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Biomedical subjects

D R Fulton

Publications and source records attributed to D R Fulton.

At least 37 records · Page 2Linked to original sources
PubMed1994

Physiologic significance of chronic coronary aneurysms in patients with Kawasaki disease.

OBJECTIVES: The aim of this study was to determine whether persistent coronary aneurysms in patients with Kawasaki disease are associated with altered myocardial perfusion and function. BACKGROUND: Some patients with Kawasaki disease have died suddenly because of severe coronary artery stenosis; others have chronic coronary aneurysms. METHODS: Eleven patients with chronic coronary aneurysms were enrolled in the study. The size of the aneurysms and the degree of associated stenosis were determined by angiography in nine patients and by echocardiography in two. All patients underwent simultaneous function and myocardial perfusion assessment during symptom-limited exercise by echocardiography and technetium-99m sestamibi imaging, respectively. RESULTS: Of 33 vascular territories, 18 contained coronary aneurysms measuring 3.5 to 10 mm. Three aneurysms were associated with significant stenosis as detected by angiography. Of the 18 vascular territories, 13 were normal, and 5 manifested stress-induced perfusion defects; of the latter 5 areas, 4 had associated wall motion abnormalities. The three territories supplied by stenotic coronary arteries had both abnormal regional function and perfusion demonstrated during exercise. CONCLUSIONS: Patients with chronic coronary aneurysms may have associated stenosis, as detected by angiography, with a subjacent myocardium that is subject to abnormal perfusion and function. However, the majority of coronary aneurysms are associated with normal regional coronary flow reserve, as assessed by myocardial perfusion imaging, and even giant coronary aneurysms may be associated with normal coronary flow reserve and preserved regional myocardial function during stress.

Adolescent↗
PubMed1994

Dynamic three-dimensional echocardiographic imaging of congenital heart defects in infants and children by computer-controlled tomographic parallel slicing using a single integrated ultrasound instrument.

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.

Child, Preschool↗
PubMed1993

Toxic shock syndrome toxin-secreting Staphylococcus aureus in Kawasaki syndrome.

Kawasaki syndrome (KS), the main cause of acquired heart disease in children, is associated with the selective expansion of V beta 2+ T cells in peripheral blood. Our study suggests that KS may be caused by a superantigen--a staphylococcal or streptococcal toxin. Bacteria were cultured without knowledge of their origin, from the throat, rectum, axilla, and groin of 16 patients with untreated acute KS and 15 controls. Bacteria producing toxins were isolated from 13 of 16 KS patients but from only 1 of 15 controls (p < 0.0001). Toxic shock syndrome toxin (TSST) secreting Staphylococcus aureus was isolated from 11 of the 13 toxin-positive cultures, and streptococcal pyogenic exotoxin (SPE) B and C were found in the other 2. These toxins are known to stimulate V beta 2+ T cells. All TSST-producing KS isolates were tryptophan auxotrophs indicating they were clonally related. S aureus isolates from acute KS patients were unusual because they produced less lipase, haemolysin, and protease compared to other isolates (p < 0.01). S aureus colonies from KS patients were white, and could be easily mistaken for coagulase-negative staphylococci, whereas colonies of non-KS isolates were gold. These observations suggest that the expansion of V beta 2+ T cells in most patients with KS may be caused by a new clone of TSST-producing S aureus, and, in a minority of patients, SPEB-producing or SPEC-producing streptococci.

Bacterial Proteins↗
PubMed1993

Prophylactic administration of respiratory syncytial virus immune globulin to high-risk infants and young children. The Respiratory Syncytial Virus Immune Globulin Study Group.

BACKGROUND: Infants with cardiac disease or prematurity are at risk for severe illness caused by respiratory syncytial virus. Immune globulin with a high titer of antibodies against respiratory syncytial virus may offer infants and young children at risk protection from this serious, common respiratory illness. METHODS: We studied 249 infants and young children (mean age, eight months) who had bronchopulmonary dysplasia due to prematurity (n = 102), congenital heart disease (n = 87), or prematurity alone (n = 60). Respiratory syncytial virus immune globulin was given monthly to some of these children in either a high dose (750 mg per kilogram of body weight; n = 81) or low dose (150 mg per kilogram; n = 79); 89 controls received no immune globulin. Group assignments were random. Assessments of respiratory illness and management were conducted without knowledge of the children's group assignments. RESULTS: There were 64 episodes of respiratory syncytial virus infection: 19 in the high-dose group, 16 in the low-dose group, and 29 in the control group. In the high-dose group there were fewer lower respiratory tract infections (7, vs. 20 in the control group; P = 0.01), fewer hospitalizations (6, vs. 18 in the control group; P = 0.02), fewer hospital days (43, vs. 128 in the control group; P = 0.02), fewer days in the intensive care unit (P = 0.05), and less use of ribavirin (P = 0.05). In the low-dose group there was a significant reduction only in the number of days in the intensive care unit (P = 0.03). Adverse events during the 580 infusions were generally mild and included fluid overload (in five children), oxygen desaturation (eight), and fever (six). Six children died: three in the high-dose group, three in the low-dose group, and none in the control group (P = 0.15), but no death was attributed to the use of immune globulin or to illness caused by respiratory syncytial virus. CONCLUSIONS: Administration of high doses of respiratory syncytial virus immune globulin is a safe and effective means of preventing lower respiratory tract infection in infants and young children at high risk for this disease.

