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Biomedical subjects

G G Sandor

Publications and source records attributed to G G Sandor.

At least 19 recordsLinked to original sources

Postpericardiotomy syndrome: no evidence for a viral etiology.

BACKGROUND: Postpericardiotomy syndrome has been considered a disorder induced by viral infection. This conclusion is based on serologic criterions, but these may be unreliable following either cardiopulmonary bypass or transfusion therapy. Previous studies have not verified the proposed etiology either by isolation of viruses, or by detection of their genome. We sought, therefore, to clarify the role, if any, of viruses in this syndrome. METHODS AND RESULTS: We studied prospectively 149 children aged from 6 months to 16 years who were undergoing open heart surgery. Blood samples were collected from all prior to operation, and again 7 to 10 days post-operatively, and 47 were sampled at the time of development of symptoms of pericardial involvement. Serums were analyzed for the presence of IgM and IgG antibodies to cytomegalovirus, herpes simplex virus, and Epstein-Barr virus. The polymerase chain reaction was used for amplification when assessing the genome of the enteroviruses. Cultures for viruses were established on samples of stool, urine, and throat swabs collected 7 days post-operatively, and at the time of postpericardial symptoms. Pericardial fluid obtained from 5 patients with the syndrome was cultured for viruses, and tested for enterovirus genome. On the basis of clinical and echocardiographic findings, 34 children were determined to have definite evidence of the syndrome, 13 were considered to have possible evidence, and the results from these patients were compared to those from patients with no pericardial symptoms, the latter being matched for age and transfusion status. We isolated viruses from one or more sites in five patients with definite evidence (16%), from one (9%) of those with possible evidence, and from seven (19%) of the controls. All serums and pericardial samples were negative for enterovirus genome. IgM antibodies were found in only 5 patients, three with symptoms of pericardial involvement and two without. Rates of seroconversion to IgG for the viruses were lower in the patients with symptoms of pericardial involvement compared to controls, but were strongly influenced by transfusion status. CONCLUSION: Our study has provided no evidence to support a viral etiology for the postpericardiotomy syndrome.

Adolescent↗

Echocardiography and pulmonary function testing in childhood onset systemic lupus erythematosus.

The aim of this paper was to investigate the frequency of echocardiography (ECHO) and pulmonary function test (PFT) abnormalities in childhood onset systemic lupus erythematosus (SLE), and to determine the relationship of these abnormalities to disease activity. The charts of 50 patients with childhood onset SLE attending a pediatric rheumatology clinic were reviewed for ECHO and PFT studies. The frequency and description of ECHO and PFT abnormalities were documented. Possible associations of PFT and ECHO abnormalities with clinical cardiopulmonary disease, radiographic findings, and measures of lupus disease activity were evaluated. Forty patients (80%) had at least one ECHO study. Twenty-seven (68%) had an abnormal initial study. Nine of 14 patients with an initial abnormal ECHO had normal findings on repeated study. Three abnormalities were considered moderately severe. Thirty-three patients (66%) had at least one PFT performed. Sixteen (48%) were abnormal initially. Four of these 'abnormal' studies were repeated and the abnormalities persisted. Nine patients (27%) were considered to have a severe abnormality. Thirty-one children (62%) had both studies performed. An initial abnormal ECHO and abnormal PFT was found in 10 (32%) of these children. No relationship between ECHO or PFT abnormality and any measure of disease activity (physician's global assessment, anti DNA, C3 or ESR) could be found. Occult cardiac and pulmonary disease as demonstrated by ECHO or PFT occurs frequently in childhood onset SLE. If we wish to understand the natural history of these abnormal heart and lung findings, it will be necessary to do serial testing with ECHO and PFTs in this population.

Adolescent↗

Vascular abnormalities in Adams-Oliver syndrome: cause or effect?

We describe a young girl diagnosed with the Adams-Oliver syndrome (AOS) associated with double outlet right ventricle, portal hypertension, and pulmonary hypertension. We hypothesize that a congenital vascular abnormality is the underlying pathogenesis and that the cutaneous defects characteristically seen in AOS represent the most common manifestation of this. We suggest that AOS should not merely be considered a syndrome consisting of aplasia cutis congenita and terminal transverse limb defects but rather a constellation of clinical findings resulting from an early embryonic vascular abnormality.

