Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for ...these patients. Although the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low-risk operations, the stage I Norwood procedure remains one of the highest-risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals of >90% for the Norwood procedure. This traditional 3-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomly assigned 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years postrandomization. In addition to modifications to the Norwood procedure, the hybrid procedure, a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a comprehensive stage II, which combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to improve not only short-term survival, but also potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but also into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also in terms of the burden of disease, neurodevelopmental outcomes, psychosocial development, and quality of life.
Objective For infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a ...single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock–Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors. Methods Participants in the Single Ventricle Reconstruction trial who survived to discharge after the Norwood procedure were included (n = 426). Interstage mortality was defined as death postdischarge after the Norwood procedure and before the stage II procedure. Univariate analysis and multivariable logistic regression were performed adjusting for site. Results Overall interstage mortality was 50 of 426 (12%)—13 of 225 (6%) for RVPAS and 37 of 201 (18%) for MBTS (odds ratio OR for MBTS, 3.4; P < .001). When moderate to severe postoperative atrioventricular valve regurgitation (AVVR) was present, interstage mortality was similar between shunt types. Interstage mortality was independently associated with gestational age less than 37 weeks (OR, 3.9; P = .008), Hispanic ethnicity (OR, 2.6; P = .04), aortic atresia/mitral atresia (OR, 2.3; P = .03), greater number of post-Norwood complications (OR, 1.2; P = .006), census block poverty level ( P = .003), and MBTS in subjects with no or mild postoperative AVVR (OR, 9.7; P < .001). Conclusions Interstage mortality remains high at 12% and is increased with the MBTS compared with the RVPAS if postoperative AVVR is absent or mild. Preterm delivery, anatomic, and socioeconomic factors are also important. Avoiding preterm delivery when possible and close surveillance after Norwood hospitalization for infants with identified risk factors may reduce interstage mortality.
Objectives We sought to identify risk factors for mortality and morbidity during the Norwood hospitalization in newborn infants with hypoplastic left heart syndrome and other single right ventricle ...anomalies enrolled in the Single Ventricle Reconstruction trial. Methods Potential predictors for outcome included patient- and procedure-related variables and center volume and surgeon volume. Outcome variables occurring during the Norwood procedure and before hospital discharge or stage II procedure included mortality, end-organ complications, length of ventilation, and hospital length of stay. Univariate and multivariable Cox regression analyses were performed with bootstrapping to estimate reliability for mortality. Results Analysis included 549 subjects prospectively enrolled from 15 centers; 30-day and hospital mortality were 11.5% (63/549) and 16.0% (88/549), respectively. Independent risk factors for both 30-day and hospital mortality included lower birth weight, genetic abnormality, extracorporeal membrane oxygenation (ECMO) and open sternum on the day of the Norwood procedure. In addition, longer duration of deep hypothermic circulatory arrest was a risk factor for 30-day mortality. Shunt type at the end of the Norwood procedure was not a significant risk factor for 30-day or hospital mortality. Independent risk factors for postoperative renal failure (n = 46), sepsis (n = 93), increased length of ventilation, and hospital length of stay among survivors included genetic abnormality, lower center/surgeon volume, open sternum, and post-Norwood operations. Conclusions Innate patient factors, ECMO, open sternum, and lower center/surgeon volume are important risk factors for postoperative mortality and/or morbidity during the Norwood hospitalization.
In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a ...modified Blalock-Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups.
Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial.
Transplant-free survival for the RVPAS versus modified Blalock-Taussig shunt groups did not differ at 6 years (64% versus 59%,
=0.25) or with all available follow-up of 7.1±1.6 years (log-rank
=0.13). The RVPAS versus modified Blalock-Taussig shunt treatment effect had nonproportional hazards (
=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48-0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86-2.17;
=0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33-1.74;
=0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year,
<0.001), primarily because of more interventions between the stage II and Fontan procedures (HR, 1.72; 95% confidence interval, 1.00-3.03). Complications did not differ by shunt type; by 6 years, 1 in 5 patients had had a thrombotic event, and 1 in 6 had had seizures.
By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock-Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications.
URL: https://www.clinicaltrials.gov. Unique identifier: NCT00115934.
Inotrope score has been proposed as a marker of illness severity after pediatric cardiac surgery despite a lack of data to support its use as such. The goal of this study was to determine the ...association between inotropic/vasoactive support and clinical outcome in infants after cardiac surgery.
