Pediatr Dev Pathol. 2003;6:414–420). The current case report illustrates an example of parvovirus B19 infection in a pregnant woman whose fetus subsequently developed hydrops fetalis and died in utero. The accompanying placental and fetal pathology depict classic findings of parvovirus B19 infection. The differential diagnosis of parvovirus B19 infection in a placental specimen is also discussed.

Prenatal diagnosis of idiopathic infantile arterial calcification with hydrops fetalis

Idiopathic infantile arterial calcification (IIAC) is a rare and nearly always fatal disorder. To date, prenatal diagnosis has been reported in fewer than 10 cases. We describe a series of three cases in which the diagnosis of IIAC was made at 23, 25 and 29 weeks' gestation. All three cases presented with a normal anatomy scan at 20 weeks' gestation with an echogenic intracardiac focus. Follow-up scans showed generalized hyperechogenicity and calcification of the walls of the large arteries, particularly the aorta and the iliac arteries. All cases developed hydrops fetalis with cardiomegaly and polyhydramnios later in gestation, resulting in intrauterine fetal death in two cases and neonatal death immediately following delivery in the third. This is the largest case series and the earliest gestational age of prenatal diagnosis of IIAC reported to date. When surveying for the disease, serial scans are important, perhaps from 20 weeks' gestation, with close examination of the iliac and aortic arteries. Detection of echogenic intracardiac focus could be an early marker in patients with a family history of the disease.

A Survey of Pregnancies with Hb Bart's Disease in Mainland China

α-Thalassemia (α-thal) is a common inherited disease in southern China. The severest form is Hb Bart's disease, in which the affected individuals are lethal in utero or shortly after birth, and the pregnancies are at high risk for severe morbidity. A survey by us revealed a total of 68 cases of Hb Bart's disease during the past 7 years at a maternal hospital and none of these showed that a risk of thalassemia had been recognized prior to the development of hydrops fetalis. The number of cases of Hb Bart's disease did not vary significantly during the study period. This means that the prevention program for α-thal has not been efficient. More effort is needed to efficiently control this disease in our region.

Survival of a profoundly hydropic fetus with a sacrococcygeal teratoma delivered at 27 weeks of gestation for maternal mirror syndrome

The development of hydrops fetalis and maternal mirror syndrome in the setting of fetal sacrococcygeal teratoma (SCT) carries a grave prognosis. Fetal surgery is not typically offered for hydropic fetuses beyond 26 weeks of gestational age. Few options exist for these families, and they are typically counseled to continue pregnancy until delivery is necessary for maternal indications or the fetus is 30 weeks old. Although a number of series report the survival of hydropic SCT infants delivered after 30 weeks of gestation, a paucity of information is available regarding the outcome of hydropic SCT infants delivered between 26 and 30 weeks of gestation. We now report successful postpartum resection and survival of a hydropic fetus delivered at 27 weeks for maternal mirror syndrome that may assist in counseling families in similar situations.

A case of galactosialidosis with a homozygous Q49R point mutation

Galactosialidosis is a rare lysosomal storage disease caused by a combined deficiency of lysosomal β-galactosidase and neuraminidase, due to a primary defect in protective protein/cathepsin A. Three subtypes are recognized: the early infantile type, the late infantile type, and the juvenile/adult type. Here, we report a case of early infantile galactosialidosis in a female who was born at 31 weeks of gestation, after detection of fetal ascites at 21 weeks of gestation and development of fetal hydrops. After birth she received intensive treatment that led to improvement of edema and pleural effusion, but ascites slowly developed. She died of renal failure on day 207. An autopsy showed that all organs contained vacuolated cells, compatible with a storage disease. The patient had decreased activity of β-galactosidase and undetectable neuraminidase activity in fibroblasts. A single A–G base transition at position 146 of exon 1 (Q49R) in protective protein/cathepsin A gene was found. The mutation has been reported previously in a Japanese patient with different phenotypes. However homozygous Q49R mutation detected in our case was severe prognosis.

