Tricuspid valve disease with significant tricuspid insufficiency in the fetus: Diagnosis and outcome

AbstractThe echocardiographic studies and clinical course of 27 fetuses (mean gestationl age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy. All fetuses had massive right atrial dilation and most who were serially studied had progressive right-sided cardiomegaly. Hydrops fetalis was found in six cases and atrial flutter in five.Associated cardiac lesions included pulmonary stenosis in five cases and pulmonary alresia in six. Four fetuses with normal forward pulmonary artery flow at the initial examination were found at subsequent study to have retrograde pulmonary artery and ductal flow in association with the development of pulmonary stenosis (n = 1) and pulmonary atresia (n = 3). On review of the clinical course of the 23 fetuses (excluding 3 with elective abortion), 48% of the fetuses died in utero and 35% who were liveborn died despite vigorous medical and, when necessary, surgical management, many of whom had severe congestive heart failure. Of the four infants who survived the neonatal period, three had a benign neonatal course, all of whom were diagnosed with mild to moderate Ebstein's anomaly; only one had pulmonary outflow obstruction. An additional finding at autopsy was significant lung hypoplasia documented in 10 of 19 autopsy reports.Tricuspid valve anomalies with tricuspid insufficiency can be identified echocardiographically in the fetus and should be searched for in the presence of right atrial enlargement. The prognosis for the fetus diagnosed in utero with significant tricuspid valve disease is extremely poor, with a prenatal course that includes progressive right heart dilation, with cardiac failure and lung hypoplasia in many and development of pulmonary stenose or pulmonary atresia later in gestation in some

Potassium chloride-induced fetal demise: a retrospective cohort study of efficacy and safety

Induction of fetal demise before second-trimester termination is performed for a number of reasons. One method for inducing fetal demise is via sonographically guided intracardiac potassium chloride (KCl) injection. We performed a retrospective cohort study to determine the efficacy and safety of intracardiac KCl injection as a method of second-trimester induced fetal demise. We reviewed records from patients who were referred for induced fetal demise from October 2002 to October 2011. We excluded patients undergoing selective fetal reduction in multiple gestations. Procedural complications, the dose of KCl, and the number of failed procedures were determined. Of the 192 completed procedures, 191 were successful (99.5%). The median gestational age at termination was 22 weeks (range, 15.4-24.9 weeks), and most terminations were surgical (68.0%). Major indications for termination were fetal anomalies (41.6%), unwanted pregnancy (20.8%), and aneuploidy (15.7%). The median dose of KCl was 10 mL (range, 3-40 mL). We found a significant correlation between the dose of KCl and estimated fetal weight. There was no significant correlation between the dose of KCl and body mass index or gestational age. We had 1 maternal complication of a seizure after needle placement but before KCl injection. Intracardiac KCl injection is an effective and safe method for induced fetal demise

Utility of routine screening fetal echocardiogram in pregnancies conceived by in vitro fertilization

