Status of the left ventricle after arterial switch operation for transposition of the great arteries Hemodynamic and echocardiographic evaluation

AbstractBackground : The potential for improved preservation of systemic ventricular function represents an important reason for the increasing popularity of the arterial switch operation. In support of this expectation, prior studies in patients early after arterial switch operation have found normal ventricular contractility and function. This study was conducted to extend those observations to up to 10 years of follow-up and to directly examine the effects of a coexisting ventricular septal defect or short-term preparatory banding of the pulmonary artery before the arterial switch operation. Methods : Patients operated on from 1983 through 1991 were included. Echocardiographic and catheterization data were collected as part of a prospective evaluation of outcome in all patients who undergo the arterial switch operation at Boston Children's Hospital, with inclusion of data from the most recent catheterization only. Echocardiograms performed at least 6 months after the operation were included, with assessment of both the most recent status as well as serial trends. Whenever possible, echocardiographic evaluation included data necessary to perform analysis of ventricular mechanics including indices of afterload, preload, and contractility. Comparison was made to normal values and between subgroups defined on the basis of an arterial switch operation with or without ventricular septal defect and those who had a rapid two-stage arterial switch operation. Results : Invasive measures of left and right ventricular filling pressures, cardiac index, and pulmonary vascular resistance did not differ among the three groups. Overall, echocardiographic left ventricular end-diastolic dimension, wall thickness, mass, afterload (end-systolic wall stress), function (fractional shortening and rate-corrected velocity of fiber shortening), contractility (stress-velocity and stress-shortening relations), and preload were normal, and none of these variables was different between the groups with and without a ventricular septal defect. Serial evaluation indicated a slight but significant trend toward ventricular dilatation, perhaps related to a relatively high incidence of at least mild aortic regurgitation (30%). In contrast, in the rapid two-stage group the echocardiographic indices of left ventricular function (fractional shortening and velocity of fiber shortening) and contractility (stress-velocity and stress-shortening relations) were found to be mildly but significantly reduced compared with normal subjects and with the other arterial switch operation groups. Over the duration of follow-up encompased by this study, no tendency toward progressive depression of function was seen. Conclusions : As the length of observation after the arterial switch operation continues to increase, left ventricular size, mass, functional status, and contractility continues to be normal, with no evidence of time-related deterioration of function. As previously reported, the rapid two-stage arterial switch operation does represent a higher risk for mild impairment of myocardial mechanics. (J T horac C ardiovasc S urg 1995;109:311-21

Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries

AbstractBetween January 1983 and January 1992, 470 patients underwent an arterial switch operation at our institution. An intact (or virtually intact) ventricular septum was present in 278 of 470 (59%); a ventricular septal defect was closed in the remaining 192. Survivals at 1 month and 1, 5, and 8 years among the 470 patients were 93%, 92%, 91%, and 91%, respectively. The hazard function for death (at any time) had a rapidly declining single phase that approached zero by one year after the operation. Risk factors for death included coronary artery patterns with a retropulmonary course of the left coronary artery (two types) and a pattern in which the right coronary artery and left anterior descending arose from the anterior sinus with a posterior course of the circumflex coronary. The only procedural risk factor identified was augmentation of the aortic arch; longer duration of circulatory arrest was also a risk factor for death. Earlier date of operation was a risk factor for death, but only in the case of the senior surgeon. Reinterventions were performed to relieve right ventricular and/or pulmonary artery stenoses alone in 28 patients. The hazard function for reintervention for pulmonary artery or valve stenosis revealed an early phase that peaked at 9 months after the operation and a constant phase for the duration of follow-up. Incremental risk factors for the early phase included multiple ventricular septal defects, the rapid two-stage arterial switch, and a coronary pattern with a single ostium supplying the right coronary and left anterior descending, with a retropulmonary course of the circumflex. The need for reintervention has decreased with time. The arterial switch operation can currently be performed early in life with a low mortality risk (<5%) and a low incidence of reintervention (<10%) for supravalvular pulmonary stenosis. The analyses indicate that both the mortality and reintervention risks are lower in patients with less complex anatomy. (J THORAC CARDIOVASC SURG 1995; 109: 289-302

The impact of socio-economic status on health related quality of life for children and adolescents with heart disease

