Effectiveness of pharmacomechanical thrombolysis in infants and children.

Between March 1995 and February 2000, 10 children with major thromboses were treated with local pharmacomechanical thrombolysis. Clinical improvement was found in 8 patients: follow-up angiography showed complete thrombus resolution in 5 patients and subtotal resolution in 4.

Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism.

OBJECTIVES: To identify infants with hyperinsulinism caused by defects of the beta-cell adenosine triphosphate-dependent potassium channel complex and to distinguish focal and diffuse forms of hyperinsulinism caused by these mutations. STUDY DESIGN: The acute insulin response to intravenous calcium stimulation (CaAIR) was determined in 9 patients <20 years with diffuse hyperinsulinism caused by defective beta-cell sulfonylurea receptor (SUR1(-/-)), 3 patients with focal congenital hyperinsulinism (6 weeks to 18 months), a 10-year-old with insulinoma, 5 with hyperinsulinism/hyperammonemia syndrome caused by defective glutamate dehydrogenase (6 months to 28 years), 4 SUR1(+/-) heterozygotes with no symptoms, and 9 normal adults. Three infants with congenital focal disease, 1 with diffuse hyperinsulinism, and the child with insulinoma underwent selective pancreatic intra-arterial calcium stimulation with hepatic venous sampling. RESULTS: Children with diffuse SUR1(-/-) disease and infants with congenital focal hyperinsulinism responded to CaAIR, whereas the normal control group, patients with hyperinsulinism/hyperammonemia syndrome, and SUR1(+/-) carriers did not. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling revealed selective, significant step-ups in insulin secretion that correlated anatomically with the location of solitary lesions confirmed surgically in 2 of 3 infants with congenital focal disease and in the child with insulinoma. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling demonstrated markedly elevated baseline insulin levels throughout the pancreas of the infant with diffuse hyperinsulinism. CONCLUSIONS: The intravenous CaAIR is a safe and simple test for identifying infants with diffuse SUR1(-/-) hyperinsulinism or with focal congenital hyperinsulinism. Preoperative selective arterial calcium stimulation of the pancreas with hepatic venous sampling can localize focal lesions causing hyperinsulinism in children. The combination of these calcium stimulation tests may help distinguish focal lesions suitable for cure by local surgical resection.

Current concepts on imaging of thoracic vascular abnormalities.

Pediatric thoracic vascular abnormalities include many anatomic variants and pathologic conditions. Although some vascular variants are inconsequential and are discovered incidentally on imaging studies, several anomalies have profound effects on the airway and pulmonary parenchyma. Imaging plays a key role in evaluating the chest and its vasculature. The chest radiograph is usually the first screening study performed during the course of evaluation for a vascular abnormality. Cross-sectional imaging with CT and magnetic resonance imaging provides precise anatomic information and has in most cases replaced invasive diagnostic angiographic procedures. We describe common thoracic vascular abnormalities that occur in children and the imaging techniques currently used in their evaluation.

Regional wall motion and strain of transplanted hearts in pediatric patients using magnetic resonance tagging.

Abnormal ventricular systolic torsion is present during histological rejection in adult cardiac transplant patients. Because biomechanical properties of transplanted hearts in the baseline state have not been studied in children, pediatric patients were evaluated to quantify ventricular wall motion and strain. Eight transplant studies and eight normal controls were evaluated. Magnetic resonance tagging was performed to determine radial shortening, twist, and strain in four ventricular anatomic areas at two short-axis levels. Controls had counterclockwise twist. Six transplant studies had clockwise twist, six had akinetic regions, and all had regions of no twist. One demonstrated paradoxical motion of the septum. A comparison between transplant patients and controls revealed strain to be similar in all regions except one (superior wall at the atrioventricular valve level) and strain distribution to be different only in two of eight regions. Pediatric transplant patients demonstrate regional wall motion abnormalities in the absence of rejection. Compared with normal controls, the transplanted left ventricle maintains normal strain in the presence of abnormal twist. This may be a compensatory mechanism and have clinical implications.

The effects of congenital heart disease on the lungs.

