Objective The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that ...might favor the modified Blalock–Taussig shunt or a right ventricle-to-pulmonary artery shunt. Methods We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors. Results Data from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status ( P = .01), obstructed pulmonary venous return ( P < .001), smaller ascending aorta ( P = .02), and anatomic subtype. Constant phase factors associated with death included genetic syndrome ( P < .001) and lower gestational age ( P < .001). The right ventricle-to-pulmonary artery shunt demonstrated better survival in the 51% of subjects who were full term with aortic atresia ( P < .001). The modified Blalock–Taussig shunt was better among the 4% of subjects who were preterm with a patent aortic valve ( P = .003). Lower pre-Norwood right ventricular fractional area change, pre-Norwood surgery, and anatomy other than hypoplastic left heart syndrome were independently associated with transplantation (all P < .03), but shunt type was not ( P = .43). Conclusions Independent risk factors for intermediate-term mortality include lower socioeconomic status, anatomy, genetic syndrome, and lower gestational age. Term infants with aortic atresia benefited from a right ventricle-to-pulmonary artery shunt, and preterm infants with a patent aortic valve benefited from a modified Blalock–Taussig shunt. Right ventricular function and anatomy, but not shunt type, were associated with transplantation.
Abstract Objective Early survival advantages after Norwood with right-ventricle-(RV)-to-pulmonary-artery conduit (NW-RVPA) over Norwood-operation with a Blalock-Taussig shunt (NW-BT) are offset by ...concerns regarding delayed RV dysfunction. We compared trends in survival, RV dysfunction, and tricuspid valve regurgitation (TR) between NW-RVPA and NW-BT for propensity-matched neonates with critical left ventricular outflow tract obstruction (LVOTO). Methods In an inception cohort (2005-2014; 21 institutions), 454 neonates with critical LVOTO underwent Norwood stage 1. Propensity-score matching paired 169 NW-RVPA patients with 169 NW-BT patients. End-states were compared between NW-RVPA and NW-BT using competing-risks, multiphase, parametric, hazard analysis. Post-Norwood echocardiogram reports (n = 2993) were used to grade RV dysfunction and TR. Time-related prevalence of ≥moderate RV dysfunction and TR were characterized using nonlinear mixed-model regression, and compared between groups via multiphase, parametric models. Results Overall 6-year survival was better after NW-RVPA (70%) versus NW-BT (55%; P < .001). Additionally, transplant-free survival during this time was better after NW-RVPA (64%) versus NW-BT (53%; P = .004). Overall prevalence of ≥moderate RV dysfunction reached 11% within 3 months post-Norwood. During this time, RV dysfunction after NW-BT was 16% versus 6% after NW-RVPA ( P = .02), and coincided temporally with an increased early hazard for death. For survivors, late RV dysfunction was <5% and was not different between groups ( P = .36). Overall prevalence of ≥moderate TR reached 13% at 2 years post-Norwood and was increased after NW-BT (16%) versus NW-RVPA (11%; P = .003). Late TR was similar between groups. Conclusions Among propensity-score–matched neonates with critical LVOTO, NW-RVPA offers superior 6-year survival with no greater prevalence of RV dysfunction or TR than conventional NW-BT operations.
Treatment of congenital heart disease may include placement of a right ventricle to pulmonary artery conduit that requires future surgical replacement. We sought to identify surgeon-modifiable ...factors associated with durability (defined as freedom from surgical replacement or explantation) of the initial conduit in children less than 2 years of age at initial insertion.
Since 2002, 429 infants were discharged from 24 Congenital Heart Surgeons' Society member institutions after initial conduit insertion. Parametric hazard analysis identified factors associated with conduit durability while adjusting for patient characteristics, the institution where the conduit was inserted, and time-dependent interval procedures performed after conduit insertion but before replacement/explantation.
In all, 138 conduit replacements (32%) and 3 explantations (1%) were performed. Conduit durability at a median follow-up of 6.0 years (range, 0.1 to 11.7) was 63%. After adjusting for interval procedures and institution, placement of a conduit with smaller z-score was associated with earlier replacement/explantation (p = 0.002). Moreover, conduit durability was substantially reduced with aortic allografts (p = 0.002) and pulmonary allografts (p = 0.03) compared with bovine jugular venous valved conduits (JVVC). The JVVC were 12 mm to 22 mm in diameter at insertion (compared with 6 mm to 20 mm for allografts); therefore, a parametric propensity-adjusted analysis of patients with aortic or pulmonary allografts versus JVVC with diameter of 12 mm or greater was performed, which verified the superior durability of JVVC.
Pulmonary conduit type and z-score are associated with late conduit durability independent of the effects of institution and subsequent interval procedures. Surgeons can improve long-term conduit durability by judiciously oversizing, and by selecting a JVVC.
There is increasing interest in reducing the duration of mechanical ventilation after pediatric cardiac operations. However, the contemporary use of an early extubation strategy and its effect on ...clinical outcomes is poorly understood.
