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Biomedical subjects

D C Fyler

Publications and source records attributed to D C Fyler.

At least 19 recordsLinked to original sources
PubMed1994

Examination of the early 'learning curve' for transcatheter closure of patent ductus arteriosus using the Rashkind occluder. PDA Closure Comparative Study Group.

BACKGROUND: Results of transcatheter implantation of the Rashkind double umbrella occluder for treatment of patent ductus arteriosus (PDA) have improved over time. We evaluated factors associated with changes in outcomes seen in the early clinical experience with this device. METHODS AND RESULTS: We reviewed records of otherwise healthy pediatric patients undergoing occluder implantation for isolated PDA between 1982 and 1987 at six major US and Canadian referral centers. Logistic regression was used to assess the impact of ductal size, patient age and weight, calendar time, prior intrainstitutional occluder experience, and residual center effects on closure rates. There were no study deaths. Of 180 occluder placement attempts, 76.7% (95% confidence limits = 70.5%, 83.0%) produced PDA closure by auscultation at 14-month cardiology follow-up or its equivalent. Success probabilities increased in a linear fashion as ductus size decreased, ranging from 41.7% for large (4.1 to 9.0-mm) ducts to 89.4% for small (1.5 to 2.5-mm) lesions. PDA occluder closure rates also improved over time, increasing from 47.4% in 1982-1983 to 84.6% in 1986 and 83.1% in 1987. Multivariate logistic regression indicated that improved outcomes were mainly attributable to decrease in the size of treated ducts over time. Independent of this patient selection, improvements in outcome were less strongly associated with cumulative intracenter experience, calendar time, and age. CONCLUSIONS: Increasing closure rates seen over time primarily reflected more judicious selection of patients as experience accumulated. However, other factors also contributed to the observed trends. An analysis of current treatment results would be of considerable interest.

Age Factors↗
PubMed1993

Clinical outcomes and costs of transcatheter as compared with surgical closure of patent ductus arteriosus. The Patient Ductus Arteriosus Closure Comparative Study Group.

BACKGROUND: Transcatheter implantation of the Rashkind PDA occluder is an alternative to conventional surgical closure of isolated patent ductus arteriosus. Neither the clinical outcomes nor the costs of these procedures have been formally compared. METHODS: We performed a retrospective cohort study to evaluate the clinical outcomes within a seven-month period for comparable patients with patent ductus arteriosus who underwent either placement of an occluder or surgical closure. The patients were treated between 1982 and 1987 at 14 major North American centers where patent ductus arteriosus was closed predominantly by a surgical procedure or by the occluder technique. To estimate inpatient and follow-up costs, we multiplied the observed use of resources by 1989 unit costs based on hospital-accounting and physician-reimbursement data. RESULTS: On the basis of cardiac auscultation at follow-up, the initial procedure resulted in closure of the ductus arteriosus in 77.3 percent of 185 patients in whom the occluder was implanted (95 percent confidence interval, 70.6 to 83.1 percent) and 99.8 percent of 446 surgical patients (95 percent confidence interval, 98.8 to 100.0 percent). Second procedures increased the percentage of successful closures to 87.6 percent (95 percent confidence interval, 81.9 to 92.0 percent) and 100.0 percent (95 percent confidence interval, 99.3 to 100.0 percent) for patients in the occluder and surgical groups, respectively. There were no deaths. Major complications occurred in 2.7 percent of the patients in whom the occluder was implanted (95 percent confidence interval, 0.9 to 6.2 percent) and 0.2 percent of the patients who underwent surgery (95 percent confidence interval, 0.0 to 1.2 percent); moderate complications in 16.8 percent (95 percent confidence interval, 11.7 to 22.9 percent) and 15.0 percent (95 percent confidence interval, 11.8 to 18.7 percent), respectively; and minor complications in 11.4 percent (95 percent confidence interval, 7.2 to 16.8 percent) and 24.9 percent (95 percent confidence interval, 20.9 to 29.2 percent). Including the cost of follow-up care, the mean estimated cost per case treated surgically was $8,838 (in 1989 U.S. dollars), as compared with $11,466 per case treated with the occluder technique. Sensitivity analyses based on our data identified no plausible situations in which the costs of surgery and of implantation of the occluder would be equal. CONCLUSIONS: The more effective and less costly surgical procedure was superior to transcatheter placement of the occluder for closure of isolated patent ductus arteriosus. Consequently, our results do not support the wide-spread dissemination of the occluder procedure for the management of this common congenital lesion.