Abnormalities, Multiple↗

Outcome of fetuses diagnosed with atrioventricular septal defect.

OBJECTIVE: To quantify the association of prenatally diagnosed atrioventricular septal defect with Down syndrome and to evaluate its impact on obstetric and neonatal outcomes. METHODS: Charts of 42 cases of atrioventricular septal defect diagnosed by fetal echocardiography from July 1985 to July 1997 were reviewed for prenatal history and outcome data (pregnancy outcome, pathologic confirmation, postnatal echocardiographic findings, and neonatal outcome). Statistical analysis was done using Fisher exact test and odds ratios. RESULTS: The mean gestational age at diagnosis was 26 weeks. Four cases could not be confirmed antenatally on repeat echocardiograms and were excluded. Reasons for referral of the remaining 38 fetuses included an abnormal four-chamber view in 76%. Twenty-two fetuses (58%) had abnormal karyotypes: 19 trisomy 21, one trisomy 18, one trisomy 13, and one mosaicism. The cardiac lesions were isolated in 20 fetuses (53%). After excluding cases of termination, ten of 12 fetuses (83%) with Down syndrome survived, compared with seven of 13 (54%) with normal karyotypes (P = .125). The odds of trisomy 21 were 16 times higher (95% confidence interval 3.0, 85.3) in fetuses with isolated cardiac lesions compared with those with associated cardiac anomalies. CONCLUSION: Prenatal diagnosis of atrioventricular septal defect was associated with a 58% risk of aneuploidy (mainly trisomy 21). Down syndrome fetuses with this cardiac anomaly appeared to have a better survival rate than fetuses with normal karyotypes. Our sample did not have enough power to show a statistically significant difference. When an isolated atrioventricular septal defect was diagnosed prenatally, the odds of trisomy 21 were significantly higher than when other associated cardiac lesions were diagnosed. This information should be considered in prenatal counseling for atrioventricular septal defect.

Adult↗

Kawasaki disease in the older child.

OBJECTIVES: To determine the prevalence of Kawasaki disease in older children and to evaluate its clinical presentation, time to diagnosis, and outcome in comparison with younger patients with the disease. METHODOLOGY: A retrospective analysis of all patients discharged with a diagnosis of Kawasaki disease at a pediatric tertiary care hospital over a 12-year period. RESULTS: A total of 133 patients were included in this study; 7.5% were 9 years of age or older at the time of illness. Patients were grouped by age: infants included children age 1 to 8 years of age and children 9 years of age or older. Older children had a higher frequency of abnormal cardiovascular physical examination (50%) versus children (6%) and infants (10%). The older age group and the infants had a higher prevalence of coronary artery abnormalities and poor left ventricular function than did the 1- to 8-year-olds. Eighty percent of the older children had coronary arteries that were either dilated or aneurysmal, and 30% demonstrated left ventricular dysfunction on initial echocardiography. The number of days to diagnosis after meeting the diagnostic criteria was 5.8 +/- 2.3 for infants, 5.2 +/- 1.5 for older children, and 1.9 +/- 0.3 for children. Older children had a complicated course of Kawasaki disease compared with younger patients. CONCLUSION: We found a higher prevalence of older children with Kawasaki disease at our center than has previously been reported. Older patients, as well as infants, had a higher rate of coronary artery abnormalities than did the children between 1 and 8 years of age. Older age at the time of illness or a delay in treatment may be important factors in determining cardiac involvement in Kawasaki disease.

Age Distribution↗

Differences in dose scheduling as a factor in the etiology of anthracycline-induced cardiotoxicity in Ewing sarcoma patients.

