Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology.
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Adolescent↗
Biomedical subjects
Kimberlee Gauvreau
Publications and source records attributed to Kimberlee Gauvreau.
Clinical outcomes and utility of cardiac catheterization prior to superior cavopulmonary anastomosis.
OBJECTIVES: We sought to characterize the outcomes of routine catheterization prior to superior cavopulmonary anastomosis and to determine if some patients were unlikely to benefit from catheterization and thus might be evaluated preoperatively with noninvasive methods alone. BACKGROUND: Congenital heart disease patients with single ventricle physiology undergo routine echocardiography and cardiac catheterization prior to superior cavopulmonary anastomosis to determine anatomic and hemodynamic suitability for this procedure. METHODS: We performed a retrospective review of all infants (n = 114) evaluated for potential superior cavopulmonary anastomosis at our institution from January 1997 to June 2000. RESULTS: Patients' median age was 5.5 months. Full echocardiograms were obtained in 79 patients (69%). At catheterization a total of 41 interventions were performed in 35 patients (31%). Twenty-seven patients (24%) were transfused, 18 patients (17%) required cardiac intensive care unit admission, and median length of stay following catheterization was 1 day (range 0 to 22). Complications occurred in 28 patients (25%), most transient. Of 51 patients who had complete echocardiograms without indication for catheterization, none subsequently had significant interventions and only 2 had new findings at catheterization. Three candidates were excluded from operation; all 111 others underwent successful procedures and survived to hospital discharge. CONCLUSIONS: Interventions were frequent at catheterization prior to superior cavopulmonary anastomosis, but transient complications, transfusion, intensive care unit admission, and prolonged hospital length of stay were common. For patients in whom no issues indicating need for catheterization are identified by echocardiogram, routine catheterization rarely results in new information or intervention. These patients may be more safely evaluated preoperatively using exclusively noninvasive techniques.
Adolescent↗
Physical and psychosocial health in children who have had Kawasaki disease.
OBJECTIVE: The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions. METHODS: The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 mm (mild-moderate aneurysm group), and with giant aneurysms >or=8 mm (giant group). RESULTS: Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1-17.9 years) and at illness onset was 3.1 years (0.2-12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample. CONCLUSIONS: KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children's health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores.
Adaptation, Psychological↗
Evidence-based referral results in significantly reduced mortality after congenital heart surgery.
OBJECTIVE: Significant interinstitutional variation in mortality after congenital heart surgery has been demonstrated. Noting an association between reduced mortality and higher volume, a center with a small annual case volume began in August 1998 to selectively refer to high-volume surgical centers based on published or "apparent" low mortality rates for specific cardiac lesions. This study was undertaken to evaluate the effect of evidence-based referral in this practice. DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort comparison over a 10-year period for a small Midwestern pediatric cardiology practice. The institutional database was retrospectively reviewed for children (<18 years) undergoing surgery from August 1992 to July 2002. Data were divided into 3 time periods (August 1992 to July 1995, period 1; August 1995 to July 1998, period 2; and August 1998 to July 2002, period 3). Hospital discharge abstract data from 5 states (California, Illinois, Massachusetts, Pennsylvania, and Washington) in 1992, 1996, and 1998 provided contemporaneous benchmarks. Risk adjustment was performed using the Risk Adjustment in Congenital Heart Surgery-1 method. Risk category, age at surgery, prematurity, and major noncardiac structural anomaly were entered into a multivariate logistic regression model to compare in-hospital mortality adjusting for case-mix differences. RESULTS: A total of 514 congenital heart surgical cases were identified from August 1992 to July 2002; 507 cases (98.6%) were assigned to a risk category and analyzed further. Unadjusted in-hospital mortality rates were 9.3% in period 1, 5.9% in period 2, and 1.3% in period 3. Unadjusted mortality rates for cases from benchmark data were 6.4% in 1992, 4.8% in 1996, and 3.7% in 1998. Risk adjusted mortality was comparable to the benchmark data in periods 1 and 2, but superior outcomes (odds ratio = 0.24) were demonstrated in period 3. CONCLUSIONS: Evidence-based referrals from a small-volume pediatric cardiac center to large-volume institutions resulted in a reduction in mortality after congenital heart surgery.