Isolated congenital atrioventricular block diagnosed in utero: Natural history and outcome

Background. Isolated congenital atrioventricular block (CAVB) diagnosed in utero is associated with a high morbidity and mortality. Prognosis is especially poor when heart rate drops below 55 beats per minute (bpm) and when fetal hydrops develops. We describe the natural history and outcome of 24 infants with isolated CAVB diagnosed in utero, review the literature, and assess the risk factors that could predict outcome.Methods. This was a retrospective multicenter study of 24 patients with isolated CAVB diagnosed in utero.Results. CAVB was detected at a mean gestational age (GA) of 24.7 ± 5.1 weeks. Ten fetuses initially presented with complete heart block. Low heart rate or incomplete heart block was the first documentation of bradyarrhythmia in the other 14 fetuses. In 11 of them, CAVB developed during pregnancy after a median time of 3 (range 1–16) weeks. Fetal hydrops developed in 10 of 24 (42%) fetuses at a mean GA of 27.6 ± 5.1 weeks. Hydropic fetuses showed lower heart rates during pregnancy (47 ± 10 bpm) than non-hydropic fetuses (57 ± 10 bpm). There were three intrauterine deaths; all were hydropic and female. Nine viable females and 12 males were born at a mean GA of 37.1 ± 6.1 weeks with an average birth weight of 3097 ± 852 g. Fifteen CAVB patients required pacemaker (PM) intervention, 10 of them immediately after birth. Dilated cardiomyopathy (DCM) developed in three infants of whom two died of congestive heart failure, shortly after the diagnosis was made; one is still alive. Mortality before or after birth was 21%, and was associated with heart rates below 50 bpm and development of fetal hydrops. Poor outcome, defined as death, PM implantation, or development of DCM, occurred in 83% of cases and was associated with heart rates below 60 bpm during pregnancy.Conclusions. Isolated CAVB diagnosed in utero is associated with high morbidity and mortality. Patients who develop fetal hydrops show lower heart rates during pregnancy than patients who do not. A fetal heart rate below 50 bpm and development of fetal hydrops is associated with increased mortality. Rates below 60 bpm are associated with PM requirement and/or DCM.

High Rate of Severe Fetal Outcomes Associated with Maternal Parvovirus B19 Infection in Pregnancy

Objective. To augment the understanding of parvovirus B19 infection in pregnancy with respect to maternal characteristics and their corresponding fetal outcomes. Study Design. Retrospective case-series of all women referred to Magee-Women_s Hospital with serologically-documented parvovirus B19 infection during pregnancy from 1998–2001. Results. All 25 cases that are available for analysis occurred from January through June. The frequency of cases varied substantially from year to year, with 14 cases in 1998, 0 cases in 1999 and 2000, and 11 cases in 2001. In contrast to previous reports, the minority of women [4/25(16%)] experienced symptoms attributable to parvovirus B-19 infection although 3 of 25 (12%) fetuses developed hydrops fetalis and 4/25 (16%) suffered an intrauterine of fetal death. Conclusions. These findings suggest that parvovirus B19 infection in pregnancy follows seasonal and annual trend variation, may produce a lower frequency of maternal symptoms and a higher fetal loss rate than previously reported. Synopsis. Maternal parvovirus B19 infection follows seasonal and annual variation is often asymptomatic and may have higher fetal loss rates than previously reported. Continued surveillance is warranted.

Neurological outcome of children who were treated for fetal tachycardia complicated by hydrops.

Fetal tachycardia is a condition associated with congestive heart failure and development of fetal hydrops, which may result in neurological morbidity and mortality. The aim of this study was to investigate the long-term outcome of hydropic fetuses. This was a retrospective study on cognitive and neurological functioning of 11 infants, aged 6 months to 12 years, who experienced fetal tachycardia complicated by hydrops. Seven fetuses had supraventricular tachycardia (SVT), three had atrial flutter (AF) and one had ventricular tachycardia (VT). Nine fetuses converted to sinus rhythm within a mean time of 8.2 days of presentation; resolution of hydrops was achieved in six of these patients in a mean time of 8.8 days. Mean gestational age (GA) at birth was 35 + 4 weeks. Neonatal cranial ultrasound was normal in seven infants and all but one of these were normal at follow-up: one infant who initially had no abnormalities developed multiple cerebral lesions as a result of a malignant long QT syndrome (LQTS) and died at the age of 2 years. Three infants had periventricular echogenicity (PVE) on neonatal cranial ultrasound, associated with a pseudocyst in one infant. The remaining infant showed a parenchymal hemorrhage of antenatal onset, seen as a porencephalic cyst at birth. One of these infants was normal at follow-up, one died 2 days after birth and two infants had neurological abnormalities at follow-up, consisting of mild hemiplegia with normal cognitive function in one, and a cognitive developmental delay in the other. In this study, neurological outcome was good in eight out of 11 infants. Initiation of therapy should not be withheld or delayed on the assumption of poor neurological outcome.

Congenital cervical fibrosacroma with hydrops fetalis

Congenital cervical fibrosacroma with hydrops fetalis