Clinical observation suggested a high prevalence of cardiac morbidity and mortality in children with Ewing sarcoma (ES) treated at B.C.'s Children's Hospital. We therefore compared 30 patients treated for Ewing sarcoma between 1978 and 1991 with 26 soft tissue sarcoma (STS) patients treated with similar chemotherapy over the same period of time. All patients were evaluated for cardiac function using echocardiography. Shortening fraction (SF) and left ventricular mass index (Massl) were compared before and after treatment. The role of chest irradiation, dose concentration (DC) of adriamycin (AD), total mean doses of AD, cyclophosphamide (CY) and actinomycin (AC) were analysed. SF for patients with ES and STS postchemotherapy was significantly lower (P < .001 and P = 0.0004, respectively) than pretreatment values. Postchemotherapy SF for ES was lower than STS (P = 0.0097). Massl for each group did not change significantly. Six of the ES patients had postchemotherapy SF of < 0.20, with three in congestive failure, two cardiac deaths and one heart transplant. One additional ES patient had sick sinus syndrome and needed a pacemaker. Among the STS patients only one had SF < .20 and none were symptomatic. There were no significant differences in the mean AD, CY and AC doses for ES versus STS. The difference in the DC of AD for ES (mean 744) compared to STS (mean = 362) was significant (P = < 0.001). Regression analysis indicated a trend for decreasing SF with increasing DC (P = 0.017). Chest irradiation did not appear to increase the likelihood of cardiotoxicity. ES patients had a higher prevalence of cardiac dysfunction compared to STS. Studies are required to evaluate the importance of the components of DC, i.e., size of the individual dose and frequency of administration of AD, and to look at other possible factors in the causation of cardiomyopathy in ES.

Anthracyclines↗

Influence of competitive pulmonary blood flow on the bidirectional superior cavopulmonary shunt. A multi-institutional study.

BACKGROUND: It is common practice to interrupt all alternative sources of pulmonary blood flow ("competitive flow") at the time of a bidirectional superior cavopulmonary anastomosis (BCPA), although the merits of this have not been systematically studied. METHODS AND RESULTS: We reviewed the early and medium-term clinical and hemodynamic findings in 108 consecutive patients 3 weeks to 25 years old (median, 1.9 years) undergoing BCPA at one of three institutions. Preoperatively, pulmonary blood flow was dependent on antegrade ventricular flow (n = 50), systemic-to-pulmonary shunts (n = 33), or mixed sources (n = 25). Postoperatively, competitive sources of pulmonary blood flow were left patent in 43 of 108 patients (40%). There were four early (3.7%) and four late deaths, none related to persistence of competitive flow. After BCPA, patients with competitive flow had significantly higher systemic oxygen saturations at 1 hour (85% versus 79%), 24 hours (84% versus 78%), and at hospital discharge (84% versus 78%) and required a shorter period of artificial ventilation (median, 9 versus 24 hours) and intensive care (median, 2 versus 4 days). Oxygen saturations at late follow-up (median, 2.8 years; range, 1 to 7) did not differ (83% versus 82%). No patient developed pulmonary arteriovenous malformations. CONCLUSIONS: Competitive flow is well tolerated in the short and medium term after BCPA, and early postoperative systemic oxygen saturations are improved. The long-term influence of competitive flow on pulmonary arterial growth, arteriovenous malformation development, and ventricular function warrants investigation.

Adolescent↗

Pulmonary artery banding is not contraindicated in double inlet left ventricle with transposition and aortic arch obstruction.

It has been widely stated that pulmonary artery banding (PAB) is contraindicated in the setting of double inlet left ventricle with transposition of the great vessels (DILV/TGA), especially if aortic arch obstruction is present. We postulated that the poor results for this condition reflect the tendency to leave the band in place long-term without early recognition and relief of subaortic stenosis (SAS). Short-term PAB with early relief of SAS remains an attractive option compared to a neonatal "Norwood" strategy. We reviewed our results applying this approach to 18 consecutive infants presenting since 1980 with DILV/TGA and an obstructive anomaly of the aortic arch (coarctation 16, interruption or atresia 2). Four of the infants (22%) were considered to have important SAS at presentation. One underwent neonatal aortopulmonary connection and died. The remaining 17 patients underwent arch repair with PAB (median age 1.4 weeks; range 2 days-22 weeks) with one early death. The 16 survivors have been followed for 5.6 +/- 3.7 years. All but one ultimately developed SAS. Relief of SAS was performed in 15 patients (median age 8 months) using a proximal aortopulmonary anastomosis. There were two early deaths, and one late death. Thirteen of the 18 patients (72%) are alive and well, and 12 have achieved Fontan repair or bidirectional superior cavopulmonary anastomosis (BCPA) with persistent relief of SAS. Most patients with DILV/TGA and aortic arch obstruction will tolerate temporary PAB with adequate protection of the pulmonary vascular bed. Our current approach (in the absence of severe SAS at presentation) is PAB at the time of arch repair, followed by careful surveillance for, and early relief of, SAS combined with BCPA in infancy.

