Faculty Bibliography

A retrospective analysis of all fetuses with prenatally detected choroid plexus cysts, identified at our institution between 1988 and 1993, was performed. Cytogenetic data, associated sonographic findings, obstetric outcome and pediatric follow-up was obtained to determine the incidence of aneuploidy and the rate of associated congenital anomalies in second-trimester fetuses with this finding. There were 211 second-trimester fetuses identified with a choroid plexus cyst. Amniocentesis was performed in 175 (83%) and postnatal chromosome analysis was performed in one newborn. Follow-up data are available on 203 (96%). Of the 176 cytogenetically studied fetuses, eight (4.5%) were aneuploid (including four cases of trisomy 18). In four of the aneuploid fetuses, the choroid plexus cyst was the only abnormal sonographic finding identified (including one case of trisomy 18). From this study and a review of the literature, we confirm that choroid plexus cysts are a sonographic marker for trisomy 18, even when identified as an isolated finding in an otherwise normal-appearing fetus. We conclude that the detection of a choroid plexus cyst merits further careful evaluation of fetal anatomy and consideration of cytogenetic evaluation

Origin of both coronary arteries from the pulmonary artery is generally a lethal condition from progressive ventricular failure. We report the clinical and surgical course of two infants, ages 3 and 6 months, with this anomaly. One patient had normal intracardiac anatomy with low pulmonary artery pressures (30/12 mm Hg). The second patient had a restrictive subpulmonic ventricular septal defect with a moderately elevated pulmonary artery pressure (50/13 mm Hg). Left ventricular ejection and shortening fractions were profoundly depressed in both patients. The common coronary trunk arose from the right anterior facing sinus in one patient and from the left posterior facing sinus in the other. Both patients underwent repair by direct coronary implantation to the aorta. Left ventricular function improved with shortening fractions near normal at a follow-up of 6 months for one patient and 1 year for the other. Early diagnosis and prompt repair is compatible with survival and return of normal shortening fraction

The concentrations in cord blood of total immunoglobulin G (IgG) and the four subclasses of IgG were measured in 34 fetuses at a mean gestational age of 25 weeks (range, 18 to 35 weeks). The blood samples were obtained by percutaneous umbilical blood sampling, and results were compared with the respective IgG subclass concentrations of the mothers. The efficiency of transplacental transfer of the different IgG subclasses was determined. Transfer of IgG1 and IgG4 was found to be significantly more efficient than that of IgG3 and IgG2. IgG2 was the subclass least efficiently transferred from mother to fetus. These differences may partly explain the susceptibility of newborns to various pathogens, such as streptococcus group B

OBJECTIVE: Our purpose was to assess the prevalence, cause, and clinical significance of fetal tricuspid valve regurgitation in structurally normal hearts during indicated fetal echocardiographic examination. STUDY DESIGN: The prevalence of fetal tricuspid regurgitation was retrospectively evaluated in a group of 733 singleton fetuses referred for routine fetal echocardiography. RESULTS: The prevalence of this abnormality was 6.8%. Tricuspid valve regurgitation was most frequent in the group referred for fetal echocardiography to evaluate indomethacin exposure, followed by maternal diabetes. Factors associated with tricuspid valve regurgitation included ductal constriction, abnormal heart rhythm, atrial septal aneurysm, congestive heart failure, pericardial effusion, myocardial hypertrophy, and extracardiac malformations. Fetal tricuspid valve regurgitation was trivial in 80% (nonholosystolic, maximum velocity < 2 m/sec) and was significant in 20% (holosystolic, maximum velocity > 2 m/sec). Neonatal follow-up of tricuspid valve regurgitation was unremarkable. CONCLUSIONS: Fetal tricuspid valve regurgitation with normal heart anatomy was a frequent finding during indicated fetal echocardiographic examination and may indicate abnormal physiologic characteristics (increased preload or afterload, myocardial impairment, or arrhythmia). In the majority of cases (92%) the possible cause may be established. In other cases (8%) there may be 'idiopathic' tricuspid valve regurgitation

