Tetralogy of Fallot: prediction of outcome in the mid-second trimester of pregnancy.

OBJECTIVE: To evaluate the usefulness of fetal echocardiography in the mid-second trimester in predicting postnatal outcome of tetralogy of Fallot (ToF), focusing on the need for early intervention (EI) and surgery type: pulmonary valve-sparing surgery (PVSS) versus placement of transannular patch (TAP). METHODS: Assessment of cardiac morphological and functional parameters in 23 live-born fetuses with isolated ToF was performed at 19 to 22 and 34 to 38 weeks. Comparisons were made between outcome groups (EI vs non-EI and PVSS vs TAP). EI was considered as requirement either of palliative procedure or corrective surgery before three months. RESULTS: Overall survival was 96%. EI was required in 32% of cases and TAP in 50%. At 19 to 22 weeks, a pulmonary valve peak systolic velocity (PVPSV) ≥87.5 cm/s predicted EI with 100% sensitivity and 93.3% specificity (p < 0.01). At 34 to 38 weeks, the size of the pulmonary valve, pulmonary valve/aortic valve and main pulmonary artery/ascending aorta were significantly different, but the PVPSV again yielded the best performance: all cases undergoing EI and/or TAP were selected using cut-off of ≥144.5 cm/s. CONCLUSION: The postnatal outcome of fetuses with ToF may be established using PVPSV from the mid-second trimester. This may be useful in providing the most appropriate perinatal management and accurate parental counselling.

Cardiopatía congénita y rabdomioma cardíaco fetal: una asociación inusual / Congenital heart disease and fetal cardiac rhabdomyoma: an unusual association

La asociación de cardiopatías congénitas con rabdomiomascardíacos fetales es infrecuente. Presentamos dosnuevos casos de diagnóstico prenatal de esta inusual asociación,y analizamos sus implicaciones para el asesoramientode los padres y el manejo perinatal(AU)

The association of congenital heart disease with fetalcardiac rhabdomyomas is uncommon. We report twonew cases of prenatal diagnosis of this unusual association,further discussing its implications for parentalcounseling and perinatal management(AU)

Application of a first-trimester prediction model for pre-eclampsia based on uterine arteries and maternal history in high-risk pregnancies.

OBJECTIVE: To assess the value of a prediction model for pre-eclampsia (PE) in the first trimester (Ultrasound Obstet Gynecol 2007;30:742-794) for the prediction of late (>34 weeks) and early (< or =34 weeks) PE in a high-risk population. METHODS: Longitudinal study performed in 152 high-risk pregnancies with at least one high-risk condition: previous PE, hypertension, pregestational diabetes, renal disease, obesity, hyperlipidemia, autoimmune disorders, thrombophilia or recurrent pregnancy loss. Mean uterine artery pulsatility index at 11 to 13 + 6 weeks and a series of maternal variables were combined in order to obtain the estimated 'a posteriori risk for PE' in each woman. This risk for unaffected women was compared with that for patients who subsequently developed late and early PE. The performance of such approach was described by receiver-operating characteristic curves. RESULTS: Late PE developed in 13 (8.6%) pregnancies and early PE in seven (4.6%). The median 'a posteriori risk for PE' in the unaffected, late PE, and early PE groups was 0.62%, 1.22%, and 2.49% (P < 0.01), respectively. For a false-positive rate of 10%, the detection rates of late and early PE were 23.1 and 42.9%, respectively. CONCLUSIONS: This referenced model shows a modest performance when applied to high-risk women.

Conotruncal anomalies in fetal life: accuracy of diagnosis, associated defects and outcome.

