Outcome of fetuses diagnosed with atrioventricular septal defect.

OBJECTIVE: To quantify the association of prenatally diagnosed atrioventricular septal defect with Down syndrome and to evaluate its impact on obstetric and neonatal outcomes. METHODS: Charts of 42 cases of atrioventricular septal defect diagnosed by fetal echocardiography from July 1985 to July 1997 were reviewed for prenatal history and outcome data (pregnancy outcome, pathologic confirmation, postnatal echocardiographic findings, and neonatal outcome). Statistical analysis was done using Fisher exact test and odds ratios. RESULTS: The mean gestational age at diagnosis was 26 weeks. Four cases could not be confirmed antenatally on repeat echocardiograms and were excluded. Reasons for referral of the remaining 38 fetuses included an abnormal four-chamber view in 76%. Twenty-two fetuses (58%) had abnormal karyotypes: 19 trisomy 21, one trisomy 18, one trisomy 13, and one mosaicism. The cardiac lesions were isolated in 20 fetuses (53%). After excluding cases of termination, ten of 12 fetuses (83%) with Down syndrome survived, compared with seven of 13 (54%) with normal karyotypes (P = .125). The odds of trisomy 21 were 16 times higher (95% confidence interval 3.0, 85.3) in fetuses with isolated cardiac lesions compared with those with associated cardiac anomalies. CONCLUSION: Prenatal diagnosis of atrioventricular septal defect was associated with a 58% risk of aneuploidy (mainly trisomy 21). Down syndrome fetuses with this cardiac anomaly appeared to have a better survival rate than fetuses with normal karyotypes. Our sample did not have enough power to show a statistically significant difference. When an isolated atrioventricular septal defect was diagnosed prenatally, the odds of trisomy 21 were significantly higher than when other associated cardiac lesions were diagnosed. This information should be considered in prenatal counseling for atrioventricular septal defect.

Multifetal pregnancy reduction and selective termination: the Canadian experience.

OBJECTIVE: To describe the experience of two Canadian referral centres with multifetal pregnancy reduction (MFPR) and selective termination (ST). METHODS: Retrospective chart review of all MFPR and ST procedures during the periods from January 1, 1990, to December 31, 1997 (Vancouver), and from September 1, 1995, to December 31, 1997 (Toronto). Outstanding outcome data were obtained by telephone. All women were managed according to standard protocols. Non-parametric analysis of continuous variables and Fisher's exact test for categorical variables were used. RESULTS: 61 women underwent transabdominal MFPR (n = 44) or ST (n = 17). Median maternal age: MFPR and ST 33.0 years; gestational age at reduction: MFPR 11.4, ST 20.2 weeks; procedure duration: MFPR 4, ST 10 min. 89% MFPR and 12% ST cases followed assisted reproduction. 7% MFPR and 18% ST pregnancies lost <24 weeks (n.s.). 97% MFPR and 83% ST non-reduced fetuses delivered alive. Median delivery gestational age: MFPR and ST 37 weeks. CONCLUSIONS: The results are similar to published series. This procedure has increased options for Canadian couples, offering the procedure 'close to home', reducing costs and, more importantly, the significant psychological morbidity following these procedures.

Effectiveness of primary level antenatal screening for severe congenital heart disease: a population-based assessment.

The four-chamber view of the fetal heart has been advocated as an effective antenatal screen for severe forms of congenital heart disease (CHD). To evaluate the sensitivity of this test at a primary care level, a retrospective review was undertaken for the period 1988 to 1992 of ultrasonograms of fetuses referred for fetal echocardiography, mothers of neonates referred for cardiac assessment, and perinatal deaths attributed to CHD. The study centers serve a population base of 3.5 million persons. Sixty-nine cases of severe forms of CHD were reviewed. The diagnoses were chosen because all show abnormal findings on four-chamber view: 38 hypoplastic left heart syndrome, 13 pulmonary atresia with intact ventricular septum, and 17 variants of univentricular heart. The cases involved a total of 94 scans. These were reviewed by diagnosis and stratified by gestational age and year of scanning. No diagnosis was made at a gestational age younger than 16 weeks. From 16 weeks to term, overall sensitivity was 40% (32/80 scans); sensitivity improved with advanced gestational age (34% to 48% at 16 to 24 and > 24 weeks' gestation, respectively). There was no longitudinal evidence of a learning curve. Reasons for this low sensitivity are related to unfamiliarity with CHD, low incidence in small populations, and inadequate evaluation of the fetal heart. These problems can be resolved with continued training and direction from a major center.