Cardiac Catheterization↗
PubMed1990

Psychological functioning in children with cyanotic heart defects.

The relationship between congenital heart disease (CHD) and psychological functioning was studied in 63 children with transposition of the great arteries (TGA) and 77 children with tetralogy of Fallot (TF). These youngsters were compared with a group of 36 children originally diagnosed with CHD, but who spontaneously recovered (SR) without medical intervention. All children were under one year of age when diagnosed and were given psychological testing between 5.5 and 6.3 years. Children with TGA and TF had poorer overall psychological functioning and significantly greater central nervous system (CNS) impairment than the SR children. However when the effects of IQ and CNS impairment were controlled, there were no differences between groups. The diagnosis of a severe cyanotic heart defect does not appear to make a child more likely to have emotional disorder in the absence of other factors.

Activities of Daily Living↗
PubMed1988

Anterior chest wall deformities and congenital heart disease.

Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.

Adolescent↗
PubMed1985

Complications and mortality associated with cardiac catheterization in infants under one year: a prospective study.

To evaluate complications and mortality following cardiac catheterization (CC) in patients under one year of age, we prospectively examined 312 CC on 273 patients from the centers in the New England Regional Infant Cardiac Program during a one-year period. Among CC performed on patients less than four months old, the incidence of complications requiring treatment was 12%; for patients 4-12 months, it was 1.5% (p less than 0.02). By pre-CC risk assessment, 13% of CC were high risk, 21% medium risk, and 66% low risk. The incidence of major complications was much greater among the High Risk (30%) compared with Medium Risk (14%) (p less than 0.05) and with Low Risk (4%) (p less than 0.001). The overall mortality rates 24 h, 48 h, and one week after CC were 3.8%, 8.3%, and 13.5%, including patients with inoperable lesions and those having cardiac surgery, while deaths directly attributable to a CC complication were 0, 0.3%, and 0.3%, respectively. We can predict, prior to CC, the high-risk cases where major complications and death are likeliest to occur. The incidence of death from CC-related complications is extremely low in patients under one year of age, compared with death from the underlying lesion or following cardiac surgery.

Age Factors↗
PubMed1985

Effect of vasodilators at rest and during exercise in young adults with cystic fibrosis and chronic cor pulmonale.

Six clinically stable patients with cystic fibrosis (24 to 31 yr of age) and severe pulmonary impairment, right ventricular hypertrophy, and previous right-sided heart failure underwent cardiac catheterization to assess the hemodynamic effects of oxygen (fraction of inspired O2, 0.31, 0.50), phentolamine (5 mg intravenously), hydralazine (0.33 mg/kg intravenously), and nifedipine (20 mg sublingually). Measurements during dynamic exercise were also obtained before and after hydralazine therapy. Studies after 5 to 8 wk of continuous, orally administered hydralazine therapy were performed in 3 patients. The resting mean pulmonary artery pressure was 31 +/- 4 mmHg. At rest, only oxygen was a selective pulmonary vasodilator, decreasing pulmonary artery pressure and pulmonary vascular resistance in all patients. Systemic arterial pressure and resistance were not significantly changed. Phentolamine, hydralazine, and nifedipine did not alter pulmonary artery pressure or selectively affect the pulmonary vascular bed, reducing both calculated pulmonary and systemic vascular resistance, the latter to a similar or greater degree. Hydralazine and nifedipine significantly increased cardiac index and decreased systemic arterial pressure. Nifedipine mildly decreased systemic oxygenation. During exercise, the mean pulmonary artery pressure increased to 51 +/- 15 mmHg. Hydralazine increased systemic and mixed venous oxygenation both at rest and during exercise but did not alter the elevation in pulmonary artery pressure observed during exercise. After orally administered hydralazine therapy, oxygen delivery and cardiac index remained increased in 2 patients. These data support the use of oxygen but not of the other agents in patients with cystic fibrosis and chronic cor pulmonale unless the ability of hydralazine to increase oxygen delivery is determined to improve prognosis.