OBJECTIVES: To assess the accuracy of prenatal echocardiography, associated anomalies, and outcome of fetuses with conotruncal anomalies (CTA). STUDY DESIGN: We searched our database for CTA prenatally diagnosed between 1990 and 2005. We included tetralogy of Fallot (TOF), transposition of the great arteries (TGA), double-outlet right ventricle (DORV), truncus arteriosus (TA), pulmonary atresia with ventricular septal defect (PA-VSD) and posterior malalignment type VSD with aortic arch obstruction (pmtVSD-AAO). Data of 144 fetuses with complete follow-up were retrieved and analyzed. RESULTS: The main reason for referral was suspected heart defect on a routine obstetric scan (72%). Most cases were detected < or =22 weeks (55%). The presence of a CTA was confirmed postnatally in 143 cases (99%), and the diagnosis of the first fetal echocardiography was correct in 126 (87.5%). Most diagnosis of TOF (33/36, 91.7%), TGA (34/38, 89.5%) and DORV (34/38, 89.5%) were proved correct. Inadequate assessment of the interventricular septum, the distal aortic arch and/or the severity of the right outflow tract obstruction accounted for most errors. The accuracy rate was lower in TA (11/14, 78.6%) and PA-VSD (4/7, 57.1%), with evaluation of the branch pulmonary arteries as the main source of discrepancies. In 7/18 incorrect cases subsequent scans allowed to obtain a correct diagnosis. Most fetuses (64%) had an isolated CTA. Thirty-seven had chromosomal anomalies (26%) but none were found in TGA. 22q11 deletion affected 8.7% of the tested patients. Nuchal translucency (NT) was above 95th centile in 19/104 cases (18%) in which NT were measured. Fifty cases were interrupted. The overall one-year survival rate was 71%, with differences between cases with and without associated defects (9/25, 36% vs. 57/68, 83.8%; p<0.01). The uncomplicated forms of TGA and TOF had the best survival rates (100%). CONCLUSIONS: Most CTA can be diagnosed by fetal echocardiography with a high degree of accuracy. Chromosomal defects should always be ruled out, except for simple TGA. Current survival figures in many isolated CTA, especially simple TGA and TOF, support a change in the "classical" concept that congenital heart defects detected prenatally often have the worst outlook. CONDENSATION: Most CTA can be diagnosed by fetal echocardiography with a high degree of accuracy. Isolated CTA are more common and most of these may have a favourable outcome.

Valoración de la velocidad sistólica máxima de la arteria cerebral media en el manejo de la anemia fetal / Evaluation of the middle cerebral artery peak systolic velocity in the management of fetal anemia

Introducción. Se valora el uso en nuestro medio de lavelocidad sistólica máxima en la arteria cerebral media(VSM-ACM) en el manejo de pacientes con riesgo de anemiafetal.Métodos. Se estudiaron de manera retrospectiva 41 gestaciones con riesgo de desarrollo de anemia fetal controladas en nuestro centro entre los años 1998 y 2006. Se midió la VSM-ACM mediante velocimetría Doppler, estudiando su correlación con los niveles de hemoglobina fetal obtenida mediante cordocentesis, tanto en fetos transfundidos como en no transfundidos. Se incluyeron en el estudio anemias de tipo inmune y no inmune.Resultados. En 21 de los 41 casos evaluados se obtuvoun valor de la VSM-ACM sugestivo de anemia moderada osevera, confirmándose el diagnóstico en el 76,2% de los casos. Utilizando los puntos de corte publicados previamente para la predicción de anemia fetal se obtuvo una sensibilidad del 100% y una especificidad del 83,3% para el diagnóstico de anemia moderada, siendo del 94,4 y el 83,3%, respectivamente, para el de anemia severa. En fetos previamente transfundidos la sensibilidad fue del 100% y la especificidad del 88,5 % en el diagnóstico de anemias moderadas, siendo la sensibilidad del 58,8 % y la especificidad del 96,2 % para la detección de anemias en grado severo. La mortalidad perinatalen nuestro estudio se situó en un 12,5%. En los reciénnacidos el desarrollo neurológico a largo plazo fue normal, con un único caso de sordera neurosensorial.Conclusiones. El presente estudio confirma la utilidad del uso de la VSM-ACM como test eficaz, seguro y sencillo para predecir la aparición de anemia fetal, confirmando los resultados obtenidos en trabajos previos tanto en la valoración de anemias de tipo inmune como en las de etiología no inmune

Introduction. We assess the feasibility of using themiddle cerebral artery peak systolic velocity (MCA-PSV) in the management of pregnancies at risk of fetal anemia.Methods. We retrospectively reviewed 41 pregnanciesat risk of fetal anemia controled in our hospital between1998 and 2006. Doppler velocimetry of the MCAPSVwas performed, checking its correlation with thelevels of fetal hemoglobin obtained by chordocentesis intransfused and non transfused fetuses. Inmune and nonimmune anemias were included in this study.Results. 21 out of 41 cases showed a value of VSMACMsuggestive of moderate or severe anemia. In 76.2%the diagnosis was confirmed. Using previously publishedcut-off points for the prediction of fetal anemia, our sensitivity was 100% and the specificity was 83.3% for the diagnosis of moderate anemia. For severe anemia the values were 94.4% and 83.3% respectively. In previously transfused fetuses our results showed a sensitivity of 100% and a specificity of 88.5% in the diagnosis of moderate anemia, and a sensitivity of 58.8% and a specificity of 96.2% for the detection of severe anemia. Perinatal mortality in our study was 12,5 %. In newborns, neurological development was normal with a single case of neurosensorial deafness.Conclusions. This study confirms the usefulness ofVSM-ACM as an effective, safe and simple test in theprediction of fetal anemia. Our results are in agreementwith previous studies in the evaluation of immune aswell as non immune anemias (AU)

Clinical significance of persistent left superior vena cava diagnosed in fetal life.