Prenatal spinal evaluation and functional outcome of patients born with myelomeningocele: information for improved prenatal counselling and outcome prediction.

OBJECTIVE: Prenatal ultrasonography can localize the level of the spinal cord malformation, allowing prediction of the potential postnatal neurological deficit and functional prognosis. METHODS: This study has two evaluations: (a) a retrospective prenatal review of 26 fetuses with spinal dysraphism (1987-1991), and (b) a follow-up descriptive study of patients (1971-1981) who underwent closure of the spinal lesion and ventricular shunting in the neonatal period. RESULTS: Prenatal ultrasound evaluation enabled the accurate definition of the last intact vertebral level which allows separation of fetuses into three functional groups (last intact level L2, L3-4, L5-sacral). Patterns of ambulation, urinary and bowel continence, and school performance vary according to level of spinal lesion and the neurological deficit. The need for ventricular shunts, the incidence of other spinal malformations and surgical interventions did not vary with the level of the spinal lesion. CONCLUSIONS: The functional outcome for patients with myelomeningocele is variable; however, distinct patterns emerge based on the level of spinal dysraphism and the resultant neurological deficit. By relating the level of the fetal spinal lesion to outcome data, more precise functional prognoses can be given to families.

Antenatal invasive and noninvasive management of alloimmune thrombocytopenia.

The outcome analysis of 10 pregnancies at risk for neonatal alloimmune thrombocytopenia (NAIT) is presented. An experimental protocol of cordocentesis and maternal administration of intravenous immunoglobulin (IVIG) is compared to a control group of older untreated affected siblings. The outcome in pregnancies treated with IVIG shows improved fetal platelet count in 70% and no intraventricular hemorrhage. We conclude that maternal administration of IVIG appears to improve clinical outcome in fetuses at risk for NAIT.

Cordocentesis: overall pregnancy loss rate as important as procedure loss rate.

A 4-year retrospective review of cordocentesis at a single center with four operators identified 182 patients undergoing 214 cordocenteses. The major indications were chromosomal, fetal platelet and rhesus evaluation. The procedure was successful in 95%. A positive genetic diagnosis was present in 18% with 64% having trisomy 18, 21 or 13. The procedural pregnancy loss rate was 3.25, 1.25 and 2.75% for chromosomal and nonchromosomal indications and total population, respectively. The overall fetal loss rate (procedural, nonprocedural) in chromosomal risk population was 47%. This overall pregnancy loss risk should be included in patient counselling.

Emergency cervical cerclage: a retrospective review of 51 cases.

Fifty-one patients underwent emergency cervical cerclage using Shirodkar or McDonald suture for incompetent cervix diagnosed during pregnancy. Fifteen patients had only sonographic evidence of cervical incompetence (group I). Eighteen patients had cervical dilation of less than 3 cm (group II), and 18 patients had cervical dilation of 3 cm or more (group III). Perinatal mortality was 0%, 50%, and 55.6%, respectively. The median length of time gained with the cerclage was 16 weeks in group I, 1.8 weeks in group II, and 1 week in group III. Twelve infants required prolonged stay in the neonatal intensive care nursery because of prematurity. Six of 30 surviving infants had some abnormal physical findings at the time of initial discharge from the nursery. Premature rupture of membranes was the most common perioperative and postoperative complication. Chorioamnionitis developed in 17 of the 51 cases. Maternal complications included cervicovaginal fistula, deep vein thrombophlebitis, and pulmonary edema.

Cystic fibrosis in pregnancy.

OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important.