Retrospective chart review.
Dedicated pediatric cardiothoracic intensive care unit at an academic, tertiary care medical center.
One hundred seventy-four patients 0 to 6 months of age admitted to the cardiothoracic intensive care unit after cardiac surgery with cardiopulmonary bypass between August 2007 and June 2008. Forty-three percent were neonates, and 39% had functional single ventricle physiology.
None.
Hourly doses of all vasoactive medications were recorded for the first 48 hrs after admission to the cardiothoracic intensive care unit and a vasoactive-inotropic score was calculated. The maximum vasoactive-inotropic score level over the first 48 hrs was a good predictor of poor clinical outcome (death, cardiac arrest, mechanical circulatory support, renal replacement therapy, and/or neurologic injury). After controlling for diagnosis, high maximum vasoactive-inotropic score was strongly associated with a poor outcome with an adjusted odds ratio of 8.1 (95% confidence interval, 3.4-19.2; p < .001) compared with patients with a low maximum vasoactive-inotropic score. High vasoactive-inotropic score was also associated with prolonged cardiothoracic intensive care unit stay, duration of mechanical ventilation, and time to negative fluid balance.
The amount of cardiovascular support in the first 48 hrs after congenital heart surgery with cardiopulmonary bypass predicts eventual morbidity and mortality in young infants. The degree of support is best characterized by a maximum vasoactive-inotropic score obtained during this period. The usefulness of vasoactive-inotropic score as an independent predictor of clinical outcome in infants after cardiac surgery may have important implications for future cardiothoracic intensive care unit research.
In some studies, tight glycemic control with insulin improved outcomes in adults undergoing cardiac surgery, but these benefits are unproven in critically ill children at risk for hyperinsulinemic ...hypoglycemia. We tested the hypothesis that tight glycemic control reduces morbidity after pediatric cardiac surgery.
In this two-center, prospective, randomized trial, we enrolled 980 children, 0 to 36 months of age, undergoing surgery with cardiopulmonary bypass. Patients were randomly assigned to either tight glycemic control (with the use of an insulin-dosing algorithm targeting a blood glucose level of 80 to 110 mg per deciliter 4.4 to 6.1 mmol per liter) or standard care in the cardiac intensive care unit (ICU). Continuous glucose monitoring was used to guide the frequency of blood glucose measurement and to detect impending hypoglycemia. The primary outcome was the rate of health care-associated infections in the cardiac ICU. Secondary outcomes included mortality, length of stay, organ failure, and hypoglycemia.
A total of 444 of the 490 children assigned to tight glycemic control (91%) received insulin versus 9 of 490 children assigned to standard care (2%). Although normoglycemia was achieved earlier with tight glycemic control than with standard care (6 hours vs. 16 hours, P<0.001) and was maintained for a greater proportion of the critical illness period (50% vs. 33%, P<0.001), tight glycemic control was not associated with a significantly decreased rate of health care-associated infections (8.6 vs. 9.9 per 1000 patient-days, P=0.67). Secondary outcomes did not differ significantly between groups, and tight glycemic control did not benefit high-risk subgroups. Only 3% of the patients assigned to tight glycemic control had severe hypoglycemia (blood glucose <40 mg per deciliter 2.2 mmol per liter).
Tight glycemic control can be achieved with a low hypoglycemia rate after cardiac surgery in children, but it does not significantly change the infection rate, mortality, length of stay, or measures of organ failure, as compared with standard care. (Funded by the National Heart, Lung, and Blood Institute and others; SPECS ClinicalTrials.gov number, NCT00443599.).
There are limited data regarding long-term outcomes after the Ross procedure in children. We evaluated mortality and reintervention in a large pediatric cohort.
A retrospective analysis of all ...patients aged younger than 18 years who underwent the Ross procedure at our institution (1991 to 2013) was conducted. Kaplan-Meier curves and Cox proportion hazard models were used to evaluate long-term outcomes and associated risk factors.