OBJECTIVES: To determine the prevalence and clinical significance of persistent left superior vena cava (PLSVC) in fetuses with and without cardiac and extracardiac anomalies. METHODS: Charts and recorded images were reviewed from high-risk patients who underwent fetal echocardiography between January 2000 and December 2005. This retrospective study included 54 fetuses with confirmed PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Associated congenital heart defects (CHDs), extracardiac abnormalities, including first trimester nuchal translucency (NT) thickness, and fetal/postnatal outcome were analyzed. RESULTS: Of 5,737 referrals, 5,233 had a normal heart, and PLSVC was observed in 10 of these fetuses (0.2%; Group 1). CHDs were present in 504 and PLSVC was observed in 44 of these cases (9%). In the latter group, 18/44 (41%) fetuses had heterotaxy syndrome (Group 2) where the most common structural heart defects were atrioventricular septal defect and double-outlet right ventricle. Fetuses without heterotaxy syndrome (Group 3) accounted for 26/44 (59%) cases of CHDs associated with PLSVC. In this group of fetuses the most common CHDs were left outflow tract obstructive defects and conotruncal anomalies. Increased NT was observed in 29%, without differences among the three groups. The survival rates among fetuses in Groups 1, 2 and 3 were 100%, 44% and 50%, respectively. After excluding patients who underwent pregnancy termination, there were no significant differences in the survival rates among the groups, probably due to the small size of the samples. CONCLUSIONS PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD.

Prenatal management of placental chorioangioma: value of the middle cerebral artery peak systolic velocity.

Placental chorioangiomas are benign tumors of the placenta. Large chorioangiomas may cause severe complications such as fetal anemia, hydrops and fetal death. We report the use of sonographic findings and peak systolic velocity in the middle cerebral artery in the diagnosis and management of fetal anemia without the occurrence of hydrops fetalis in a pregnant woman with a large placental chorioangioma. Successful intrauterine blood transfusion was performed at 26 weeks. Spontaneous thrombosis of the main supplying blood vessel of the chorioangioma was detected at 33 weeks. The child was delivered at 39 weeks of pregnancy in normal clinical condition.

Typical isolated ectrodactyly of hands and feet: early antenatal diagnosis.

Ectrodactyly is a rare dominant autosomal malformation with variable expression. Herein we report a case early diagnosed by ultrasound at 15 weeks of gestation of isolated ectrodactyly involving the four limbs. The sonographic findings were bilateral split hands and split foot. Diagnosis of typical isolated ectrodactyly was pathologically confirmed. Clinical forms, pathogenesis, differential diagnosis, and early prenatal diagnosis are discussed.

Influence of estradiol and gestagens on oxidative stress in the rat uterus.

OBJECTIVE: We studied the effect of ovariectomy, estradiol (E2), and E2 + medroxyprogesterone (MPA) on the Wistar rat uterus. METHODS: We used 15 adult female rats. The study was divided into the following four stages: (a) extirpation of the upper half of the left hemi-uterus (basal state) and ovariectomy; (b) animals were maintained for 15 days without treatment, performance of a new laparotomy, and extirpation of the remaining left hemi-uterus (OVX state); (c) beginning of E2 replacement therapy (ERT) (8 microg/day) for 15 days, followed by extirpation of the upper half of the right hemi-uterus (ERT state); and (d) the administration of E2 was continued, and oral treatment with MPA was begun (20 microg/day) to last for a further 15 days. At the end of the combined hormone replacement therapy (HRT) the remaining right hemi-uterus was extirpated (HRT state). At the end of each intervention, the plasma concentrations of E2 and PRG were measured. RESULTS AND DISCUSSION: The ovariectomy significantly reduced the malonaldehyde (MDA) levels (P < 0.0008) and catalase activity (P < 0.0006). The ERT very significantly (P < 0.0033) raised the catalase and MDA levels; these significance levels were maintained after the Bonferroni method was applied (overall error 5%). The HRT reduced the levels of MDA and catalase, but not significantly after the Bonferroni test was applied.Conclusions. Uterine oxidative stress is increased by E2, resulting in a significant increase in MDA. This may be modulated in part by the catalase activity. Although it cannot be confirmed categorically, MPA seems to intervene by decreasing the said oxidative stress.

Influence of estrogen replacement therapy on plasma lipid peroxidation.