Data from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) were analyzed to determine the association of early extubation with postoperative length of stay (PLOS). Two operations were analyzed: complete repair of tetralogy of Fallot (TOF) in patients older than 45 days and the Fontan procedure. Centers were stratified into tertiles by frequency of early extubation, defined as less than 6 hours after leaving the operating room. Patients extubated after 48 hours were excluded.
Among 92 centers, early extubation was performed in 31.5% (478 of 1,519) of children undergoing TOF repair and in 69.8% (1,153 of 1,653) of those undergoing the Fontan procedure. Early extubation after TOF repair was associated with heavier weight at operation (p < 0.001) and fewer preoperative risk factors (p = 0.016). After adjustment for covariates, average PLOS after TOF repair was shorter for centers in the highest tertile of early extubation rate than for the lowest tertile centers, which have low early extubation rate (p = 0.04). No association was found between center early extubation rate and PLOS for Fontan procedures (p = 0.08).
Early extubation is common after repair of TOF and the Fontan procedure. A high institutional rate of early extubation after TOF repair is associated with shorter PLOS. Further analysis is needed to understand what effect early extubation might have on other meaningful measures such as resource use.
Extracorporeal cardiopulmonary resuscitation (ECPR) has been shown to improve survival after in-hospital pediatric cardiac arrest. We describe our experience with ECPR for refractory cardiac arrest ...in pediatric cardiac patients.
We performed a retrospective analysis of the use of venoarterial extracorporeal membrane oxygenation (ECMO) for in-hospital cardiac arrest from 2002 to 2011. The primary endpoint was survival to discharge, and the secondary endpoint was long-term functional neurologic status.
Of 160 total uses of cardiac ECMO in 159 patients, 90 (56%) were ECPR (mean age 2.05 years; range, 0 days to 16.5 years). Sixty-four patients (71%) were postoperative, of which 36 were single ventricle and 28 were biventricular. Nine patients (10%) had cardiomyopathy-myocarditis, and 17 patients (19%) were nonpostoperative (5 single ventricle; 12 biventricular). Fifty-nine patients (66%) had open chest cannulation, and 31 (34%) had peripheral cannulation. Fifty patients (56%) survived to discharge. Duration of ECMO was 4.3±4.0 days (median 3) for survivors and 6.3±5.4 days (median 5) for nonsurvivors (p<0.05). On follow-up, almost half of survivors without genetic syndromes had normal neurologic status.
Extracorporeal cardiopulmonary resuscitation is an appropriate application of ECMO in pediatric cardiac patients. We report overall survival of 56%. Cardiomyopathy patients have favorable outcomes (89% survival). Biventricular patients have better outcomes then single ventricle patients (p<0.01). Extracorporeal cardiopulmonary resuscitation also seems to be a good strategy for nonpostoperative patients (71% survival). Nearly half of postoperative patients (46%) resuscitated with ECPR survived to hospital discharge.
Abstract Objectives To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in ...patients with tricuspid atresia. Methods Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes. Results Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention ( P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA. Conclusions Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.
Pulmonary vein stenosis (PVS), both congenital and acquired, is challenging to treat surgically with uncertain long-term results. We reviewed an 11-year surgical experience in 52 children.
From 2002 ...to 2012, 52 children age 0 days to 13 years (mean 1.9 years, median 11.7 months) weighing 2.2 to 32.5 kg (mean 9.3 kg, median 7.6 kg) had surgical relief of PVS. Based on clinical characteristics or complexity, 33 (63%) had a sutureless pericardial well repair and 19 (37%) had a more standard patch repair. There were no significant differences in clinical characteristics between the 2 techniques. Twenty children (38%) had prior anomalous pulmonary vein repair and 8 had primary pulmonary vein stenosis; 26 (50%) had other operations at the time of PVS relief.
There were 2 hospital deaths (10.5%) in the "standard" group and 5 (15.2%) in the sutureless group (p>0.99). Despite postoperative evidence of PVS relief by echocardiogram or cardiac cath in all patients, at 5 years, actuarial freedom from PVS recurrence or death in the hospital survivors was 67% in the standard group and 58% in the sutureless group. Most recurrences or deaths occurred within 6 months of operation. Heterotaxy, single ventricle anatomy, bilateral disease, and previous anomalous pulmonary vein repair were not predictors of failure.
Surgical treatment of pulmonary vein stenosis remains a challenging problem with nontrivial early mortality and ongoing risk for recurrence or death regardless of surgical technique employed. Clearly, development of methods for earlier intervention or detection and improved surgical techniques are warranted.
Cerebral near-infrared spectroscopy (NIRS) is being used with increasing frequency in the care of pediatric patients after surgery for congenital heart disease. Near-infrared spectroscopy provides a ...means of evaluating regional cerebral oxygen saturation (cSaO(2)) noninvasively, with correlations to cardiac output and central venous saturation. Prior studies have demonstrated that systemic venous saturation can predict outcome after the Norwood procedure. With this in mind, we sought to determine whether regional cSaO(2) by NIRS technology could predict risk of adverse outcome after the Norwood procedure.