The alterations in anatomy and physiology, which often accompany congenital heart disease, may have a major impact on the child's lungs. These changes in pulmonary blood flow and ventilation are usually demonstrable on chest radiography and should be recognized as manifestations of the underlying lesion and not mistaken for primary lung disease. Correlation with the clinical history and physical examination are essential for correct interpretation, but radiological findings should be viewed objectively and without bias to provide accurate and valuable information to the treating physician.

The impact of extended radiology attending coverage in a children's hospital.

OBJECTIVE: In order to achieve more "timely" interpretation of radiologic examinations, a 3 to 11 p.m. attending radiologist slot was incorporated into the daily schedule utilizing existing staff. Our purpose was to assess the effectiveness of this practice by measuring report generation times. MATERIAL AND METHODS: Using a radiology information system (DecRAD), the time between completion of the technical examination and dictation of the official report for general (plain film) studies was determined for a 2-month period and compared to similar periods 1 and 2 years prior to instituting extended hours. Emergency and portable (ICU) exams were similarly analyzed. RESULTS: The number of examinations reported within 2 h of technical completion increased by 8.5% (mean); reporting within 4 h increased by 20%; reporting within 6 and 12 h of completion each increased by 24%. Over 80% of cases were dictated within 12 h after the change in practice occurred; whereas, it took up to 24 h in preceding years. Analysis of emergency and portable ICU exams showed similar trends, and the number of next day "call-backs" to the emergency department was significantly reduced. CONCLUSION: By extending attending coverage, more plain film examinations, especially emergency cases and ICU portables, were read and reported within a shorter time providing prompt communication with clinicians and more opportunity to influence management decisions.

Effect of surgical reconstruction on flow profiles in the aorta using magnetic resonance blood tagging.

BACKGROUND: The aorta that has undergone an aorta-pulmonary artery anastomosis may not exhibit the same velocity profile as the nonreconstructed aorta, whose velocity profile is thought to be uniform across the vessel diameter (plug flow). This may have an impact on fluid dynamics and will alter Doppler flow calculations. Our objective was to determine the impact of surgical reconstruction on the velocity and flow profiles of the reconstructed ascending and descending aorta. METHODS: Using a magnetic resonance imaging tagging technique that labels flowing blood (bolus tagging), we studied 22 patients (mean age, 8.6 +/- 4.7 years) who had had a Fontan procedure. A cine sequence labeled the blood and acquired the image after 20 ms in the middle of the ascending aorta and behind the left atrium in the descending aorta. The repetition time was 50 ms. RESULTS: The reconstructed ascending aorta displayed a velocity profile skewed anteriorly, whereas in the nonreconstructed aorta, the velocity profile was flat. Reconstructed aortas also displayed flows that were higher anteriorly, took a longer time to reach maximum velocity, and were less like "plug" flow than the nonreconstructed aorta. The descending aorta, regardless of whether aortic reconstruction was present, displayed velocity profiles (at various phases of systole) skewed posteriorly. CONCLUSIONS: The reconstructed aorta displays disturbed flow, and the velocities across the ascending aortic diameter are more varied than those in aortas without reconstruction and are skewed anteriorly. The descending aortic velocity profile in children is skewed posteriorly, regardless of whether aortic reconstruction is present. This information may help design and build a "better" aortic reconstruction.

The nature of flow in the systemic venous pathway measured by magnetic resonance blood tagging in patients having the Fontan operation.

