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We report a case of infective endocarditis secondary to Enterococcus avium in a 1-year-old infant with tetralogy of Fallot and a Blalock-Taussig shunt. To our knowledge, this is the first case of E. avium endocarditis to be reported.

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OBJECTIVES: We sought to determine the prevalence and natural history of cardiac tumors in patients referred for fetal echocardiography. BACKGROUND: Cardiac tumors are rare; the prevalence, reported from autopsy studies of patients of all ages, varies from 0.0017% to 0.28%. Despite many case reports, the prevalence and natural history of fetal cardiac tumors are unclear. METHODS: Fourteen thousand fetal echocardiograms recorded over an 8-year period in seven centers were available for retrospective review. Medical records and echocardiograms were studied to determine the reason for referral, family history of tuberous sclerosis, prenatal and postnatal course and tumor description and type. RESULTS: Cardiac tumors were present in 19 pregnancies (0.14%). Gestational age at diagnosis ranged from 21 to 38 weeks. The most common indication for referral was a mass on an obstetric ultrasound study. The tumors were singular in 10 patients and multiple in 9. Tumor size ranged from 0.4 x 0.4 to 3.5 x 4 cm, and the majority of tumors were not hemodynamically significant. There were 17 patients with rhabdomyomas, 1 with a fibroma and 1 with an atrial hemangioma. Tuberous sclerosis complex was diagnosed in 10 patients. Partial or complete tumor regression was seen in eight patients; tumors were unchanged in five; and three required operation. CONCLUSIONS: Fetal cardiac tumors, a rare condition, are often benign. The majority of tumors are rhabdomyomas, but not all fetuses with rhabdomyoma have tuberous sclerosis.

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Seventeen consecutive patients undergoing bidirectional cavopulmonary anastomosis (BDCPA) using normothermic, noncardioplegic, cardiopulmonary bypass were studied preoperatively and early postoperatively (12.2 days) using transthoracic echocardiographic techniques. The purpose of the study was to assess the changes in left ventricular mechanics associated with the change in blood flow related to the BDCPA. Of the 17 patients, pulmonary atresia and tricuspid stenosis was present in 7, tricuspid atresia in 6, double inlet left ventricle in 3, and severe pulmonic stenosis with straddling of the tricuspid valve in 1. All other forms of single ventricle type anatomy and other patients undergoing BDCPA were excluded for the purposes of this study. The mean left ventricular end-diastolic volume index fell from 120 mL/m2 to 91.1 mL/m2 (p < 0.05). Similarly the left ventricular end-systolic volume index fell from 55.8 mL/m2 to 42.3 mL/m2, respectively (p < 0.05). The stroke volume index also fell from 64.5 mL/m2 to 48.8 mL/m2. Left ventricular ejection fraction was preserved and was unchanged in every patient. It was concluded that BDCPA, performed as described above, preserves left ventricular function and that the systolic and diastolic volumes as well as the stroke volume are significantly decreased as evidence of improvement of left ventricular mechanics. The continued use of the BDCPA either as a definitive procedure or as a staged procedure in preparation for total cavopulmonary connection is therefore endorsed.

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Recent development of small diameter (7 mm) biplane transesophageal echocardiography (TEE) probes has extended the select application of TEE to neonates, infants, and small children. We reviewed indications and uses for TEE in our neonatal and pediatric cardiac intensive care units over a 2-year period. Sixty-six TEE studies were performed in preoperative and postoperative settings in the intensive care unit after standard transthoracic studies. Age and weight of subjects ranged from 1 day to 16 years (mean 19 months) and 2.5 to 52 kg (mean 8.3 kg), respectively. Eighty-three percent of studies were performed in the immediate postoperative period after correction or palliation of congenital heart disease (including transposition of the great arteries, tetralogy of Fallot, atrioventricular septal defect, right ventricular conduit revision, Glenn shunt, and neonatal orthotopic heart transplantation). TEE advantages included unobstructed echo windows, particularly in postoperative settings of open sternum or high ventilatory support. Biplane imaging and Doppler provided additional hemodynamic information not otherwise detectable by noninvasive means. Posttransplant left and right ventricular function was immediately assessable. Overall findings included: residual shunts 24%, valvar insufficiencies 43%, residual gradients 4%, and left ventricular function or wall motion abnormalities 12%. TEE is a useful noninvasive adjunct in intensive care management of pediatric cardiac patients.