Antibodies, Viral↗
PubMed1993

Controlled trial to evaluate protection of high-risk infants against respiratory syncytial virus disease by using standard intravenous immune globulin.

We performed a randomized, controlled trial of intravenous immune globulin (respiratory syncytial virus [RSV] neutralizing [Nt] antibody titer of 1:950 in 5% solution) to evaluate protection against RSV-induced disease over two respiratory virus seasons. Forty-nine children (mean age at enrollment, 4.5 months) with severe congenital heart disease or bronchopulmonary dysplasia were randomized as follows. Twenty-four patients were followed as controls and received no immune globulin. Twenty-five patients received monthly infusions of immune globulin at a dose of 500 mg/kg of body weight. There was a similar distribution between groups of patients with heart disease and bronchopulmonary dysplasia. There were 12 culture-proven RSV infections, 6 in the prophylaxis group and 6 in the control group. There was a trend toward less severe RSV illness in immune globulin recipients, as measured by length of hospitalization. Four of the six immune globulin recipients were hospitalized for a total of 35 days (mean, 8.8 +/- 5.0 days) because of RSV illness, in contrast to 51 hospital days (mean, 12.8 +/- 7.6 days) among RSV-infected controls. We conclude that monthly infusions of standard immune globulin containing RSV Nt antibodies may be safely administered to high-risk children, but that standard intravenous immune globulin does not contain sufficient RSV Nt antibody titer to fully protect against severe RSV illness.

Humans↗
PubMed1993

A new noninvasive method for the estimation of peak dP/dt.

BACKGROUND: Peak dP/dt is a good index of ventricular performance that is not influenced by afterload, wall motion abnormalities, or the variations in ventricular anatomy and morphology commonly encountered among patients with congenital heart disease. Unfortunately, the clinical utility of peak dP/dt has been limited by the fact that its measurement generally requires an intraventricular catheter. However, peak dP/dt occurs during isovolumetric contraction, and the pressure rise during isovolumetric contraction is almost linear. Therefore, the mean dP/dt during isovolumetric contraction (mean dP/dtic), ie, the ratio of the rise in pressure during isovolumetric contraction (aortic diastolic pressure minus the systemic ventricular end-diastolic pressure [VEDP]) over the isovolumetric contraction time, should provide a good estimate of peak dP/dt that could be generated noninvasively. METHODS AND RESULTS: Echo/phonocardiography was used to measure the isovolumetric contraction time and a blood pressure cuff to estimate aortic diastolic pressure of 27 patients (age, 1 day to 77 years) with congenital or acquired heart disease. VEDP was determined by three methods: (1) intraventricular catheter, (2) assumed VEDP of 10 mm Hg, and (3) assignment of a normal or elevated value on the basis of clinical history. The three estimates of mean dP/dtic thus generated were compared with simultaneous measurements of peak dP/dt obtained during cardiac catheterization. Invasively measured peak dP/dt correlated well with the indirect determinations (r = .95, .89, and .92 for methods 1, 2, and 3, respectively; P < .0001). CONCLUSIONS: Echo/phonocardiography can be used in conjunction with a blood pressure cuff and indirect estimates of VEDP to generate mean dP/dtic, an index of ventricular function that approximates and closely correlates with peak dP/dt. This noninvasive measurement can be obtained in almost any patient and may be useful in the assessment of ventricular performance in a variety of cardiovascular disorders.

Adolescent↗
PubMed1993

Restoration of the pulmonary valve reduces right ventricular volume overload after previous repair of tetralogy of Fallot.

