A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 ... more A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. Thirteen children without chromosomal aberrations were then selected as a control group, matched 1 to 1 for gender, age, weight, lesion, and type of Fontan procedure. The following variables were evaluated: pulmonary artery pressure and vascular resistance, weight, hemoglobin, degree of atrioventricular regurgitation, previous Glenn operation, fenestration, and length of stay in the hospital. In children with Down syndrome, mortality after the Glenn operation was 28%. Mortality after the Fontan operation was increased significantly (p = 0.001) in children with Down syndrome (35%) compared with those without Down syndrome (10%). Between patients with Down syndrome and controls, there were no significant differences in the perioperative parameters evaluated. Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery.
ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease ... more ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10–15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre. Our hypothesis was that patients with pulmonic valve autograft would have better survival than mechanical prosthetic.MethodsThis is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional US-based registry of paediatric cardiac interventions, linked with the National Death Index and United Network for Organ Sharing through 2019. Children (0–20 years old) receiving aortic valve replacement (AVR) from 1982 to 2003 were identified. Kaplan-Meier transplant-free survival was calculated, and Cox proportional hazard models estimated hazard ratios for mechanical AVR (M-...
Abstract Fifteen patients with ventricular septal defect and previous pulmonary artery banding un... more Abstract Fifteen patients with ventricular septal defect and previous pulmonary artery banding underwent complete cardiac catheterization including angiocardiography. Distance between band and pulmonary valve varied from immediate adjacency to 22 mm. In all cases in which a tight band had been applied in close proximity to the pulmonary valve, there appeared to be pulmonary doming and a distinct thickening of one or all pulmonary valve leaflets. These data suggest that placing the band as distally as possible to the pulmonary valve may minimize trauma to the leaflet which may result in fibrotic thickening and residual pressure gradient following band removal.
Journal of Cardiovascular Translational Research, Mar 12, 2009
Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations,... more Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.
A clinical and pathologic study of 47 cases with endocardial fibroelastosis is presented. These c... more A clinical and pathologic study of 47 cases with endocardial fibroelastosis is presented. These cases have been classified according to the associated cardiac anomaly and the anatomic condition of the left ventricle. In so doing, a better understanding is had of the role the cardiac anomaly associated with endocardial fibroelastosis plays in the production of cardiac signs and symptoms. In each of the specimens available for review, a change of the mitral valve was present that rendered it insufficient. Those cases grouped as primary endocardial fibro-elastosis had this as the only abnormality present, other than the endocardial fibroelastosis. Many of the clinical and laboratory findings in this group could be explained on the basis of mitral insufficiency, and one cannot necessarily assign the cardiac signs and symptoms to the endocardial process itself. In those cases with associated cardiac anomalies the hemodynamic consequences appear to be a summation of the combined effects o...
This article describes the first patient treated surgically for cor triatriatum, and also describ... more This article describes the first patient treated surgically for cor triatriatum, and also describes preoperative testing, operative findings, and procedures, as well as follow-up.
A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 ... more A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. Thirteen children without chromosomal aberrations were then selected as a control group, matched 1 to 1 for gender, age, weight, lesion, and type of Fontan procedure. The following variables were evaluated: pulmonary artery pressure and vascular resistance, weight, hemoglobin, degree of atrioventricular regurgitation, previous Glenn operation, fenestration, and length of stay in the hospital. In children with Down syndrome, mortality after the Glenn operation was 28%. Mortality after the Fontan operation was increased significantly (p = 0.001) in children with Down syndrome (35%) compared with those without Down syndrome (10%). Between patients with Down syndrome and controls, there were no significant differences in the perioperative parameters evaluated. Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery.
ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease ... more ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10–15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre. Our hypothesis was that patients with pulmonic valve autograft would have better survival than mechanical prosthetic.MethodsThis is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional US-based registry of paediatric cardiac interventions, linked with the National Death Index and United Network for Organ Sharing through 2019. Children (0–20 years old) receiving aortic valve replacement (AVR) from 1982 to 2003 were identified. Kaplan-Meier transplant-free survival was calculated, and Cox proportional hazard models estimated hazard ratios for mechanical AVR (M-...
Abstract Fifteen patients with ventricular septal defect and previous pulmonary artery banding un... more Abstract Fifteen patients with ventricular septal defect and previous pulmonary artery banding underwent complete cardiac catheterization including angiocardiography. Distance between band and pulmonary valve varied from immediate adjacency to 22 mm. In all cases in which a tight band had been applied in close proximity to the pulmonary valve, there appeared to be pulmonary doming and a distinct thickening of one or all pulmonary valve leaflets. These data suggest that placing the band as distally as possible to the pulmonary valve may minimize trauma to the leaflet which may result in fibrotic thickening and residual pressure gradient following band removal.
Journal of Cardiovascular Translational Research, Mar 12, 2009
Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations,... more Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.
A clinical and pathologic study of 47 cases with endocardial fibroelastosis is presented. These c... more A clinical and pathologic study of 47 cases with endocardial fibroelastosis is presented. These cases have been classified according to the associated cardiac anomaly and the anatomic condition of the left ventricle. In so doing, a better understanding is had of the role the cardiac anomaly associated with endocardial fibroelastosis plays in the production of cardiac signs and symptoms. In each of the specimens available for review, a change of the mitral valve was present that rendered it insufficient. Those cases grouped as primary endocardial fibro-elastosis had this as the only abnormality present, other than the endocardial fibroelastosis. Many of the clinical and laboratory findings in this group could be explained on the basis of mitral insufficiency, and one cannot necessarily assign the cardiac signs and symptoms to the endocardial process itself. In those cases with associated cardiac anomalies the hemodynamic consequences appear to be a summation of the combined effects o...
This article describes the first patient treated surgically for cor triatriatum, and also describ... more This article describes the first patient treated surgically for cor triatriatum, and also describes preoperative testing, operative findings, and procedures, as well as follow-up.
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Papers by james moller