Fetal nuchal fluid--physiological or pathological?--In pregnancies less than 17 menstrual weeks.

For pregnancies less than 17 menstrual weeks, increasing amounts of nuchal fluid increase the risks of chromosome abnormalities with localized nuchal fluid, diffuse nuchal fluid, cystic hygroma, and fetal hydrops having chromosomal risks of 12, 23, 50, and 78 per cent, respectively. The ultrasound appearance of localized or diffuse nuchal fluid is not a specific discriminator, but a fluid depth of greater than or equal to 5 mm may be an indicator of increased risk of fetal chromosomal abnormalities. If the fluid depth is less than 5 mm, there is a stronger negative predictive value and negative likelihood risk of a fetal chromosome abnormality. Gestational age did not improve the fluid depth predictive value. Differentiation of physiological from pathological requires chromosome analysis, serial ultrasound evaluation, and good clinical examination as a newborn and possibly as a young child. Long-term follow-up of those cases identified with resolving nuchal fluid abnormalities is not available and is required for a complete understanding of physiological and pathological aetiologies. Genetic counselling for fetal nuchal fluid would be recommended.

Cardiovascular collapse following an overdose of prostaglandin F2 alpha: a case report.

A case report is presented of a parturient who suffered severe hypotension and pulmonary oedema following an overdose of intramyometrial prostaglandin F2 alpha. Oxytocin induction of labour in this patient led to a rapid delivery, followed by a hypotonic uterus and postpartum haemorrhage. After resuscitation with blood and crystalloid fluids, the uterus was explored under general anaesthesia. The uterus was free of retained products but the lower uterine segment failed to contract despite bimanual uterine compression and intravenous oxytocin. Prostaglandin F2 alpha was injected into the lower uterine segment via a transvaginal approach. This was rapidly followed by cardiovascular collapse and later by pulmonary oedema. The differential diagnosis and subsequent management are discussed.

Management of quintuplet pregnancy by selective embryocide.

Selective embryocide was performed as a two-stage procedure in a patient with a quintuplet pregnancy in the first trimester. No complications occurred, and the patient was delivered of healthy twins at term. This procedure may be offered to selected patients with pregnancies with greater than five embryos.

Endocrine response to selective embryocide in a gonadotropin-induced quintuplet pregnancy.

With the increase of medical induction of ovulation, the incidence of grand multiple pregnancy is becoming more frequent. We report the endocrine response of a quintuplet pregnancy that was reduced at 9 weeks' gestation to a twin pregnancy by selective embryocide and compared with quadruplet, triplet, and twin pregnancies. The human chorionic gonadotropin titer declined fourfold, but there were no clinically significant changes in the progesterone or estradiol levels. We conclude that, despite the drop in human chorionic gonadotropin, the placenta had attained adequate secretory maturation to prevent any significant decline in progesterone and estradiol levels, which demonstrates the efficacy of this procedure at this gestational age.

The role of feticide in the management of severe twin transfusion syndrome.

Twin transfusion syndrome is a serious complication of monozygotic twin pregnancy. Diagnostic ultrasound now allows early diagnosis of this condition, but therapy has remained more elusive. In this article we present a case of severe twin transfusion syndrome diagnosed early in the second trimester. At 25 weeks' gestation, severe hydramnios, premature labor, and growth retardation of the donor twin suggested that selective feticide be contemplated to allow continuation of the pregnancy for the remaining twin. This was accomplished successfully by an in utero approach with subsequent follow-up and delivery of a healthy female infant at 37 weeks' gestation. Discussion of diagnosis, management, follow-up, and pathologic features is provided.

Management of post-Date Pregnancy.

The problems of prolonged gestation place the modern obstetrical care giver in a delicate situation between increasing consumer pressures for non-interventionist approaches to obstetrical care and more active management with the potential pitfalls of failed induction, caesarean section, and other hazards. This paper will briefly discuss the definition, etiology and pathophysiology of prolonged gestation, but will focus on appropriate use of antepartum monitoring methods with the determination of fetal well-being, and methods of labour induction as well as intrapartum management.