Faculty Bibliography

Color-flow Doppler is used for special localization of cardiac flows. The transeptal jet in supracristal ventricular septal defect (VSD) is directed toward the pulmonary valve and is often difficult to distinguish from the right ventricular (RV) outflow tract flow. Pulsed- and continuous-wave Doppler can misread the VSD jet as a stenotic lesion in the RV outflow tract. This study describes the color-flow Doppler characteristics of supracristal VSD and determines if color-flow Doppler can differentiate supracristal VSD jets from RV outflow tract flow. The study group comprised 28 patients ranging in age from 3 days to 23 years (mean 6.4), with catheter-diagnosed supracristal VSD in 14, isolated pulmonary valve stenosis in 10 and RV infundibular obstruction in 4. The echocardiographic Doppler and color-flow Doppler data of all patients were reviewed without knowledge of catheterization diagnosis. In all patients with supracristal VSD, color-flow Doppler revealed an abnormal transeptal jet directed toward the pulmonary valve that occurred 5 to 10 ms before RV outflow tract flow was identified. VSD and RV outflow tract jet could be differentiated by pulsed- and continuous-wave Doppler alone in 10 of 14 patients (71%). In 4 patients, differentiation was possible only with color-flow Doppler and electrocardiographic gating. Associated aortic regurgitation, or a fistula, or both, were detected in 6 patients. Pulsed- and continuous-wave Doppler with or without color-flow Doppler correctly identified the level of obstruction in all patients with RV outflow tract lesions (14 of 14). Supracristal VSD jet occurs in early systole and is directed toward the pulmonary valve.(ABSTRACT TRUNCATED AT 250 WORDS)

This chapter will review the evolving role of intravascular ultrasound imaging and transesophageal echo in the care of children, infants and adults with congenital heart disease. The technologies relevant to congenital heart disease applications differ from those involving coronary disease since the intravascular structures imaged often involve visualization of large vessels and cardiac chambers. On the other hand, the requirements for transesophageal echo in children with congenital heart disease involve intraoperative (surgical) and imaging procedures in the catheterization laboratory which are performed for monitoring interventional catheterization therapy. As such, whereas the intravascular devices needed for pediatric cases involve lower frequency and sometimes larger catheters, the requirements for transesophageal echocardiography require higher frequency and smaller esophagoscopes. Applications of intravascular imaging including sizing of congenital stenoses, dilation of coarctation and valvular stenoses, imaging of intrapulmonary thrombi and monitoring of placement of ASD 'button' devices in the heart will be reviewed. The intraoperative transesophageal uses for monitoring infant surgery include procedures for tetralogy repair, transposition repair and repair of AV septal defects and other complex congenital heart disorders. Both of these 'invasive' methods of echocardiography have an important and evolving role in the management of congenital heart disease in children and infants

Developmental defects in neutrophil function, including diminished expression of plasma membrane receptors, may play an important role in the susceptibility of the newborn infant to infection. We used monoclonal antibodies and flow cytometry to study the expression of complement receptor type one (CR1), complement receptor type three (CR3), and Fc gamma receptor type three (FcRIII) on neutrophils from six fetuses with Rh disease, 10 preterm infants, nine term infants, and nine adults. Expression of the complement receptors on unstimulated cells was similar for all groups, but significant differences in complement receptor expression were observed after stimulation with N-formyl-methionyl-leucyl-phenylalanine (FMLP). Fetal, preterm, and term infant neutrophils expressed less CR3 than FMLP-stimulated neutrophils of adults [61 +/- 2, 48 +/- 4, and 66 +/- 4% (mean +/- SEM) of the mean for adults, p less than 0.05]. FMLP-stimulated CR1 expression for these groups was 61 +/- 6, 73 +/- 6, and 91 +/- 9% of the adult mean (p less than 0.05, fetal versus term infant and adult). Expression of both CR3 and CR1 increased with postconceptional age in the infants (r2 = 0.49, p less than 0.001 for CR3; r2 = 0.23, p less than 0.05 for CR1). Neutrophils of the preterm and term infants expressed less FcRIII than adult neutrophils (68 +/- 10 and 77 +/- 7% of the adult mean, p less than 0.05, for FMLP-stimulated cells), whereas fetal neutrophil FcRIII expression did not differ from that of the adult.(ABSTRACT TRUNCATED AT 250 WORDS)

We attempted to investigate mechanisms, in addition to sexual contact, by which human papillomaviruses associated with anogenital tract lesions could be transmitted. Samples of exfoliated cervical cells were obtained from 45 pregnant women and were assayed by Southern blot hybridization analysis for the presence of human papillomavirus nucleic acids. Twenty-five of the 45 women had cells positive for human papillomavirus deoxyribonucleic acid. A neonatal nasopharyngeal aspirate was obtained at term and analyzed for the presence of human papillomavirus deoxyribonucleic acid. We documented the presence of human papillomavirus deoxyribonucleic acid in the oral pharyngeal cavity of the neonates in 15 of 45 nasopharyngeal samples analyzed. Amniotic fluid was obtained from 13 patients when their membranes were artificially ruptured. These samples were assayed for the presence of human papillomavirus deoxyribonucleic acid; two of the 13 amniotic fluid samples contained human papillomavirus deoxyribonucleic acid. The detection of human papillomavirus deoxyribonucleic acid in the oral cavity of neonates is indicative of a perinatal mechanism of viral transmission. The detection of human papillomavirus deoxyribonucleic acid in the amniotic fluid may suggest an in utero mechanism of transmission. However, problems encountered in collecting the amniotic fluid samples preclude us from definitive interpretation of these data