OBJECTIVES: Our objectives were twofold: (1) to determine cardiac and respiratory dependency of systemic venous pathway flow of patients having the Fontan operation with a total cavopulmonary connection and (2) to describe the velocity profile. Systemic venous pathway flow is hypothesized to be mostly respiratory dependent, to be laminar, and to have a smooth velocity profile. METHODS: Twenty-two patients having the Fontan operation (aged 8.6 +/- 4.7 years) underwent magnetic resonance blood tagging (bolus tagging). Systemic venous pathway spin-echo images parallel to the blood flow were used as a localizer. A saturation pulse labeled the blood, and a cine image was acquired at the inferior and superior venae cavae and midportion of the baffle in the systemic venous pathway, triggered to the electrocardiogram and gated to both end-expiration and end-inspiration. Repetition time was 50 msec. RESULTS: Flow in the systemic venous pathway was laminar throughout its course and was found to be phasic to both cardiac and respiratory cycles. Approximately 70% of flow was cardiac dependent, and the rest was respiratory. Highest flow occurred near end-systole and early diastole and in inspiration. Lowest flow occurred in diastasis. Velocity was highest and flow least "pluglike" in the mid-baffle area during cardiac or respiratory imaging (45 +/- 17 and 32 +/- 11 cm/sec, respectively). CONCLUSION: A substantial amount of pulmonary blood flow in patients who have undergone a total cavopulmonary connection type of Fontan operation has a cardiac component. Furthermore, we confirm that this flow is laminar but nonuniform across the systemic venous pathway. Highest flows occurred near end-systole and early diastole, as well as in inspiration, and the lowest flow occurred in diastasis. This information may help in designing the systemic venous pathway and optimizing medical management.

Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging.

OBJECTIVES: We sought to test the hypothesis that late ventricular geometry and performance changes occur in functional single ventricles as they progress through staged Fontan reconstruction. BACKGROUND: Indexes of ventricular geometry and performance are important in evaluating the functional state of the heart. Magnetic resonance imaging determines these indexes in complex ventricular shapes with minimal geometric assumptions. Previous studies have shown that 1 week after hemiFontan, the mass/volume ratio markedly increases. METHODS: Multiphase, multislice, spin echo (n = 5) and cine (n = 30) magnetic resonance imaging was performed in 35 patients with a functional single ventricle (1 week to 12 years old) at various stages of Fontan reconstruction (15 in the pre hemiFontan stage, 11 after [6 to 9 months] the hemiFontan procedure and 9 after [1 to 2 years] the Fontan procedure). Volume and mass were calculated at end-systole and end-diastole. Ventricular output was then obtained. Ventricular centroid motion was also calculated. RESULTS: No difference was noted (power > 72%) from the pre hemiFontan stage to 6 to 9 months after the hemiFontan procedure in (mean +/- SD) end-diastolic volume (104 +/- 24 vs. 123 +/- 40 cc/m2), mass (171 +/- 46 vs. 202 +/- 61 g/m2), ventricular output (7.9 +/- 2.2 vs. 6.6 +/- 2.4 liters/min per m2) or centroid motion (6.9 +/- 2.8 vs. 6.7 +/- 2. mm/m2). Patients in the Fontan group demonstrated a marked decrease in all indexes, indicating significant volume unloading and decrease in mass and ventricular performance. Mass/volume ratio was not significantly different among all three groups. CONCLUSIONS: No geometric and performance changes from the volume-loaded stage are noted 6 to 9 months after the hemiFontan procedure; however, major changes occur 1 to 2 years after the Fontan procedure. The dramatic changes in the mass/volume ratio seen early after the hemiFontan procedure were not detected at 6 to 9 months. Furthermore diminution of mass, volume and ventricular performance are present at least 2 years after the Fontan procedure.

Three-dimensional reconstruction of MR images in congenital heart disease.

Commercially available software is now available for reconstructing three-dimensional (3D) tomographic slices. We have used these 3-D images for 8 years to enhance anatomic diagnosis and functional evaluation pre- and postoperatively in children with congenital cardiac and great vessel diseases. The advantages of 3-D imaging are reduced examination time, improved display of complex intracardiac relationships, better understanding of relationships between great vessels and adjacent major airways, and facilitated demonstration of cardiovascular anatomy for those unfamiliar with tomographic images.

MR imaging and heart function in patients pre- and post-Fontan surgery.

MR imaging allows functional evaluation of the ventricles of the entire heart because it evaluates cardiac anatomy three-dimensionally. Such evaluation is independent of chamber size and shape, and virtually independent of mathematical assumptions. Applying standard measurements of volume and function in children who have undergone multi-staged Fontan operations, we have shown diminished ventricular volumes and mass, and decreased cardiac indices, in post-Fontan procedure. These variations may be predictive of ultimate outcome in these complex patients.