A rotational data acquisition system was used to create three-dimensional images from thoracic and subxiphoid echocardiographic windows in children with various types of subaortic stenosis. Thirteen patients, ranging in age from 2 days to 17 years, were examined. Subaortic obstruction was caused by a discrete fibrous ridge in six patients, hypertrophic cardiomyopathy in two patients, subaortic tunnel in two patients, and septal malalignment, restrictive VSD, and abnormal suture placement each in one patient. Unique views could be obtained equivalent to surgical or autopsy dissections, and allowed more complete understanding of morphology than conventional imaging techniques

Transthoracic real-time three-dimensional echocardiography using the rational scanning approach for data acquisition became a feasible modality for cardiac imaging during the last 4 years. Several attempts for reconstruction of the heart have been made using different methods. In this study we evaluate the data acquisition using the rotational approach from the transthoracic window. Thirty-five children with congenital heart disease were enrolled in the study. All of them underwent complete two-dimensional and Doppler echocardiogram followed by three-dimensional reconstruction using rotational image acquisition. The rotational approach enabled us to acquire good data from subcostal, apical, and suprasternal notch positions by rotating the transducer from 0 degrees-180 degrees. Novel views of the atrioventricular valve, semilunar valve, atrial, and ventricular septi were established by spatial plane imaging from the three-dimensional echocardiography. This reconstruction enables spatial imaging of cardiac structures and deficiencies. Three-dimensional echocardiography will enhance the understanding of complex congenital heart disease

Cordocentesis has pioneered perinatal medicine in the last 10 years with the ability to obtain genetic information, to diagnose and to treat erythrocyte and platelet alloimmunization, to discover the presence of perinatal infections, and to determine the acid-base status of the fetus. Fetal blood sampling has also allowed the perinatologist a greater understanding of fetal physiology, such as thyroid-pituitary function. This review will concentrate on the role of cordocentesis in determining fetal physiology with acid-base status, compare noninvasive testing in its prediction of the hypoxic-acidemic state, and also how cordocentesis has enabled obstretricians to have a better understanding of fetal thyroid development and function

Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing

Three-dimensional cardiac reconstruction generated from transesophageal interrogation can be performed using an integrated unit that captures, processes, and postprocesses tomographic parallel slices of the heart. This probe was used for infants and young children in the transthoracic position to evaluate the feasibility of producing three-dimensional cardiac images with capability for real-time dynamic display. Twenty-two infants and children (range 1 day-3.5 years) underwent image acquisition using a 16 mm 5 MHz 64 element probe placed over the precordium. Two infants were also imaged from the subcostal position. Data was obtained and stored over a single cardiac cycle after acceptable cardiac and respiratory gating intervals were met. The transducer was advanced in 0.5-1 mm increments over the cardiac structures using identical acquisition criteria. The images were reconstructed from the stored digital cubic format and could be oriented in any desired plane. In 9 of the 22 infants the images obtained were of optimal quality. The images obtained displayed normal cardiac structures emphasizing depth relationships as well as visualization of planes not generally demonstrated by two-dimensional imaging. Several lesions were also depicted in a unique fashion using this technique. Though the method employed was limited by movement artifact and reconstruction time, the quality of the three-dimensional display was excellent and enhanced by real-time demonstration. The transthoracic approach was successful in capturing sufficient data to create three-dimensional images, which may have further application in more accurate diagnosis of complex cardiac abnormalities and generation of planes of view which could duplicate surgical visualization of a lesion. Further assessment of the technique in infants with congenital heart disease is warranted

Cordocentesis is a feasible, reliable, and safe method for fetal evaluation of a wide variety of disorders. It has allowed for in-utero treatment of fetal red cell isoimmunization and opened future routes for other fetal therapies. In this review, results of a multicenter intrauterine fetal blood sampling registry will be presented, outlining current indications, techniques, and complications of the procedure. In addition, current research in the assessment and treatment of fetal erythrocyte isoimmunization and infection, as well as the role of cordocentesis in rapid genetic karyotyping, work-up of non-immune hydrops, fetal platelet abnormalities, and fetal growth restriction will be reviewed. Recent work using cordocentesis to better understand fetal metabolism, endocrinology, and immunology will also be reviewed