Background Red blood cell transfusion is associated with morbidity and mortality among adults undergoing cardiac surgery. We aimed to evaluate the association of transfusion with morbidity among ...pediatric cardiac surgical patients. Methods Patients discharged after cardiac surgery in 2003 were retrospectively reviewed. The red blood cell volume administered during the first 48 postoperative hours was used to classify patients into nonexposure, low exposure (≤15 mL/kg), or high exposure (>15 mL/kg) groups. Cox proportional hazards modeling was used to evaluate the association of red blood cell exposure to length of hospital stay (LOS). Results Of 802 discharges, 371 patients (46.2%) required blood transfusion. Demographic differences between the transfusion exposure groups included age, weight, prematurity, and noncardiac structural abnormalities (all p < 0.001). Distribution of Risk Adjusted Classification for Congenital Heart Surgery, version 1 (RACHS-1) categories, intraoperative support times, and postoperative Pediatric Risk of Mortality Score, Version III (PRISM-III) scores varied among the exposure groups ( p < 0.001). Median duration of mechanical ventilation (34 hours 0 to 493 versus 27 hours 0 to 621 versus 16 hours 0 to 375), incidence of infection (21 14% versus 29 13% versus 17 4%), and acute kidney injury (25 17% versus 29 13% versus 34 8%) were highest in the high transfusion exposure group when compared with the low or nontransfusion groups (all p < 0.001). In a multivariable Cox proportional hazards model, both the low transfusion group (adjusted hazard ratio HR 0.80, 95% confidence interval CI: 0.66 to 0.97, p = 0.02) and high transfusion group (adjusted HR 0.66, 95% CI: 0.53 to 0.82, p < 0.001) were associated with increased LOS. In subgroup analyses, both low transfusion (adjusted HR 0.81, 95% CI: 0.65 to 1.00, p = 0.05) and high transfusion (adjusted HR 0.65, 95% CI: 0.49 to 0.87, p = 0.004) in the biventricular group but not in the single ventricle group was associated with increased LOS. Conclusions Blood transfusion is associated with prolonged hospitalization of children after cardiac surgery, with biventricular patients at highest risk for increased LOS. Future studies are necessary to explore this association and refine transfusion practices.
Objective Two randomized trials of hematocrit strategy during hypothermic cardiopulmonary bypass in infant heart surgery have been performed. The first suggested worse outcomes were concentrated in ...patients with lower hematocrit levels (approximately 20%), whereas the second suggested there was little benefit to increasing the hematocrit level above 25%. The form of the relationship between continuous hematocrit levels and outcomes requires further study. Methods In the two trials, 271 infants who underwent biventricular repair not involving the aortic arch were enrolled. Analysis was undertaken of the effects of hematocrit level, as a continuous variable, at the onset of low-flow cardiopulmonary bypass. Results Psychomotor Development Index scores at age 1 year varied nonlinearly with hematocrit levels, with increasing scores up to 23.5% hematocrit ( P < .001) and a plateau effect beyond 23.5% ( P = .42), based on a piecewise linear model. Lower hematocrit levels were associated with more positive intraoperative fluid balance ( P < .001 for linear trend) and marginally associated with higher serum lactate levels at 60 minutes after bypass ( P = .08 for linear trend), but not with blood products given, nadir of cardiac index in the first 24 hours, or Mental Development Index scores. Conclusions A hematocrit level at the onset of low-flow cardiopulmonary bypass of approximately 24% or higher is associated with higher Psychomotor Development Index scores and reduced lactate levels. Because the effects of hemodilution may vary according to diagnosis, age at operation, bypass variables such as pH strategy and flow rate, and other perioperative factors, this study cannot ascertain a universally “safe” hemodilution level.
Objective Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The ...multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes. Methods All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test. Results Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively. Conclusion In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.
Objectives The goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle ...palliation (SVP). Background Management of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV. Methods Patients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group. Results Mean initial z -scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z -scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum. Conclusions In these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.
Abstract Objectives The goal of this single-center series was to assess differences in reintervention by the type of valve used for surgical bioprosthetic pulmonary valve replacement and to identify ...independent predictors of reintervention. Methods Data were retrospectively collected for 611 patients undergoing pulmonary valve replacement from 1996 to 2014. Kaplan–Meier estimation and Cox proportional hazards regression methodologies were used. Results The median age of patients was 17.8 years (interquartile range, 11.9-27.3). The diagnosis was tetralogy of Fallot in 69% of patients. The median follow-up was 3.0 years (interquartile range, 1.1-5.3). Valve types included Sorin Mitroflow (Milan, Italy), 316 (50%; median age 16.5 years); Carpentier-Edwards (Irvine, Calif) Magna/MagnaEase, 223 (35%; median age, 19.3 years); and Carpentier-Edwards Perimount, 72 (11%; median age, 21.9 years). Reintervention occurred in 6.7% of patients (41/633) and was higher in children than adults (hazard ratio, 4.8). Age-adjusted 5-year reintervention rates were Sorin Mitroflow, 13.4%; Carpentier-Edwards Magna/MagnaEase, 2.1%; and Carpentier-Edwards Perimount, 0%. Reintervention was not associated with gender, valve insertion method, or concurrent procedures. The only independent risk factor for reintervention after controlling for age was valve type ( P < .001). The Sorin Mitroflow valve had a shorter time to reintervention than the other 2 valve types (hazard ratios both >7, each P < .001). Differences by valve type did not depend on age (interaction P = .61). Conclusions Bioprosthetic pulmonary valve replacement in patients with congenital heart disease has excellent short-term outcomes, but children have an approximately 5-fold greater risk of reintervention than adults. Independently of age, reintervention rates vary by valve type. These differences may be important in valve selection and follow-up.