Adolescent↗
Neurologic outcome of choreoathetoid encephalopathy after cardiac surgery.
Postoperative encephalopathy with choreoathetosis is a serious neurologic complication of childhood cardiac surgery. An inadequate understanding of the long-term outcome has limited family counseling and the institution of intervention strategies. Our objective was to define the long-term neurologic, cognitive, and behavioral outcome in this group of patients. By retrospective review we identified all cases of choreoathetosis in our cardiac intensive care unit from 1986 through 1995. During this decade we described a sudden transient increase in postoperative choreoathetosis that disappeared as we modified treatment strategies in perioperative care. These children underwent a comprehensive battery of neurologic, cognitive, and behavioral tests to determine in detail their long-term outcome. Of the 36 cases identified of which three were deceased, four were abroad, and eight were lost to follow-up, 21 families were approached, and 15 of the 21 (71%) consented to testing. We found pervasive deficits in memory, attention, and language, with a median full-scale IQ of 67 (range = 40-122), as well as in motor function, including persistent dyskinesia in seven of 15 (47%). The pervasive and enduring cognitive and motor deficits in survivors of postoperative choreoathetosis caution for guarded predictions of outcome and highlight the need for careful neurologic and cognitive evaluation in all children with choreoathetosis after open heart surgery.
Athetosis↗
Gastrointestinal complications after pediatric cardiac transplantation.
BACKGROUND: The incidence of major gastrointestinal complications after pediatric heart transplantation has not been well characterized. Studies in adults suggest significant morbidity and mortality from post-transplant gastrointestinal complications. In this study, we investigated major gastrointestinal complications in the pediatric heart transplant population. METHODS: We performed a retrospective analysis of all patients who underwent heart transplantation at Children's Hospital, Boston, including all pertinent clinical, radiologic, endoscopic, and pathologic findings. Between May 1986 and December 2000, 104 patients underwent 105 orthotopic heart transplantations. Gastrointestinal complications were defined as major if they significantly prolonged hospital course, required hospital admission, or required surgical intervention. RESULTS: Median age at transplant was 8.7 years (range, 2 weeks to 23 years). Median duration of follow-up was 3.3 years (range, 2 days to 14.9 years). All patients initially received standard triple immunosuppression with cyclosporine, prednisone, and azathioprine. During this period, 30 major complication episodes occurred in 19 patients (18%) and included pancreatitis (7), cholecystitis (6), recurrent abdominal infection (5), malignancy (4), intestinal pneumatosis (4), colonic perforation (2), appendicitis (1), Crohn's disease (1), and partial small bowel obstruction (1). Ten (53%) of the 19 patients with major gastrointestinal complications required surgical intervention. CONCLUSIONS: Serious gastrointestinal complications can occur after pediatric cardiac transplantation, with an incidence similar to that seen in adults. Gastrointestinal symptoms should be aggressively evaluated in the pediatric heart transplant patient because of the high incidence of complications that may require surgery.
Adolescent↗
Outcome of ventricular septal defect repair in a developing country.
OBJECTIVES: To examine the impact of nutrition and lung infection on outcome early after ventricular septal defect (VSD) repair in infants in a developing country. STUDY DESIGN: One hundred consecutive infants (age, 7.4 +/- 3.3 months) with large VSD(s) who underwent surgical repair at one institution in South India from July 1998 to June 2000 were analyzed. Primary outcome variables were postoperative death, duration of mechanical ventilation, intensive care unit (ICU) stay, and hospital stay. Preoperative variables analyzed included age, weight and length Z scores, and lung infection. RESULTS: Preoperative nutrition was poor (weight Z score, -2.8 +/- 1.3), and 25 patients had pneumonia. Six patients died after repair. No preoperative variable was associated with death. Mechanical ventilation, ICU stay, and hospital stay were longer for younger patients (r (s) for ventilation, -0.23, P =.02; for ICU stay, -0.33, P <.001; for hospital stay, -0.27, P =.007) and for those with preoperative pneumonia (median ventilation duration, 46 vs 24 hours, P <.001; median ICU stay 7 vs 4 days, P <.001; median hospital stay 10 vs 7 days, P =.001). Preoperative weight and length Z scores were not associated with any outcome variable. CONCLUSIONS: Poor nutritional status, preoperative pneumonia, and age do not increase mortality rates after VSD repair. Repair of large VSDs should not be delayed because of these preoperative characteristics.