Anastomosis, Surgical↗

Systolic and diastolic function in tricuspid valve atresia before the Fontan operation.

Systolic and diastolic function were assessed at preoperative cardiac catheterization in 6 patients with classic tricuspid atresia and were compared with those in control subjects. All patients had systemic-to-pulmonary shunts. Left ventricular end-systolic and end-diastolic volumes and masses were significantly larger in the patient group; stroke volume and the ratio of volume to mass were also higher, but not significantly. Mean end-diastolic, peak systolic and end-systolic pressures were similar. Mean stress at end-systole and peak systole were significantly higher in the patient group. Operant chamber stiffness tended to be lower than normal, but was not statistically significant, and myocardial stiffness was normal for the patient group also. Indexes of systolic function were compared. The rate-corrected mean velocity of fiber shortening in the patient group was lower and its relation with stress at end-systole was variable with 4 abnormal patients. The ratio of stress at end-systole/end-systolic volume was significantly lower in patients. The duration of hospital stay did not correlate with end-diastolic volume or any parameter. Thus, this study found abnormalities of load-dependent and independent indexes of systolic function with normal diastolic function in young preoperative patients with tricuspid atresia.

Cardiac Catheterization↗

Pulmonary wedge angiography for prediction of pulmonary vascular disease in Down syndrome.

We performed high resolution pulmonary wedge angiography (PWA) and conventional hemodynamics to predict the reversibility of structural pulmonary vascular disease. Sixty-one pulmonary wedge angiograms were performed on 41 patients with intracardiac shunts and Down syndrome (median age 8 months). Balloon occlusion wedge angiograms were analyzed for (1) monopedial branches from the distal 10 mm of muscular arteries, (2) capillary blush, (3) tapering indices, and (4) tortuosity. Twenty-five patients had open lung biopsy, graded by the Health Edwards classification, and analyzed morphometrically. Pulmonary vascular resistance of > or = 6 units was 100% sensitive and 94% specific for Heath Edwards Grade III-IV. A monopedial count < 3 vessels was 83% sensitive and 100% specific for Heath Edwards Grade III-IV. Abnormal capillary blush was 83% sensitive and 69% specific for Heath Edwards Grade III-IV. Tapering indices and tortuosity showed no significant correlation with lung biopsy. A combination of pulmonary vascular resistance < 6 units, monopedial count > or = 3, and normal capillary blush was 100% sensitive and specific for Heath Edwards Grade 0-II, and a combination of pulmonary vascular resistance > or = 6 units, monopedial count < 3, and abnormal capillary blush was 100% sensitive and specific for Heath Edwards Grade III-IV. Using the 3 criteria, Heath Edwards Grade was accurately predicted in 17 patients. In 4 patients, only 2 criteria were available. Morphometric analysis showed an inverse relationship between the lowest monopedial count and the number of occlusive vessels per cm of tissue, r = -0.74 p < 0.001. Arteries showing intimal and/or medial thickening causing > 90% luminal narrowing were scored as "occlusive." These results show that when the hemodynamic and pulmonary wedge angiography data are concordant, the structural changes of pulmonary vascular disease can be accurately predicted and lung biopsy may be avoided.

Angiography↗

Immunogenicity of Haemophilus influenzae type b conjugate vaccine in children with congenital asplenia.

The immunogenicity of Haemophilus influenzae type b polysaccharide-protein conjugate vaccines in congenitally asplenic children is unknown. The short-term immunogenicity of the H. influenzae type b polysaccharide-diphtheria toxoid conjugate vaccine was therefore assessed in 10 children with congenital asplenia by measuring antipolyribosyl-ribitol phosphate antibody titers. An excellent antibody response was seen in 9 children (mean geometric titer in responders after immunization 44.7 micrograms/mL; range, 2.59-402). The remaining child responded to a booster dose. Further studies are required to assess whether H. influenzae type b conjugate vaccines are immunogenic in infancy in the presence of congenital asplenia.

Adolescent↗

Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum.