Background Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this ...report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. Methods Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. Results Sixteen children with UCAVC (8 patients 50% were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m2 (range, 22 to 35 mL/m2 ). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m2 (range, 26 to 64 mL/m2 ). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. Conclusions Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.
Background We have shown previously that technical performance score (TPS) is strongly associated with early mortality and major postoperative adverse events in a diverse group of patients. We now ...report evaluation of the validity of TPS in predicting late outcomes in the same group of patients. Methods Patients who underwent surgery between June 1, 2005 and June 30, 2006 were included. The TPS were assigned based on discharge echocardiograms and certain clinical criteria as previously described. Follow-up data for up to 4 years were retrospectively collected. Cox proportional hazards models were used for analysis. Results A total of 679 patients were included in the analysis. One hundred twenty-three (18%) were neonates, 213 (31%) infants, 291 (435) children, and 52 (8%) adults. Four hundred ninety-one (72%) were in low-risk adjustment in congenital heart surgery (RACHS; 1 to 3), 109 (16%) in high risk (4 to 6), and 27 (4%) were less than 18 years and could not be assigned a RACHS score. Three hundred thirty-one (48%) had an optimal TPS, 283 (42%) adequate, 61 (9%) inadequate, and 4 (1%) could not be scored. There were 34 (5%) late deaths and 149 (22%) late unplanned reinterventions. By univariate analysis, age, RACHS-1 categories, and TPS were all significantly associated with late reintervention (p < 0.001 for all), while TPS and RACHS-1 were significant factors for mortality ( p < 0.001). On multivariable modeling, inadequate TPS was strongly associated with both late mortality ( p = 0.001; HR hazard ratio 3.8, CI confidence interval 1.7 to 8.4) and late reintervention ( p = 0.002, HR 2.1, CI 1.3 to 3.3) after controlling for RACHS-1 and age. Conclusions The TPS has a strong association with late outcomes across a wide range of age and disease complexity and may serve as a tool to identify patients who are at a higher risk for late reintervention or mortality.
Objective We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure. Methods From January 2001 through ...December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children’s Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis–aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan–Meier method was used to assess survival. Results Thirty-eight (23%) of 165 patients had mitral stenosis–aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis–aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle–subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis–aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time ( P = .02) and the need for postoperative extracorporeal membrane oxygenation support ( P < .001) were associated with a higher mortality rate. Conclusions With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis–aortic atresia variant and left ventricle–subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.
Objective No method of measuring technical performance exists for the stage I Norwood procedure. Hospital mortality is usually used as a surrogate for technical performance, but evidence is lacking ...to support this concept. A technical score was designed by expert consensus. Methods The technical score included the following steps: (1) Stage I was divided into subprocedures according to anatomic areas where an intervention is performed. (2) For each subprocedure, three score categories (optimal, adequate, and inadequate) were defined on the basis of echocardiography, catheterization, and/or clinical data. (3) Subprocedures were analyzed for the whole group and by surgeon. (4) Overall repair was also scored: optimal if all attempted subprocedures were optimal, inadequate if any was inadequate, and adequate for everything in between. (5) All patients undergoing the stage I procedure from January 2004 to December 2006 were retrospectively studied. Results One hundred ten patients were included (operated on by six surgeons), and 4 were excluded for lack of reliable postoperative data. Most subprocedures were scored as optimal. Subprocedures with the largest inadequate scores were distal arch reconstruction in 7 (6%) patients and aortopulmonary shunt in 3 (5%). No statistical differences were found among surgeons either by subprocedure or by overall outcome, although individual sample sizes were small. The overall score correlated with length of stay, extracorporeal membrane oxygenator support, and hospital mortality. Conclusions Technical performance can be measured after the stage I procedure, and performance score correlates with early outcome. This score may also be useful as a self-assessment tool.
Objectives We previously reported that postoperative hemodynamics and developmental outcomes were better among infants randomized to a higher hematocrit value during hypothermic cardiopulmonary ...bypass. However, worse outcomes were concentrated in patients with hematocrit values of 20% or below, and the benefits of hematocrit values higher than 25% were uncertain. Methods We compared perioperative hemodynamics and, at 1 year, developmental outcome and brain magnetic resonance imaging in a single-center, randomized trial of hemodilution to a hematocrit value of 25% versus 35% during hypothermic radiopulmonary bypass for reparative heart surgery in infants undergoing 2-ventricle repairs without aortic arch obstruction. Results Among 124 subjects, 56 were assigned to the lower-hematocrit strategy (24.8% ± 3.1%, mean ± SD) and 68 to the higher-hematocrit strategy (32.6% ± 3.5%). Infants randomized to the 25% strategy, compared with the 35% strategy, had a more positive intraoperative fluid balance ( P = .007) and lower regional cerebral oxygen saturation at 10 minutes after cooling ( P = .04) and onset of low flow ( P = .03). Infants with dextro-transposition of the great arteries in the 25% group had significantly longer hospital stay. Other postoperative outcomes, blood product usage, and adverse events were similar in the treatment groups. At age 1 year (n = 106), the treatment groups had similar scores on the Psychomotor and Mental Development Indexes of the Bayley Scales; both groups scored significantly worse than population norms. Conclusions Hemodilution to hematocrit levels of 35% compared with those of 25% had no major benefits or risks overall among infants undergoing 2-ventricle repair. Developmental outcomes at age 1 year in both randomized groups were below those in the normative population.
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