To assess the usefulness of balloon valvuloplasty in patients with a dysplastic pulmonary valve, the files of 36 patients (aged 1 day to 18.5 years) who had two-dimensional echocardiography before and continuous wave Doppler echocardiography late after balloon valvuloplasty (balloon diameter greater than or equal to 20% anulus diameter) were reviewed. Results of relief of pulmonary stenosis were graded by catheter gradient in the catheterization laboratory and compared with Doppler echocardiographic findings at follow-up. There were 32 patients with typical pulmonary stenosis and 4 with a dysplastic valve. In the 32 patients with typical pulmonary stenosis, transvalvular gradient changed from a mean of 67 +/- 32 to 20 +/- 20 mm Hg (p less than 0.0001, mean reduction 72.6%). The gradients at follow-up by Doppler echocardiography averaged 20 mm Hg including 15 that increased, 3 that were unchanged and 14 that decreased. Only 3 (9%) of 32 patients had a gradient greater than 25 mm Hg at follow-up and only one gradient was greater than 35 mm Hg. All four patients with a dysplastic valve had a gradient that decreased with valvuloplasty from a mean of 85 +/- 33 to 33 +/- 20 mm Hg (p less than 0.05); gradient reduction in this group ranged from 40 to 85% (mean 57.5%). The gradient at follow-up increased in three of these four patients and decreased in one (the only late gradient less than 25 mm Hg). Late gradient was less than 35 mm Hg in two of the four patients and was reduced by 43 and 57%, respectively, in the other two.(ABSTRACT TRUNCATED AT 250 WORDS)

Percutaneous umbilical blood sampling allows direct access to the fetal circulation. We describe our experience with the procedure in the first 100 patients whose fetuses were at risk for hemolytic anemia, chromosomal abnormalities, coagulopathy, or intrauterine infection. Hematologic indices, including hemoglobin, hematocrit, red blood cell count, white blood cell count, and platelet count, were analyzed from 50 of the fetuses who were normal at delivery. Normal values and gestational age regression curves (from 17 to 37 weeks' gestation) are presented. The technique and complications of the procedure are described. Percutaneous umbilical blood sampling affords useful information in prenatal diagnosis and entails a low rate of complications

The purpose of this investigation was to evaluate the efficacy, technique, and follow-up results of balloon dilation angioplasty for valvular pulmonary stenosis. Percutaneous dilation was performed on 63 patients with pulmonary stenosis (ages 3 months to 76 years, mean = 4.3 years). In 43 patients, a single balloon was used; in 20 patients two balloons were used simultaneously. The pressure gradient across the pulmonary valve was determined with right ventricular and main pulmonary artery catheters. Pressure gradients simultaneously were estimated by continuous wave Doppler (CWD) during catheterization. The peak systolic ejection gradient was obtained by both techniques both pre- and postangioplasty. There was excellent linear correlation between the simultaneous catheter pressure gradient and the pressure gradient estimated by Doppler (r = 0.99). Follow-up pressure gradient estimations by Doppler echocardiogram were obtained in 30 patients between 6 months and 30 months postcatheterization (mean = 13 months). The mean preangioplasty gradient of 64 mm Hg (range 30-160 mm Hg) was reduced to 22 mm Hg (range 2-31 mm Hg). A significant reduction of transvalvular gradient (52-95%, mean 68%) occurred in each patient. A linear correlation was found between the predilation gradient and the pressure gradient drop (r = 0.92). Mean follow-up gradient by Doppler was 20 mm Hg (range 0-31 mm Hg), and there was no significant difference between these gradients and the postdilation gradient. No important complications were noted. These data confirm that balloon dilation angioplasty for valvular pulmonary stenosis is safe and effective, and suggest that stenosis does not recur

Despite the generally excellent success with balloon dilation for the stenotic lesions of congenital and acquired heart disease, technical difficulties sometimes prevent satisfactory results. Such technical difficulties include: a large diameter of the anulus of the stenotic lesion relative to available balloon diameter, difficulty in the insertion or removal of the larger balloon catheters, and permanent damage to or obstruction of the femoral vessels by the redundant deflated balloon material of the large balloons. A double balloon technique was initiated to resolve these difficulties. With this method, percutaneous balloon angioplasty catheters were inserted in right and left femoral vessels, placed side by side across the stenotic lesion and inflated simultaneously. Dilation procedures using the two balloon technique were performed in 41 patients: 18 with pulmonary valve stenosis, 14 with aortic valve stenosis, 5 with mitral valve stenosis, 3 with vena caval obstruction following the Mustard or Senning procedure and 1 with tricuspid valve stenosis. Patient ages ranged from 1 to 75 years (mean 17.8) and patient weights ranged from 8.9 to 89 kg (mean 42.3). Balloon catheter sizes ranged from 10 to 20 mm in diameter. Average maximal pressure gradient in mm Hg before dilation was 61 in pulmonary stenosis, 68 in aortic stenosis, 21 in mitral stenosis, 12 in tricuspid stenosis and 25 in vena caval stenosis. Average maximal valvular pressure gradient after dilation was 13 in pulmonary stenosis, 24 in aortic stenosis, 4 in mitral stenosis, 0 in tricuspid stenosis, and 1 in vena caval stenosis. No major complications were encountered with the procedures.(ABSTRACT TRUNCATED AT 250 WORDS)