OBJECTIVE: To observe whether any relationship exists between the concentration of plasma estradiol (E2) and the plasma concentrations of malondialdehyde (MDA) or whether a relationship exists between the concentration of plasma E2 and the activity of the erythrocyte enzymes, superoxide dismutase (SOD) and catalase, in ovariectomized female Wistar rats (treated and untreated with E2). DESIGN: We used 40 ovariectomized Wistar rats randomly assigned to four groups. The first group was allowed to evolve freely with no treatment. A gel containing 17beta-estradiol was administered transdermally to the other three groups at doses of 4, 8, and 16 microg/day, respectively. After 15 days of treatment, blood samples were obtained from the four groups. The concentrations of plasma MDA and E2 and the activities of erythrocyte catalase and SOD were determined. RESULTS: There were significant correlations between the MDA levels and the logarithm (base 10) of the plasma E2 concentrations in both linear (p = 0.00093) and quadratic (p = 0.000001) regression analyses. No relationship was found between the E2 concentrations and the catalase and SOD activities. CONCLUSIONS: There was a clear relationship between the plasma levels of MDA and the logarithm of the plasma E2 concentrations, which was best demonstrated with a quadratic regression. This model may explain the contradictory findings presented by estrogens with respect to their pro-or antioxidant action.

Influence of estradiol on oxidative stress in the castrated rat uterus.

OBJECTIVE: We set out to study how the concentration of estradiol influences oxidative stress (malondialdehyde) and the superoxide dismutase and catalase antioxidant systems in the castrated female Wistar rat uterus. METHODS: We used 28 castrated female Wistar rats: 7 were left to evolve freely and the rest were divided into three groups of 7 animals receiving respective doses of 4, 8, and 16 microg/day of estradiol (E2) for 15 days. At the end of the study period, we determined the plasma concentrations of E2 and the concentrations of malondialdehyde (MDA) and superoxide dismutase and catalase activities in the uterus. RESULTS: There was a significant correlation (P < 0.000028) between the uterine malondialdehyde levels and the logarithm (base 10) of the plasma E2 concentrations and also between malondialdehyde and the uterine catalase activity (P < 0.002). The regression plane that best fitted the correlation among the three variables was MDA = 10.21 + 12.88 x Log [E2] - 0.49 x catalase activity. We found no significant relationships with the superoxide dismutase activity. CONCLUSIONS: There was a linear correlation between the base-10 logarithm of the estradiol plasma concentration and the phenomenon of uterine lipid peroxidation as measured by the MDA concentration in the uterus. This phenomenon was in part modulated by the inverse linear relationship between the antioxidant activity of the uterine catalase and the concentration of uterine MDA.

Effect of bilateral ovariectomy and ovarian steroid hormones on the antioxidant systems and plasma malondialdehyde levels in Wistar rats.

We studied in the Wistar rat the effect of bilateral ovariectomy and of the administration of estradiol (E2) alone or together with medroxyprogesterone (MPA) on the lipid peroxidation (MDA) and the enzymatic (SOD and catalase) and non-enzymatic (vitamins A and E) antioxidant systems. At 15 days after castration, we observed no variations in the lipid peroxidation levels (MDA), with there being a slight increase in vitamins A and E (p<0.05), catalase (p<0.01), and SOD (n.s.) activity. The addition of estradiol led to a decline (n.s.) in the MDA values and in the catalase and SOD activity, with no modification of the vit. A and E concentrations. The combined (E2+MPA) therapy did not modify the results obtained with E2 alone, although the catalase decline did now reach significance (p<0.05).

[The P-10 as a tool in the evaluation of the use of a pediatric hospital emergency service by the primary care network]. / El P-10 como instrumento para la evaluación del uso de un servicio hospitalario de urgencias pediátricas por parte de la red de asistencia primaria.

With the aim of determining if the Primary Care network adequately uses our centre's Emergency service, the 390 P-10s received during May and June 1990 were examined. The presence of clinical judgement, the percentage of admissions, the place of origin of the P-10, the relationship of the issuing centre to our Health Area, the diagnosis made in the Emergency Centre and the patient's destination were all analysed. 42.3% came from rural areas. Clinical judgement was exercised in 56.13%. The P-10s from urban areas showed that clinical judgement had been exercised in 63.6% of cases, as against only 49.7% in the rural areas and 43.2% in the special emergency service. 13.2% were admitted. 41.7% of the referral forms did not come from our health area. The results led us to conclude that there is an insufficient use of the P-10 in our field, as the majority of these patients could have been taken on by the Primary Care network.

Serum gastrin levels during pregnancy.

Serum Gastrin levels were measured by radioimmunoassay in serum samples taken from nongravid normal women and normal healthy pregnant volunteers who were divided into four groups according to gestation time. In nonpregnant control subjects the gastrin values found were 52.23 +/- 2.66 pg/ml. The gastrin levels observed from 5 to 15 and from 36 to 42 weeks of pregnancy are similar to the values of control subjects, whilst the gastrin levels found from 16 to 35 weeks are significantly lower. These results could help us to unravel the distressing problem of dyspeptic symptoms from 16th to 35th week of pregnancy.