To study the incidence and clinical significance of congenital heart defects (CHDs) detected by fetal echocardiography in pregnancies conceived by in vitro fertilization (IVF). Cohort study comparing a prospectively maintained database of all fetal echocardiograms from 2012 to 2018 and pooled data from the Connecticut Birth Defects Registry and statewide hospital discharge data. Large tertiary care center. A total of 181,749 live births and 9,252 fetal echocardiograms were analyzed. Fetal echocardiograms in patients with a previous child with a CHD, a family history of CHD, medication exposure, diabetes, anomaly in previous pregnancy, cardiac or other abnormality noted on previous ultrasound, or monochorionic twins were excluded from the final analysis. Treatment with IVF. Incidence of CHD and odds ratios with 95% confidence intervals (CIs). Infant outcomes for cases of CHD were evaluated for clinically significant disease, defined a priori as disease requiring any medical or surgical intervention or continued follow-up with pediatric cardiology. Fetal echocardiography was performed in 2,230 IVF pregnancies, of which 2,040 were without other known risk factors for CHD. The mean gestational age at the time of fetal echocardiography was 22.2 ± 1.4 weeks. The odds ratio for CHD in the IVF group compared with statewide population rates was 1.4 (95% CI 0.9–2.1). CHD was diagnosed in 26 fetuses, of which 21 were clinically insignificant ventricular septal defects. One fetal echocardiogram was concerning for pulmonary stenosis that was not present at birth. Four defects were clinically significant, indicating that 510 fetal echocardiograms were performed for every diagnosis of one clinically significant CHD in the IVF group. The incidence of CHD in IVF pregnancies without other risk factors is not significantly different from baseline population rates, and most CHDs diagnosed by fetal echocardiography in this group are clinically insignificant. Routine screening with fetal echocardiography in all IVF pregnancies provides limited utility beyond routine prenatal care and need not be recommended without the presence of other risk factors. Utilidad del screening ecocardiográfico fetal rutinario en embarazos concebidos con Fecundación in Vitro. Estudiar la incidencia y significado clínico de los defectos cardíacos congénitos (CHDs) detectados por ecocardiografía fetal en embarazos concebidos con fecundación in vitro (IVF). Estudio de cohortes comparando una base de datos mantenida prospectivamente de todas las ecocardiografías fetales entre 2012 y 2018 y los datos recopilados del Connecticut Birth Defects Registry y los datos de alta hospitalaria de todo el estado. Centro hospitalario terciario de gran tamaño. Se analizaron un total de 181,149 nacidos vivos y 9,252 ecocardiografías fetales. Se excluyeron del análisis final las ecocardiografías fetales de pacientes con un hijo previo con CHD, historia familiar de CHD, exposición a fármacos, diabetes, malformaciones en embarazos previos, malformaciones cardíacas u otras malformaciones detectadas en ecografías previas y gemelos monocoriónicos. Tratamiento de IVF. Incidencia de CHD y odds ratios con intervalos de confianza (CIs) del 95%. Los resultados de los casos de niños con CHD se evaluaron para enfermedad clínicamente significativa, definida a priori como enfermedad que requería cualquier intervención médica o quirúrgica o seguimiento continuado por cardiología pediátrica. Se realizó ecocardiografía fetal en 2,230 gestaciones de FIV, de las cuales 2,040 no tenían otros factores de riesgo conocidos para CHD. La edad gestacional media en el momento de la ecocardiografía fetal fue de 22.2 ± 1.4 semanas. La odds ratio para CHD en el grupo de IVF comparado con las tasas generales del estado fue de 1.4 (95% CI 0.9-2.1). Se diagnosticó CHD en 26 fetos, de los cuales 21 fueron defectos del septo ventricular clínicamente insignificantes. Una ecocardiografía fetal se refería a estenosis pulmonar que no se detectó en el nacimiento, Cuatro defectos eran clínicamente relevantes, indicando que se realizaron 510 ecocardiografías fetales para cada diagnóstico de una CHD clínicamente significativa en el grupo de IVF. La incidencia de CHD en embarazos de IVF sin otros factores de riesgo no es significativamente diferente de las tasas basales de la población general y la mayoría de CHDs diagnosticadas por ecocardiografía fetal en este grupo no son clínicamente relevantes. El screening rutinario de todos los embarazos de IVF con ecocardiografía fetal es poco útil más allá de los cuidados prenatales de rutina y no debería recomendarse sin la presencia de otros factores de riesgo. Defectos congénitos, patología cardíaca congénita, screening fetal, fecundación in vitro

Congenital heart disease in pregnancies complicated by maternal diabetes mellitus. An international clinical collaboration, literature review, and meta-analysis.

PURPOSE: Investigation of the incidence and distribution of congenital structural cardiac malformations among the offspring of mothers with diabetes type 1 and of the influence of periconceptional glycemic control. METHODS: Multicenter retrospective clinical study, literature review, and meta-analysis. The incidence and pattern of congenital heart disease in the own study population and in the literature on the offspring of type 1 diabetic mothers were compared with the incidence and spectrum of the various cardiovascular defects in the offspring of nondiabetic mothers as registered by EUROCAT Northern Netherlands. Medical records were, in addition, reviewed for HbA(1c) during the 1st trimester. RESULTS: The distribution of congenital heart anomalies in the own diabetic study population was in accordance with the distribution encountered in the literature. This distribution differed considerably from that in the nondiabetic population. Approximately half the cardiovascular defects were conotruncal anomalies. The authors' study demonstrated a remarkable increase in the likelihood of visceral heterotaxia and variants of single ventricle among these patients. As expected, elevated HbA(1c) values during the 1st trimester were associated with offspring fetal cardiovascular defects. CONCLUSION: This study shows an increased likelihood of specific heart anomalies, namely transposition of the great arteries, persistent truncus arteriosus, visceral heterotaxia and single ventricle, among offspring of diabetic mothers. This suggests a profound teratogenic effect at a very early stage in cardiogenesis. The study emphasizes the frequency with which the offspring of diabetes-complicated pregnancies suffer from complex forms of congenital heart disease. Pregnancies with poor 1st-trimester glycemic control are more prone to the presence of fetal heart disease

Maternal-fetal medicine fellows' perception and comfort with obstetrical ultrasound and prenatal diagnosis.