Adult↗
PubMed1984

Cognitive function and age at repair of transposition of the great arteries in children.

To assess the effect of the duration of chronic hypoxemia on cognitive function, we studied 38 children with d-transposition of the great arteries and an intact ventricular septum who underwent corrective surgery at six months to six years of age (median, 1.6 years). Tests included the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) in 33 children and the visual-association and auditory-association subtests of the Illinois Test of Psycholinguistic Abilities in 38 and 37 children, respectively. When controlled for social index, age at repair was inversely associated with the WPPSI intelligence-quotient score (P less than 0.01), the visual-association subtest score (P less than 0.01), and the auditory-association subtest score (P less than 0.1). In contrast, age at repair correlated poorly with cognitive function in children with ventricular septal defect, an acyanotic congenital heart defect. These data suggest that postponing repair of a cyanotic congenital heart disease, such as transposition of the great arteries, is associated with progressive impairment of cognitive function.

Age Factors↗
PubMed1984

Congenital pulmonary vein stenosis: structural changes in a patient with normal pulmonary artery wedge pressure.

A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and veins in all lobes with or without pulmonary vein stenosis. Arterial changes-muscle extension, medial hypertrophy and decreased arterial size--analyzed quantitatively were found to be similar in all lobes. Venous medial hypertrophy was more marked in obstructed lobes. These anatomic changes are presumably due to fixed venous obstruction in the pulmonary lobes drained by stenotic veins and to high flow in the left upper lobe.

Cardiac Catheterization↗
PubMed1983

Origin of pulmonary artery branch from ascending aorta. Primary surgical repair in infancy.

1. Surgical repair of origin of a pulmonary artery branch from the ascending aorta should be performed as early as possible in order to prevent death from congestive heart failure or the development of irreversible pulmonary vascular obstructive disease. 2. Deep hypothermic circulatory arrest greatly facilitates surgical repair of this lesion in infancy, as is illustrated by the three cases reported herein. 3. Direct anastomosis of the ectopic pulmonary artery branch to the main pulmonary artery is the surgical technique of choice. 4. Origin of the RPA or the LPA from the ascending aorta results from origin of the RPA or LPA from the aortic sac, instead of from the confluent sixth arches. Typically, the RPA has failed to migrate leftward because of abnormal development of the wall of the aortic sac, abnormal development of the sixth arches, or both. 5. Origin of the RPA or the LPA from the ascending aorta should be distinguished from origin of the "RPA" or of the "LPA" from the innominate artery or from the aortic arch via a PDA or a collateral artery.

Cardiac Catheterization↗
PubMed1982

Radionuclide angiocardiographic assessment of pulmonary vascular reactivity in patients with left to right shunt and pulmonary hypertension.

Radionuclide angiocardiography was used to assess pulmonary vascular reactivity in eight patients (nine studies) with a large, relatively unrestrictive intracardiac defect and pulmonary arterial hypertension. Radionuclide angiocardiograms, using technetium-99m pertechnetate, were performed first with the patient breathing room air and then after 10 minutes of breathing a mixture containing 90 percent or more of oxygen. The pulmonary to systemic flow ratios obtained by gamma variate analysis of the radionuclide time-activity curves were compared with those calculated with the Fick principle at the time of cardiac catheterization. There was a good correlation between the two methods both in room air studies (r = 0.88) and in those obtained with 90 percent or more of oxygen (r = 0.94). All six studies (in five patients) with a reactive pulmonary vasculature (judged by a pulmonary vascular resistance at cardiac catheterization of less than 6 units/m2 with oxygen or after tolazoline) had a radionuclide pulmonary to systemic flow ratio of 3.0 or greater with oxygen. The three patients with a nonreactive pulmonary vasculature had a radionuclide pulmonary to systemic flow ratio of 2.3 or less with oxygen, a value that was unchanged from the room air value. These data suggest that radionuclide angiocardiography may be a useful, relatively noninvasive method of assessing pulmonary vascular reactivity in patients with a large, relatively unrestrictive intracardiac defect.

Angiocardiography↗
PubMed1982

Pediatric arterial catheterization using a 3.2 French catheter.