We reviewed the first 48 hours of postoperative hemodynamic data on 50 patients with hypoplastic left heart syndrome at our institution who underwent the Norwood procedure. Cerebral oxygen saturation data within 48 hours of surgery were analyzed for association with subsequent adverse outcome, which was defined as intensive care unit length of stay greater than 30 days, need for extracorporeal membrane oxygenation, or hospital death after 48 hours.
There were 18 adverse events among the 50 subjects. The mean cSaO(2) for the entire cohort at 1 hour, 4 hours, and 48 hours after surgery was 51% +/- 7.5%, 50% +/- 9.4%, and 59% +/- 8.1%, respectively. Mean cSaO(2) for the first 48 postoperative hours of less than 56% was a risk factor for subsequent adverse outcome (odds ratio 11.9, 95% confidence interval: 2.5 to 55.8). Mean cerebral NIRs of less than 56% over the first 48 hours after surgery yielded a sensitivity of 75.0% and a specificity of 79.4% to predict those at risk for subsequent adverse events.
Low regional cerebral oxygen saturation by NIRS in the first 48 hours after the Norwood procedure has a strong association with subsequent adverse outcome. Monitoring of cerebral saturation can serve as a valuable monitoring tool and can identify patients at risk for poor outcome.
Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 190 primary pediatric heart transplants.
Since 1988, 27 ...(14.2%) of 190 children less than 18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 163 (85.8%) non-Fontan primary transplants, the Fontan patients were similar in age (8.2 ± 5.0 vs 6.5 ± 6.0 years), presensitization, and pretransplant clinical status. More Fontan patients had prior operations (100% vs 50%; p < 0.0001) and needed pulmonary artery reconstruction (100% vs 23.5%; p < 0.0001). Twelve (44%) had protein-losing enteropathy.
Donor ischemic times (211 ± 72 vs 170 ± 61 minutes; p = 0.0018) and cardiopulmonary bypass times (197 ± 91 vs 121 ± 53 minutes; p < 0.0001) were greater in the Fontan group as were durations of ventilator support (4.9 ± 6.6 vs 2.6 ± 3.9 days; p = 0.018) and hospital stay (20.2 ± 17.5 vs 14.3 ± 12.4 days; p = 0.0435). The Fontan group had one 30-day mortality. One-year actuarial survival (81.5% vs 84.6%, Fontan vs non-Fontan) and five-year actuarial survival (65.5% vs 66.2%, Fontan vs non-Fontan) were similar, as was rejection incidence at one year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes per patient; p = 0.3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years posttransplant compared with 18 non-Fontan patients (11.0%) retransplanted 5.2 ± 3.4 years posttransplant (p = 0.3346).
Contrary to prior reports, we did not identify any early or midterm disadvantage for children undergoing heart transplantation after a previous Fontan procedure despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.
Objective Patients with single ventricle heart defects often undergo a palliative bidirectional Glenn operation. For this operation, we analyzed potential risk factors for morbidity and mortality. We ...also evaluated the effects of a persistent left superior vena cava by comparing the outcomes of unilateral and bilateral operations. Methods We reviewed the clinical records of 270 consecutive patients who underwent a bidirectional Glenn operation between 2001 and 2007. A total of 226 patients underwent unilateral operations and 44 patients underwent bilateral operations. Patient characteristics included weight and age, single ventricle morphology, vena caval anatomy, and previous surgery. Operative details included cardiopulmonary bypass technique and duration, pulmonary artery management, hemi-Fontan construction, concomitant procedures, and hemodynamics. Outcome data included duration of chest tube drainage, lengths of intensive care unit and hospital stay, morbidity, and mortality (<30 days). Results The median length of chest tube drainage was 2.4 days (range 1–20 days). Risk factors for prolonged drainage were elevated central venous pressure ( P = .015) and transpulmonary gradient ( P = .011). The median lengths of stay in the intensive care unit and hospital were 50 hours (range 20–1628 hours) and 5 days (range 2–83 days), respectively. Risk factors for both included prolonged cardiopulmonary bypass time, elevated central venous pressure and transpulmonary gradient, and right ventricular morphology. Overall, 72 of 270 patients (27%) had 116 postoperative complications. Risk factors included prolonged cardiopulmonary bypass time ( P = .002) and elevated central venous pressure ( P = .029). Mortality was 2 of 270 patients (0.7%). No risk factors for death were identified. Weight (median 6.8 kg vs 6.2 kg, P = .038) and age (median 186 days vs 159 days, P = .001) at the time of surgery were significantly greater in the bilateral bidirectional Glenn group compared with the unilateral group. However, there was no difference in any of the outcome variables. Conclusion Outcomes were adversely affected primarily by prolonged cardiopulmonary bypass time, elevated central venous pressure and transpulmonary gradient, and right ventricular morphology. Specifically, outcomes were unaffected by the presence of a left superior vena cava, cannulation strategy, or antegrade pulmonary blood flow. There were few differences between the unilateral and bilateral groups, none of which were postoperative outcomes.
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