Background: Socioeconomic status (SES) is known to influence children’s health-related quality of life. Many SES indicators assess distinct dimensions of a family’s position rather than measuring the same underlying construct. Many researchers, however, see SES indicators as interchangeable. The primary aim of this study was to determine which measure of SES had the strongest impact on health-related quality of life. Methods: This is a secondary analysis of the Pediatric Cardiac Quality of Life Inventory Validation Study. The SES variables were family income, Hollingshead Index (occupational prestige), and highest parent educational attainment level. Health-related quality of life was measured using the Pediatric Cardiac Quality of Life Inventory. Correlations tested the relationship among the three SES indicators. Regression-based modeling was used to calculate the strength of the association between SES measures and the Pediatric Cardiac Quality of Life Inventory. Results: The correlations among the SES measures were moderately high, with the correlation between the Hollingshead Index and parental education being r = 0.62 (95% CI = 0.56-0.65). There were equally high correlations between family income and the Hollingshead (r = 0.61, 95% CI = 0.57-0.65) and a slightly lower correlation between family income and parental education (r = 0.55, 95% CI = 0.52-0.59). Family income had the highest explanatory value compared to the Hollingshead Index or parental educational attainment, while controlling for sex, race, current cardiac status, and original diagnosis, accounting for 4-5% of the variation in patient and parent Pediatric Cardiac Quality of Life Inventory Total score, respectively, compared to the other SES measures. Conclusion: Family income as an SES measure demonstrated the greatest fidelity with respect to health-related quality of life as measured by the Pediatric Cardiac Quality of Life Inventory across respondent groups and explained more of the variation compared to the Hollingshead Index or highest parental educational attainment

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction

ObjectiveWe compare the hemodynamics and perioperative course of shunt type in hypoplastic left heart syndrome at the time of stage 2 reconstruction and longer-term survival.MethodsWe retrospectively reviewed the echocardiograms, catheterizations, and hospital records of all patients who had a stage 1 reconstruction between January 2002 and May 2005 and performed a cross-sectional analysis of hospital survivors.ResultsOne hundred seventy-six patients with hypoplastic left heart syndrome and variants underwent a stage 1 reconstruction with either a right ventricle–pulmonary artery conduit (n = 62) or a modified Blalock–Taussig shunt (n = 114). The median duration of follow-up is 29.1 months (range, 0-57 months). By means of Kaplan–Meier analysis, there is no difference in survival at 3 years (right ventricle–pulmonary artery conduit: 73% [95% confidence limit, 59%–83%] vs modified Blalock–Taussig shunt: 69% [95% confidence limit, 59%–77%]; P = .6). One hundred twenty-four patients have undergone stage 2 reconstruction (78 modified Blalock–Taussig shunts and 46 right ventricle–pulmonary artery conduits). At the time of the stage 2 reconstruction, patients with right ventricle–pulmonary artery conduits were younger (153 days [range, 108–340 days]; modified Blalock–Taussig shunt, 176 days [range, 80–318 days]; P = .03), had lower systemic oxygen saturation (73% [range, 58%–85%] vs 77% [range, 57%–89%], P < .01), and had higher preoperative hemoglobin levels (15.8 g/dL [range, 13–21 g/dL] vs 14.8 g/dL [range, 12–19 g/dL], P < .01) compared with those of the modified Blalock–Taussig shunt group. By means of echocardiographic evaluation, there was a higher incidence of qualitative ventricular dysfunction in patients with right ventricle–pulmonary artery conduits (14/46 [31%] vs 9/73 [12%], P = .02). However, no difference was observed in common atrial pressure or the arteriovenous oxygen difference.ConclusionInterim analyses suggest no advantage of one shunt type over another. This report raises concern of late ventricular dysfunction and outcome in patients with a right ventricle–pulmonary artery conduit

A Population Pharmacokinetic Analysis of Milrinone in Pediatric Patients After Cardiac Surgery

The purpose of this study was to ascertain the optimal pharmacokinetic model for milrinone in pediatric patients after cardiac surgery when milrinone was administered as a slow loading dose followed by a constant-rate infusion. The data used for pharmacokinetic analysis were collected in a prospective, randomized, placebo-controlled multi-center trial of milrinone as prophylaxis for the development of low cardiac output syndrome after surgery for repair of complex congenital cardiac defects. Two blood samples were randomly collected from each patient for determination of plasma milrinone concentrations with subsequent population pharmacokinetic modeling. The pharmacokinetics of milrinone in pediatric patients under 6year's age were best described by a weight-normalized one compartment model after a slow loading dose followed by a constant-rate infusion. The volume of distribution was 482mlkg −1 , and was independent of age. Clearance was a linear function of age given by Cl=2.42mlkg −1 min −1 [1+0.0396*age].Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/45063/1/10928_2004_Article_485925.pd

Impact of Surgical Complexity on Health‐Related Quality of Life in Congenital Heart Disease Surgical Survivors

Background: Surgical complexity and related morbidities may affect long‐term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS‐1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. Methods and Results: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS‐1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient–parent pairs were included. Although higher ABC scores and RACHS‐1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=−0.10 to −0.29) for all groups. Ordinary least squares regression showed weak association with R 2=0.06 to R 2=0.28. After accounting for single‐ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. Conclusions: ABC scores and RACHS‐1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long‐term QOL scores. Further studies are warranted to determine other predictors of QOL variation