BACKGROUND: Severe pulmonary regurgitation (PR) and associated right ventricular (RV) dilatation are late complications of surgical repair of tetralogy of Fallot (TOF). For the past several years, we have restored pulmonary valve competence with the exclusive use of cryopreserved allografts. METHODS AND RESULTS: Sixteen patients with symptoms of diminished exercise tolerance and echocardiographic evidence of progressive PR with severe RV dilatation underwent placement of allografts in the RV outflow tract at a median age of 12 years (10 years after TOF repair). Abnormal exercise tolerance tests were documented in 10 patients. Additional surgical procedures included pulmonary artery augmentation (n = 6), closure of residual left to right shunts (n = 3), and subendocardial resection for monomorphic ventricular tachycardia (n = 1). Six patients had either preoperative or postoperative balloon dilations of pulmonary artery stenoses. All patients had symptomatic improvement after allograft insertion. At a mean follow-up of 26.4 +/- 3.4 months, the severity of PR improved in all but one patient. In 12 patients (group 1), conduit regurgitation was either trace (n = 11) or mild (n = 1). Four patients (group 2) had moderate conduit regurgitation. In a retrospective analysis, pulmonary artery diameters and cross-sectional areas were significantly smaller in the group 2 patients compared with the group 1 patients. With the exception of one patient, RV end-diastolic diameter (RVEDD/BSA) fell after allograft insertion in each patient (P < .01). The reduction in RVEDD/BSA was significantly greater in group 1 than in group 2 (31.8 +/- 3.4% versus 21.4 +/- 11.0%, P < .05). CONCLUSIONS: Thus, restoration of the pulmonary valve with cryopreserved allografts improved exercise tolerance and diminished RV volume overload in patients with severe PR after previous repair of TOF. Optimal results were achieved in patients who did not have significant residual pulmonary artery distortion.

Child↗
PubMed1992

Immune hemolysis, disseminated intravascular coagulation, and serum sickness after large doses of immune globulin given intravenously for Kawasaki disease.

One week after treatment with intravenously administered immune globulin and aspirin, a child with Kawasaki disease had persistent fever and an increase in coronary artery diameter to greater than 3 mm. Two additional doses of immune globulin were given intravenously. Rapid hemolysis occurred, followed by disseminated intravascular coagulation and serum sickness. Clinicians should be aware that immune globulin preparations contain antibodies to blood-type antigens that may cause significant hemolysis and disseminated intravascular coagulation.

ABO Blood-Group System↗
PubMed1991

A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome.

BACKGROUND: Treatment of acute Kawasaki syndrome with a four-day course of intravenous gamma globulin, together with aspirin, has been demonstrated to be safe and effective in preventing coronary-artery lesions and reducing systemic inflammation. We hypothesized that therapy with a single, very high dose of gamma globulin would be at least as effective as the standard regimen. METHODS: We conducted a multicenter, randomized, controlled trial involving 549 children with acute Kawasaki syndrome. The children were assigned to receive gamma globulin either as a single infusion of 2 g per kilogram of body weight over 10 hours or as daily infusions of 400 mg per kilogram for four consecutive days. Both treatment groups received aspirin (100 mg per kilogram per day through the 14th day of illness, then 3 to 5 mg per kilogram per day). RESULTS: The relative prevalence of coronary abnormalities, adjusted for age and sex, among patients treated with the four-day regimen, as compared with those treated with the single-infusion regimen, was 1.94 (95 percent confidence limits, 1.01 and 3.71) two weeks after enrollment and 1.84 (95 percent confidence limits, 0.89 and 3.82) seven weeks after enrollment. Children treated with the single-infusion regimen had lower mean temperatures while hospitalized (day 2, P less than 0.001; day 3, P = 0.004), as well as a shorter mean duration of fever (P = 0.028). Furthermore, in the single-infusion group the laboratory indexes of acute inflammation moved more rapidly toward normal, including the adjusted serum albumin level (P = 0.004), alpha 1-antitrypsin level (P = 0.007), and C-reactive protein level (P = 0.017). Lower IgG levels on day 4 were associated with a higher prevalence of coronary lesions (P = 0.005) and with a greater degree of systemic inflammation. The two groups had a similar incidence of adverse effects (including new or worsening congestive heart failure in nine children), which occurred in 2.7 percent of the children overall. All the adverse effects were transient. CONCLUSIONS: In children with acute Kawasaki disease, a single large dose of intravenous gamma globulin is more effective than the conventional regimen of four smaller daily doses and is equally safe.

Acute Disease↗
PubMed1990

Autoimmune hemolytic anemia in Kawasaki disease: a case report.