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We describe for the first time the use of transesophageal echocardiography to guide cardiac catheterization performed at the bedside in the pediatric cardiac intensive care unit. The procedure was performed on a 2.6-kg infant with ventricular and atrial septal defects after repair of coarctation of the aorta. Poor hemodynamic status prevented obtaining the required hemodynamic information from cardiac catheterization in the cardiac catheterization laboratory. Transesophageal echocardiography provided cardiac and vascular imaging, which helped guide catheter placement where the small size of the infant and the extensive thoracic postoperative bandages prevented obtaining the echocardiographic information from other windows.

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TEE applications for children and infants with congenital heart disease are now clinically established and TEE is being used in most major pediatric cardiovascular centers. This technology has evolved to the point that it is capable of providing diagnostic quality imaging, even for small infants. With expanding technology and increasing experience, new and more sophisticated applications will be found for pediatric TEE.

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Dramatic changes occur in the circulation of the newborn during the transition from fetal to neonatal life. Closure of the foramen ovale and ductus arteriosus, decrease in pulmonary vascular resistance, and improvement in right ventricular compliance are among these changes. These physiological-anatomical events were characterized by means of two-dimensional, Doppler and color flow echocardiography. Forty-five full-term infants (22 male, 23 female) were studied at a mean age of 4.2 hours (T1), 25.5 hours (T2), 49.8 hours (T3), and 73.8 hours (T4) by two-dimensional, Doppler and color flow echocardiography. At T1, T2, T3, and T4, the ductus arteriosus was patent by color flow echocardiography in 100%, 34%, 22%, and 11%, respectively. Conversely, patency of the ductus by Doppler alone was detected in 100% (T1), 13% (T2, T3), and 11% (T4). Reversal of flow in the descending aorta, reflective of diastolic ductal filling, was not sensitive in detecting ductal patency (T1 50%, T2 3%, T3 and T4 0%). The patency of the foramen ovale was noted to decrease over the course of the study. Right ventricular compliance was quantitatively assessed by pulsed-Doppler diastolic properties (E-to-A ratio). This changed significantly from T1 to T4 (0.90 to 0.97) reflecting improving compliance of the right ventricle. The ratio of acceleration to ejection time, a Doppler estimation measure of pulmonary vascular resistance, increased from 0.28 to 0.33 (T1 to T4) reflecting a decrease in pulmonary vascular resistance.(ABSTRACT TRUNCATED AT 250 WORDS)

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Two newborn infants with transposition of the great vessels and an intact ventricular septum were cyanotic when they underwent bedside balloon atrial septostomy in the neonatal intensive care unit. The procedure was conducted under direct transesophageal two-dimensional echocardiographic guidance. Success of the procedure was evaluated with color flow Doppler and the two-dimensional assessment of the septostomy. This procedure has advantages over catheterization and its attendant fluoroscopy, which usually are performed in the catheterization laboratory, expose the patient to radiation, and necessitate transportation of a patient who is hemodynamically unstable to an often distant catheterization laboratory. Transesophageal echocardiography has several advantages over transthoracic echocardiography in aiding this procedure. It provides an optimal view of the atrial septum, while keeping the chest exposed for observation of respiratory effort and for improved thermoregulation.

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Panoramic transesophageal echocardiography is a new development in transesophageal echocardiography (TEE) technology, which yields a wide-angle imaging field for real-time two-dimensional and color flow imaging. We report our early experience in patients with the use of an annular-array TEE probe that provides a wide, 270 degrees angle imaging field for two-dimensional echocardiographic imaging. The field of view can, however, be narrowed to 15 degrees . The field of view for color flow imaging can be varied from 180 degrees to 10 degrees . Pulsed-Doppler recordings of flow velocity are also possible. This TEE system provides a panoramic vision of cardiac and paracardiac structures from the esophagus and stomach. Besides cardiovascular structures, other thoracic and upper abdominal organs can be visualized. The wide field of view allows a better comprehension of the cardiac anatomy and its relationship with adjacent structures. The initial experience suggests that this method, besides providing the usually required diagnostic information, may have a number of additional applications. Its clinical potential and directions for future developments are reviewed.