Included were 240 consecutive patients undergoing a Ross/Ross-Konno procedure: 18% infants, 48% children, and 33% adolescents. Infants were more likely to have complex left heart disease (p = 0.005). Overall survival to hospital discharge was 96%; infants had the highest mortality (18%). Long-term survival status was known for 99.6% (median follow-up, 10.7 years). Overall 15-year survival was 87% (lowest in infants, 72%; p = 0.003). Reintervention status was known in 87%. Overall 15-year freedom from any left ventricular outflow tract reintervention was 59%; 85% still had their autograft valve at the latest follow-up. Left ventricular outflow tract reintervention was uncommon in infants (n = 2). Overall 15-year freedom from right ventricular outflow tract reintervention was 53%, and was lower in infants (19%) than in children (51%) and adolescents (76%; p < 0.0001).
Outcomes after the Ross procedure in children vary by age. Infants more commonly have complex left heart disease and experience higher mortality but have excellent long-term autograft durability. Children and adolescents have higher rates of left ventricular outflow tract reintervention, whereas infants are at highest risk of right ventricular outflow tract reintervention.
Structural deterioration of allografts over time is believed to be at least partly related to an immune response mounted against human leukocyte antigen specific to the transplanted tissue. ...SynerGraft (SG) processing (CryoLife, Kennesaw, GA) is a technology that decellularizes an allograft leaving only connective tissue, therefore, reducing immunogenicity and potentially increasing durability of the implant.
We performed a retrospective review of 163 SG patients and 124 standard allograft controls from 3 medical centers. Patient demographics were tabulated, and conduit stenosis and insufficiency were measured by echocardiography.
There were 28 deaths (15 of 163 9% SG patients vs 13 of 124 11% standard patients; p = 0.72), but no deaths were attributed to structural failure of the conduit. The actuarial survival for SG vs standard cohorts was not different at 5 and 10 years. Among the 274 hospital survivors, 17% SG vs 42% standard had evidence for significant conduit dysfunction at the most recent follow-up or before conduit replacement. Freedom from conduit dysfunction was significantly worse at 10 years in the standard group (58%) than in the SG group (83%, p < 0.001).
This study represents a multiinstitutional retrospective comparison of SG and standard cryopreserved allografts used in right ventricular outflow tract reconstruction in a broad range of patient ages. Our results demonstrate that at an intermediate-term to long-term follow-up, conduit dysfunction and pulmonary insufficiency and stenosis are higher among patients receiving standard allografts. We postulate that the improved durability of SG is related to decreased immunogenicity of the SG technology.
In the Single Ventricle Reconstruction (SVR) trial, 1-year transplantation-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a ...modified Blalock-Taussig shunt (MBTS). At 3 years, we compared transplantation-free survival, echocardiographic right ventricular ejection fraction, and unplanned interventions in the treatment groups.
Vital status and medical history were ascertained from annual medical records, death indexes, and phone interviews. The cohort included 549 patients randomized and treated in the SVR trial. Transplantation-free survival for the RVPAS versus MBTS groups did not differ at 3 years (67% versus 61%; P=0.15) or with all available follow-up of 4.8±1.1 years (log-rank P=0.14). Pre-Fontan right ventricular ejection fraction was lower in the RVPAS group than in the MBTS group (41.7±5.1% versus 44.7±6.0%; P=0.007), and right ventricular ejection fraction deteriorated in RVPAS (P=0.004) but not MBTS (P=0.40) subjects (pre-Fontan minus 14-month mean, -3.25±8.24% versus 0.99±8.80%; P=0.009). The RVPAS versus MBTS treatment effect had nonproportional hazards (P=0.004); the hazard ratio favored the RVPAS before 5 months (hazard ratio=0.63; 95% confidence interval, 0.45-0.88) but the MBTS beyond 1 year (hazard ratio=2.22; 95% confidence interval, 1.07-4.62). By 3 years, RVPAS subjects had a higher incidence of catheter interventions (P<0.001) with an increasing HR over time (P=0.005): <5 months, 1.14 (95% confidence interval, 0.81-1.60); from 5 months to 1 year, 1.94 (95% confidence interval, 1.02-3.69); and >1 year, 2.48 (95% confidence interval, 1.28-4.80).
By 3 years, the Norwood procedure with RVPAS compared with MBTS was no longer associated with superior transplantation-free survival. Moreover, RVPAS subjects had slightly worse right ventricular ejection fraction and underwent more catheter interventions with increasing hazard ratio over time.
http://www.clinicaltrials.gov. Unique identifier: NCT00115934.
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