A 3-year-old boy presented with the fever, conjunctivitis, rash, and lymphadenopathy diagnostic of Kawasaki disease. Treatment with antibiotics, aspirin, and intravenous immunoglobulin was instituted. The hematocrit decreased from 35 percent on admission to 11 percent by hospital Day 10, and the white cell count had increased from 13.7 to 42 x 10(3) per microL, and the patient had a leukoerythroblastic blood smear. The direct antiglobulin test demonstrated IgG but not complement on the red cell (RBC) surface. An acid eluate reacted (titer of 4) with all panel cells in the antiglobulin phase. Intravenous immunoglobulin from the same lot used for treatment did not contain antibody that reacted with the patient's group O RBCs or a panel of group O RBCs, but did contain IgG anti-A and -B (titer of 4). The patient received a transfusion and was given methylprednisone. The direct antiglobulin test and acid eluate were negative 4 days later. The patient had an uneventful recovery. The distinction between antibody-mediated hemolytic anemia and autoimmune hemolytic anemia is important in the treatment of this disease.

Anemia, Hemolytic, Autoimmune↗
PubMed1988

Effects of current therapy of Kawasaki disease on eicosanoid metabolism.

Coronary artery inflammation in Kawasaki disease is accompanied by thrombocytosis and platelet activation. It was hypothesized that abnormal metabolism of bioactive eicosanoids could result from or contribute to these events. Circulating plasma thromboxane B2, 6-keto-prostaglandin F1 alpha and prostaglandin E were measured by double antibody radioimmunoassay in patients with Kawasaki disease before and after aspirin alone or aspirin and intravenous gamma globulin therapy. Plasma prostaglandin E concentrations were normal in all patient groups. Pretreatment thromboxane B2 was elevated compared with age-matched controls, fell moderately with high-dose aspirin (60 to 100 mg/kg/day) and marginally increased with low-dose aspirin (3 to 5 mg/kg/day) 6 to 8 weeks after treatment. Plasma 6-keto-prostaglandin F1 alpha was not detected in 12 of 16 patients before therapy and remained low in all but 1 patients by 6 to 8 weeks. Thromboxane B2 correlated weakly with serum salicylate concentration but had no relation to platelet mass. The results in these patients with Kawasaki disease indicate only partial thromboxane suppression and depressed prostacyclin generation regardless of therapy. This balance favors coronary vasoconstriction and platelet aggregation capable of potentiating myocardial ischemia or infarction. The results justify consideration of higher or more frequent aspirin doses for longer duration and thromboxane receptor blockade in this disease.

6-Ketoprostaglandin F1 alpha↗
PubMed1988

One-year follow-up of cardiac and coronary artery disease in infants and children with Kawasaki disease.

Of 75 patients meeting clinical criteria for Kawasaki disease, 67 patients were followed with serial ECG and echocardiograms (echo) for at least 1 year. Sixteen patients had coronary artery aneurysms and three had dilated coronary arteries as assessed by initial two-dimensional (2D) echo and/or angiocardiographic studies. ECG findings of first and second-degree heart block and ST-T wave changes (36%) as well as M-mode echo abnormalities (45%) including pericardial effusion, decreased ventricular septal motion, and decreased left ventricular function were all normalized by 6 months, except in two infants who continued to have decreased function and flat ventricular septal motion. In four patients with single proximal coronary aneurysm and two with dilated coronary artery, resolution of lesions occurred by 1 year. Among 11 patients with multiple coronary aneurysms discovered by initial studies, the 2D echo became normal in seven and showed aneurysms in four as corroborated by angiogram. One patient with an isolated distal coronary aneurysm, not seen by initial or serial echo, had a normal follow-up angiogram. Our study suggests that in Kawasaki disease careful, serial, 2D echo is sufficient to monitor the course of coronary artery disease that tends to regress and follow a benign course. However, the long-term effects of this disease on cardiac and coronary arteries remain to be elucidated.

Child↗
PubMed1987

Cor triatriatum associated with partial anomalous pulmonary venous connection to the coronary sinus: echocardiographic and angiocardiographic features.

An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.

Angiocardiography↗
PubMed1986

The use of reversed phase cartridges (C18) in processing plasma for analysis of indomethacin by high performance liquid chromatography.