A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation.

BACKGROUND: Ventricular-ventricular interaction is known to occur in normal human heart. To determine whether it plays a role in the function of single right ventricles, systemic right ventricles were compared with and without a left ventricle mechanically coupled to it. METHODS AND RESULTS: A noninvasive magnetic resonance tagging technique (spatial modulation of magnetization [SPAMM]) that lays intersecting stripes down on the myocardium was used to examine 18 patients with systemic right ventricles: 7 with a single right ventricle who have undergone the Fontan procedure (age, 38.8 +/- 8.9 months) and 11 with transposition of the great arteries who have undergone an atrial inversion operation (age, 16.3 +/- 3.9 years). The motion of the intersection points was tracked through systole to determine regional twist and radial shortening. Shortening rates also were evaluated. Finite strain analysis was applied to the grid lines using Delaunay triangulation, and the two-dimensional strain tensor and principal E1 strains were derived for the various anatomic regions. Basal and apical short-axis planes through the ventricular wall were categorized into four distinct regions spaced equally around the circumference of the slice. We observed the following results. (1) Strain was greatest and heterogeneity of strain was least in patients with transposition of the great arteries who were status post atrial inversion operation (six of eight regions). Marked differences were noted in the distribution of strain within a given region, from endocardium to epicardium, and from atrioventricular valve to apical plane between patient subtypes and those with a normal left ventricle. (2) Contrary to the normal subject studied by the use of the same method, for both patient subtypes, there was counterclockwise twist in one region, clockwise twist in the posterior or inferior wall, and a transition zone of no twist at which the two regions of twist met. Normal human adult left ventricles studied in short-axis twist uniformly counterclockwise as viewed from apex to base. (3) Radial inward motion was greatest in the superior wall of both types of systemic right ventricle. The inferior walls of Fontan patients and the posterior (ie, septal) walls of patients with transposition of the great arteries, status post atrial inversion, moved paradoxically in systole. The shortening rate at the atrioventricular valve of patients with transposition of the great arteries, status post atrial inversion, was significantly lower than at the apex or in Fontan patients. CONCLUSIONS: Marked differences in regional wall motion and strain were demonstrated in systemic right ventricles, depending on whether a left ventricle was present to augment its function. Ventricular-ventricular interaction appears to play an important role in affecting the biomechanics of systemic right ventricles. These observations were markedly different from those in the normal systemic left ventricle. These techniques demonstrate tools with which we can begin to evaluate surgical outcomes using regional myocardial mechanics and may provide a clue to single right ventricle failure.

Transhepatic central venous access for cardiac catheterization and radiologic intervention.

We describe a technique of direct puncture and catheterization of the hepatic veins for cardiac catheterization and radiologic intervention in children who lack conventional access routes. Four patients underwent eight direct hepatic vein punctures to allow cardiac catheterization (3 patients) and 5-12 F sheath insertions for balloon dilatation of hepatic vein stenoses (1 liver transplant patient). Gelfoam was injected in the sheath tract for hemostasis. All procedures were successful, including five hepatic vein dilatations and right heart access for three cardiac catheterizations. There were no complications. When conventional venous access is precluded, direct percutaneous catheterization of hepatic veins is easy, effective, and probably low risk.

Contemporary interventional procedures for vascular disorders in children.

Pediatric interventional radiology offers new treatment options not possible a few years ago. Interventional radiologists treat not only certain causes of hemorrhage but also symptomatic arteriovenous malformations, renovascular hypertension, and portal hypertension. Interventionalists help maintain liver and renal transplants, increasing transplant patient survival. In addition to these selected disorders and types of radiological intervention, specific procedures such as thrombolysis, transjugular intrahepatic portosystemic shunts, and maintenance of hemodialysis access are discussed.

Vascular imaging in children.

Advances in technology have brought about the wide variety of imaging modalities that are presently available. The development of digital subtraction angiography, computed tomography, ultrasonography, and magnetic resonance imaging have had a particular impact on vascular imaging. These modalities, general principles of study selection, and imaging approaches for specific entities are reviewed.