BackgroundUltrasound training is a vital component of maternal-fetal medicine fellowships in the United States. Of the 18 months of core clinical training, the American Board of Obstetrics and Gynecology currently requires a minimum of 3 months to be dedicated to ultrasound to be eligible for board certification. However, the experience and degree of hands-on training differ among the fellowship programs and have not been reassessed for nearly a decade.ObjectiveTo assess regional heterogeneity in the ultrasound training experience during maternal-fetal medicine fellowship in the United States.Study designA survey was distributed to postgraduate year (PGY)-6 maternal-fetal medicine fellows registered to attend an annual ultrasound training course before the conference (n=114). For programs with >1 fellow attending (n=39), only 1 of them completed the survey to represent the program. The questions included demographics of the program, ultrasound training structure, the fellows' self-perception of ultrasound capabilities, research, mentorship, and technical aspects of sonography.ResultsSeventy two postgraduate year 6 fellows with a wide geographic distribution as follows completed the survey (96% response rate): 10 (14%) from the West, 16 (22%) from the Midwest, 17 (24%) from the South, and 29 (40%) from the Northeast. Respondents undergoing training in the South were less likely to report feeling comfortable performing nuchal translucency and detailed anatomic surveys than those from other regions (nuchal translucency: P=.046; anatomy: P=.011). Most of the respondents reported feeling comfortable performing growth (78%) and umbilical artery Doppler (58%) and feeling uncomfortable with three-dimensional ultrasound, neurosonography, and fetal echocardiography. Respondents in the Northeast were more likely to report feeling comfortable performing chorionic villus sampling (P=.001). There was no difference among fellowship programs in the presence or absence of ultrasound curriculum, bedside teaching, ultrasound-focused research mentorship, or months of ultrasound training.ConclusionDespite the standardization of ultrasound training structure across the United States, there remains regional heterogeneity in fellow self-reported comfort with specific ultrasound techniques and chorionic villus sampling at a midpoint in their fellowship training. The maternal-fetal medicine attending involvement at the bedside did not affect the fellow self-reported comfort with ultrasound surveys. This study highlights the need for further optimization of maternal-fetal medicine fellowship ultrasound training, especially in advanced sonography and diagnostic procedures

Sonographic myometrial thickness predicts the latency interval of women with preterm premature rupture of the membranes and oligohydramnios

Term labor is associated with global thinning of the myometrium. We hypothesized that a thickened myometrium at the time of preterm premature rupture of membranes (PPROM) predicts less myometrial wall stress and, consequently, a longer latency interval. Myometrial thickness was measured prospectively in 76 pregnant women enrolled in the following groups: PPROM (n = 28, mean [range], gestational age [GA]: 29.5 weeks [w] [21.0 w-33.0 w]), preterm nonlabor control group (P-CTR), (n = 21, GA: 27.5 w [23.0 w-32.0 w]) and term nonlabor control (T-CTR) (n = 27, GA: 38.6 w [37.0 w-41.6 w]). All PPROM women had oligohydramnios (AFI: 1.4 cm [0.0 cm-5.1 cm]). MT was measured ultrasonographically at the midanterior, fundal, posterior, and lower uterine segment wall in cases and controls with an intraoperator variability <10%. Women in the PPROM group displayed uniform thickness of the uterine body (mean ± SEM, anterior: 10.6 ± 0.6 mm, fundal: 10.7 ± 0.7 mm, posterior: 8.9 ± 0.5 mm, P = .078). At midanterior site the myometrium of the PPROM group was thicker compared to both P-CTR ( P < .001) and T-CTR ( P = .025) groups. This difference was preserved at the fundus (PPROM vs P-CTR, P < .001; PPROM vs T-CTR, P = .015). There was a positive correlation between fundal MT and latency period ( r = 0.43, P = 0.02) that persisted after adjusting for GA ( P = .04). A fundal MT less than 12.1 mm was 93.7% sensitive and 63.6% specific for the identification of women whose latency period was less than 120 hours. Significant thickening of the anterior and fundal walls of the uterus follows PPROM. A thick myometrium in nonlaboring patients with PPROM is associated with longer latency interval. Sonographic evaluation of MT may represent an alternative clinical tool for the prediction of a short latency interval in women with PPROM