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Transesophageal echocardiography using Doppler color flow mapping with both single plane transverse and longitudinal pediatric probes was performed in 127 infants and children both intraoperatively and postoperatively. The 127 patients ranged in age from 1 day to 18 years (mean 37 months) and in weight from 2.4 to 45 kg (mean 13.2); 157 studies were successfully performed (100% of attempted) with no complications. Transesophageal two-dimensional echocardiography combined with pulsed wave Doppler ultrasound and color flow mapping provided detailed morphologic as well as physiologic information, with additional information provided overall in 56% of the cases. Typical limitations of single-plane transverse imaging were overcome using a prototype longitudinal-axis pediatric probe. Both left and right ventricular outflow tracts, distal pulmonary arteries and all of the interventricular and atrial septa were visualized. Assessment of surgical repair intraoperatively as well as assessment postoperatively for cardiac function, residual intracardiac shunts, residual valvular sequelae and other hemodynamic events was important and easily performed in an otherwise inaccessible patient. Transesophageal echocardiography in infants and in small children is a valuable "noninvasive" imaging technique that offers important additional and complementary information.

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The use of Doppler echocardiography is a routine part of the noninvasive assessment of the patient with heart disease. In children with congenital heart disease, pulsed- and continuous-wave Doppler echocardiographic techniques provide accurate, reproducible hemodynamic data relative to structural defects. Doppler color flow imaging, however, allows for qualitative assessment of blood flow patterns, which may give important insights into the changing physiology of the newborn infant or that of a patient in the medical or surgical intensive care settings. Ten cases are presented in which this flow information is instructive in understanding the physiological sequelae of congenital heart disease.

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An infant with complete atrioventricular septal defect (atrioventricular canal) was examined by standard transthoracic two-dimensional pulsed Doppler and color Doppler echocardiography. No evidence of ductus arteriosus was present. Preoperative transesophageal echocardiography identified a patent ductus: the left-to-right shunt seen by color Doppler echocardiography was enhanced by pharmacologic maneuvers aimed at decreasing pulmonary vascular resistance and increasing systemic vascular resistance. Transesophageal echocardiography was found to be more sensitive than transthoracic echocardiography even in a small infant.

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In children with a ventricular septal defect, transseptal blood flow has been demonstrated angiographically to be bidirectional in all but the smallest defects. To investigate this phenomenon noninvasively, two-dimensional Doppler color flow echocardiography was used in 77 patients (aged 1 day to 15.5 years, mean 24 months). During isovolumetric contraction, flow was seen from the left to the right ventricle in 82 (98%) of 84 studies. During isovolumetric relaxation, right to left flow was noted in 72 (96%) of 75 studies. During early diastole in patients with an isolated defect, flow was initially from the right to the left ventricle and subsequently reversed to become left to right in 29 (91%) of 32 studies. In patients with concomitant right ventricular volume overload, flow across the ventricular defect was from the right to the left ventricle throughout diastole in 30 (86%) of 35 studies. In each of four patients with d-transposition of the great vessels and each of two patients with 1-transposition of the great vessels, blood flow was from the morphologic left ventricle to the morphologic right ventricle during isovolumetric contraction and from the morphologic right ventricle to the morphologic left ventricle during isovolumetric relaxation. These results demonstrate 1) the complex nature of intracardiac shunting in children with ventricular septal defect as previously shown by angiography; 2) an intrinsic functional difference in the contractile and relaxation properties of the morphologic left and right ventricles; and 3) differences in diastolic blood flow patterns between children with an isolated defect and those with a ventricular septal defect and right ventricular volume overload (p = 0.0001).

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Noninvasive cardiac imaging has dramatically altered the practice of cardiology, specifically, the pediatric patient with congenital heart disease over the past decade. Since the introduction of transesophageal echocardiography nearly 15 years ago, addition of high-resolution cross-sectional imaging combined with Doppler color flow mapping has provided a new window to examine the heart. Recently, miniaturization of the transesophageal probe to "pediatric-size" has enabled its use in the smallest of infants to add significantly to the assessment of congenital heart malformations. Most recently, addition of a longitudinal-plane probe to the already-existent, transverse-plane probe has made biplane transesophageal echocardiography a reality with significant additional information being provided by orthogonal images of the cardiac structures. We used these probes in complementary fashion in 30 studies performed in 23 patients ages 1 day to 12 years with a mean of 35 months, weighing 2.6-40 kg (mean 12.4 kg). These studies were performed in the operating room, intensive care unit, cardiac catheterization laboratory, and outpatient department. Limitations of single-plane, transverse transesophageal echocardiography were overcome using the longitudinal-axis pediatric probe: left and right ventricular outflow tracts, distal pulmonary arteries, and all of the interventricular and atrial septa were easily visualized. Its use in the operating room and postoperative cardiac intensive care unit for continuous ventricular monitoring in otherwise-inaccessible patients also provided critical information. Transesophageal echocardiography in infants and small children is a valuable "noninvasive" imaging technique which, with addition of complementary longitudinal-plane views, offers important additional information regarding congenital heart malformations and their repair.

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