Retrograde arterial catheterization was performed in 105 infants and small children (median age 6 mo) using a 3.2 French pigtail catheter made of ultrathin walled white Teflon. Three catheter lengths were used, (40, 50, and 65 cm). Pressure recordings and ease of sampling were judged excellent in all. All 118 angiograms (60 ventricular) were performed without complication except for two episodes of external catheter rupture. Angiographic quality was rated excellent in 41%, good in 39%, fair in 18%, and poor in only 2%. Among the 81 patients in whom only this catheter was used percutaneously, pedal pulses were lost in 5%. Postcatheterization testing identified ranges of volume and flow-rate characteristics which included 8 ml at 15 ml/sec (40 cm length), at 11 ml/sec (50 cm length), and at 9 ml/sec (65 cm catheter). We conclude that this small catheter is relatively safe and satisfactory for retrograde catheterization of infants and small children.

Cardiac Catheterization↗
PubMed1982

Interruption of the aortic arch with aorticopulmonary septal defect. An anatomic review.

Interruption of the aortic arch may occur with aorticopulmonary septal defect (APSD) as part of a developmental complex. The anatomic details of 46 cases of interruption of the aortic arch with APSD revealed the following characteristics: (1) type A interruption of the aortic arch occurred nearly six times more commonly than type B interruption; (2) the APSD was variable in size and position, conforming to the types previously described; (3) the ventricular septum was usually intact, but a ventricular septal defect was present in six cases and occurred more frequently with type B interruption of the aortic arch; (4) subaortic stenosis was not present in any of the 16 cases in which adequate details were available to make a judgment. The association of APSD with interruption of the aortic arch in infancy is not uncommon. Evaluation of infants with APSD should thus include evaluation of the aortic arch. The occurrence of interruption of the aortic arch with an intact ventricular septum is distinctly unusual. In such cases an APSD should be suspected and sought.

Aorta↗
PubMed1982

Marital stability and congenital heart disease.

The incidence of divorce or legal separation was studied in 438 families of children born with heart disease who entered the New England Regional Infant Cardiac Program between 1968 and 1973. The parents were interviewed when the children were 5 1/2 years old. The rate of divorce in 438 families of children with critical congenital heart disease was not significantly different from the rate in two comparison groups: (1) 25 families of children whose cardiac defect was spontaneously cured, and (2) 26 families of children catheterized in infancy for suspected cardiac defect but who were found to be free of heart disease. Rates of divorce or legal separation for the three groups were: critical congenital heart disease, 12.1%, spontaneously cured, 4.2% free of heart disease, 11.5% these rates were not significantly different. The average national divorce rate was 20.3% for the same period.

Adult↗
PubMed1979

Exogenous hormones and other drug exposures of children with congenital heart disease.

A history of oral contraceptive use, hormonal pregnancy tests, prescribed hormones and other drugs was obtained from 390 mothers of infants with congenital heart disease and 1254 mothers of normal infants in Massachusetts. The data show a small positive association between estrogen/progesterone exposure and cardiac malformation, the prevalence ratio estimate of exposed to non-exposed being 1.5 (90 per cent confidence limits are 1.0, 2.1). No association was evident, however, between hormones and trunco-conal or any other class of defect among the cases, an observation which casts doubt on a causal relationship betweem hormones and cardiovascular malformations. Several other drugs were reported more frequently by cases' mothers. These include: ampicillin; aspirin; a combined anti-nausea agent (doxylamine succinate, dicyclomine hydrochloride and pyridoxine hydrochloride); chlordiazopoxide, codeine, diazepam, diphenylhydantoin; insulin; phenobarbital; phenothiazine; phenylephrine; and tetracycline.

Estrogens↗
PubMed1978

Birthweight of infants with congenital heart disease.

A subnormal birth weight distribution was noted in a population of infants seen during the first year of life for major congenital heart disease (CHD). When arranged according to cardiac diagnostic categories, 17 of the 21 major cardiac lesions were associated with subnormal birth weight distribution. Major extracardiac anomalies present in 19.9% of all infants appeared not to influence the birth weight distributions of either the total population of 19 of 21 cardiac lesion categories. An increased incidence (6.1%) of small-for-dates infants was observed in the study population. Extra-cardiac anomalies occurred in 28% of the small-for-dates infants. Survival to 1 year of age was 42.8% for the total population with CHD and 49.2% in the small-for-dates group.

Abnormalities, Multiple↗