The purpose of these studies was to develop a high performance liquid chromatographic (HPLC) assay for plasma levels of indomethacin. A reversed phase (C18 Sep-Pak) cartridge was used to process plasma for absorption of indomethacin, the internal standard, and impurities. The recovery of plasma indomethacin and the added internal standard was quantitative from 0.5 ml of plasma. The assay was linear from 50 micrograms/l to 10 mg/l without concentration of the effluent from the Sep-Pak cartridge. The intra-assay coefficients of variation, for ten injections each to calibrate points at 0.15, 0.30, 0.50 and 1.00 mg/l, were 8.15%, 6.29%, 5.47% and 5.39%, respectively and for duplicates of duplicate calibration points were 7.51%, 6.32%, 4.41%, and 2.05%, respectively. The inter-assay coefficients of variation were 8.49%, 6.48%, 5.10%, and 2.22%, respectively. The sensitivity of the assay can be increased by a 3-5 fold concentration of the effluent from the Sep-Pak and preliminary experiments have indicated that as little as 100 microliter of starting plasma can be utilized in the assay. The assay can be used to determine the concentration of indomethacin in small volumes of plasma. Since C18 Sep-Pak cartridges were employed to remove contaminating substances, sensitivity and reproducibility were both high while column longevity and efficiency were excellent.

Chromatography, High Pressure Liquid↗
PubMed1985

Ventricular tachycardia in children without heart disease.

To assess the natural history of ventricular tachycardia (VT) in children without heart disease, the clinical course of 26 children, aged 1 day to 15 years at initial detection, was reviewed. Symptoms related to the rhythm were present in 8 children at some time during their course of follow-up. Evaluation included echocardiogram (19 patients), Holter monitoring (22 patients), graded treadmill exercise testing (16 patients) and invasive electrophysiology (4 patients). Exercise induced or exacerbated VT in 9 patients, but suppressed rhythm in 7. Ten patients were never treated. Of the remaining 16 treated patients, therapy was discontinued in 10 on the basis of the electrocardiographic or Holter monitor recordings. Six continued to receive therapy without complications. There were no known deaths over a period of 1 month to 34 years (mean 59 months, median 42 months). Although 2 patients have been lost to follow-up, based on our findings and a review of the reported cases of VT in children to date, after undergoing complete noninvasive cardiac evaluation symptomatic patients should be treated and studied with invasive electrophysiology if antiarrhythmic control is inadequate. The rate of VT or age at onset are not predictive of outcome in asymptomatic patients. As a group, these patients do not appear to benefit from therapy, but warrant follow-up since deaths have been reported in untreated asymptomatic patients.

Adolescent↗
PubMed1984

Measurement of extravascular lung water in infants and children after cardiac surgery.

Extravascular lung water (EVLW) was measured in 17 patients with congenital heart disease by the cold-green-dye, double indicator-dilution technique. In 5 control patients, EVLW was 4.7 +/- 0.5 ml/kg (111 +/- 13 ml/m2) (mean +/- standard deviation). Twelve patients were studied immediately after correction of their heart defects. In 6 patients with normal or decreased pulmonary blood flow preoperatively (Group I), EVLW was 6.2 +/- 1.9 ml/kg (122 +/- 46 ml/m2). This value is not significantly different from that of the control patients. In 6 patients with increased pulmonary blood flow and congestive heart failure preoperatively (Group II), EVLW was 15.7 +/- 3.8 ml/kg (270 +/- 60 ml/m2), which is significantly different from both control and Group I patients (p less than 0.01). There was no correlation of EVLW with pre- or postoperative left atrial pressure, length of cardiopulmonary bypass or deep hypothermic circulatory arrest, postoperative serum protein, albumin, hematocrit or cardiac index. Thus, EVLW in the immediate postoperative period is determined by preoperative pathophysiologic characteristics rather than by intraoperative management, and patients with congestive heart failure resulting from left-to-right shunts have increased EVLW despite normal left atrial pressures.

Blood Pressure↗
PubMed1984

Combined discrete subaortic stenosis and ventricular septal defect in infants and children.

Eight patients, aged 1 to 8 years, with discrete subaortic stenosis (DSS) and ventricular septal defect (VSD) were studied by 2-dimensional (2-D) and M-mode echocardiography. Initial cardiac catheterization and angiocardiography showed VSD and other associated cardiac lesions, including coarctation of the aorta and patent ductus arteriosus. None had evidence of DSS. Six patients underwent surgical repair of the associated lesions, but none required closure of the VSD. Ultimately, 6 patients had spontaneous closure of VSD, and 2 had a residual small VSD. Subsequent serial echocardiography showed evidence of subaortic membrane, prompting repeat cardiac catheterization, which confirmed moderate to severe peak systolic pressure gradients between the left ventricle and ascending aorta. Surgical resection of the membrane was performed in 5 patients. Thus, in patients with small or spontaneously closed VSDs, DSS may develop. Evaluation of the left ventricular outflow tract area is recommended in patients with small or closed VSD in whom a significant heart murmur or electrocardiographic abnormality remains.

Abnormalities, Multiple↗