BACKGROUND: Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. OBSERVATIONS: Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a close ductus arteriosus within the first two hours of birth while on prostaglandin E1 infusion. CONCLUSIONS: In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.

Female↗

Rate-corrected mean velocity of fiber shortening-stress at peak systole as a load-independent measure of contractility.

The relation of mean velocity of fiber shortening-stress at end-systole is a good load-independent index of left ventricular contractility, but involves simultaneous M-mode echocardiography, carotid tracing and blood pressure determination, which may be impractical in children. As stress at peak systole and end-systole are closely related, this study examined whether the relation of mean velocity of fiber shortening-stress at peak systole could be used as a simpler method of assessing left ventricular contractility in a normal population of children. In addition, the cross-sectional normal range for a pediatric population was obtained. In 25 normal children aged 4 to 17 years (mean age 11), rate-corrected mean velocity of fiber shortening (MVCFc), stress at peak systole (sigma PS), and end-systolic stress (sigma ES) were determined using echocardiography, carotid pulse tracing and blood pressure measurement. Six patients with cardiomyopathy (mean age 8 years) were also studied. The relation of stress at peak systole and end-systolic stress was sigma PS = 1.004 sigma ES + 12.0 (r = 0.91, SEE = 4.98; p less than 0.001) for the normal group, and sigma PS = 1.083 sigma ES + 7.7 (r = 0.99, SEE = 4.07; p less than .001) for the cardiomyopathic group. This slope of the regression line was slightly higher and the difference was statistically significant. The slope of MVCFc = 0.0066 sigma PS + 1.55 (SEE = 0.131, r = -0.52) was nearly identical to that of MVCFc = 0.0065 sigma ES + 1.46, (SEE = 0.135, r = -0.46), with no difference in the regression coefficients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Doppler echocardiographic observations of diastolic trans-septal flow through small ventricular septal defects.

Turbulent left-to-right trans-septal diastolic blood flow with a peak velocity greater than 0.5 m.s-1 was detected by pulsed Doppler echocardiography in 10 out of 204 (4.9%) children with isolated small ventricular septal defects. For children older than 10 years, it was observed in over a quarter of cases (27%). Peak diastolic velocities ranged from 0.6-1.9 m.s-1 (mean 1.2 m.s-1) and in all patients the duration of diastolic flow exceeded that of the systolic trans-septal jet. Three different patterns of diastolic flow were observed and in eight cases pre-systolic acceleration was present. Turbulent diastolic flow across small ventricular septal defects is a common finding in older children and should not be confused with other causes of disturbed diastolic flow within the right ventricle.

Adolescent↗

Early prediction of anthracycline cardiomyopathy using standard M-mode and digitized echocardiography.

In this study, we attempted to identify echocardiographic findings that can be used as early predictors of left ventricular dysfunction due to anthracyclines in 26 pediatric oncology patients. Retrospective review identified six patients (group 1 mean anthracycline dose = 337 +/- 123 mg/m2) who developed clinical evidence of cardiomyopathy and 20 consecutive patients followed prospectively (group 2 mean anthracycline dose = 298 +/- 102 mg/m2) who did not. All of the patients had serial m-mode echocardiography before and at approximately every 50 mg/m2 of anthracycline treatment depending on the dosage schedule. The following measurements were recorded: left ventricular dimensions and wall thickness, indices of left ventricular function, including systolic time intervals, shortening fraction, and ejection fraction. Using digitized echocardiography, the following parameters were recorded: peak and normalized systolic and diastolic velocities of the left ventricular chamber, posterior and septal wall velocities, and percent of posterior wall thickening. Variations from established normal longitudinal bounds and actual changes from pre-anthracycline values were compared for the two groups throughout the course of therapy. At 70 mg/m2 of anthracycline, group 1 showed significant deterioration in shortening fraction (mean change = 8.8 versus 2.5, p = .03), left ventricular velocity in diastole (2.9 versus 0.21, p = .04), left ventricular normalized velocity in systole (.65 versus 0.03, p = .02), and left ventricular normalized velocity in diastole (1.15 versus 0.002, p = .04) compared to group 2. These values were mostly compensated by 125 mg/m2, but they slowly deteriorated again at higher doses of anthracycline, leading to the appearance of clinical evidence of cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