Female↗
Consensus-based method for risk adjustment for surgery for congenital heart disease.
OBJECTIVE: The aim was to develop a consensus-based method of risk adjustment for in-hospital mortality among children younger than 18 years after surgery for congenital heart disease (designated RACHS-1). METHODS: An 11-member national panel of pediatric cardiologists and cardiac surgeons used clinical judgment to place surgical procedures into six risk categories. Categories were refined after review of information from the Pediatric Cardiac Care Consortium and three statewide hospital discharge data sets. The effects of including additional clinical variables were explored by comparing areas under receiver-operator characteristic curves. RESULTS: Among 4602 surgical patients in the Pediatric Cardiac Care Consortium data set and 4493 in the hospital discharge data, 3767 (81.9%) and 3832 (85.3%), respectively, had a single cardiac procedure, and 98.5% and 89.2%, respectively, were able to be assigned to one of six risk categories defined by the panel. Mortality rates showed expected trends (P <.001). For the Pediatric Cardiac Care Consortium data, mortality rates were 0.4% in category 1, 3.8% in 2, 8.5% in 3, 19.4% in 4, and 47.7% in 6; rates were similar in the hospital discharge data. There were too few cases in category 5 to estimate mortality rates. In multivariable models, younger age, prematurity, and the presence of a major noncardiac structural anomaly added to the risk of in-hospital death predicted by risk category alone. Best performance was obtained when cases with multiple procedures were placed in the risk category of the most complex procedure. CONCLUSION: The RACHS-1 method should adjust for baseline risk differences and allow meaningful comparisons of in-hospital mortality for groups of children undergoing surgery for congenital heart disease.
Cardiac Surgical Procedures↗
Center-specific differences in mortality: preliminary analyses using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method.
OBJECTIVE: We sought to explore the usefulness of the Risk Adjustment in Congenital Heart Surgery method (designated RACHS-1) of adjusting for case-mix differences when comparing institutional mortality after surgery for congenital heart disease. METHODS: By using 1996 hospital discharge data from 6 states, centers performing at least 100 operations for congenital heart disease (patient age <18 years) were identified. Using the RACHS-1 method, procedures were grouped into 6 risk categories, and institutions were ranked in order of increasing mortality rate. A graphic display of ranks by risk category identified patterns of performance. Incorporating age, prematurity, and presence of a major noncardiac structural anomaly into multivariate models allowed computation of an overall risk-adjusted rank for each institution on the basis of its standardized mortality ratio. RESULTS: Among 109 centers performing 7177 operations for congenital heart disease, 22 performed at least 100 cases (72.3% of total operations). Unadjusted mortality rates ranged from 2.5% to 11.4%. A total of 4318 cases could be placed into 1 of the 6 risk categories. Few deaths occurred in risk category 1, and few institutions performed procedures in risk categories 5 and 6, making institutional comparisons in these categories uninformative. Considering mortality rates in categories 2 through 4, institutions displayed either relatively consistent ranks, a threshold increase in mortality as higher-risk procedures were performed, or a threshold decrease in mortality. Standardized mortality ratios indicated which institutions performed better or worse than expected on the basis of their case mix. CONCLUSIONS: The RACHS-1 method can be used to judge relative institutional performance, either by evaluating within-risk-category differences or by comparisons of observed and expected mortality rates.
Adolescent↗