Background Circulatory vulnerability reflected by low systemic venous oxygen saturation after surgical palliation of hypoplastic left heart syndrome predicts adverse neurologic outcome and reduced ...survival, and targeting venous saturation may improve outcome. We herein test the hypothesis that near-infrared spectroscopy (NIRS)-derived cerebral and somatic/renal regional saturations can predict survival. Methods Patient data, from a prospective Institutional Review Board-approved registry of hemodynamic measures after initial palliation of hypoplastic left heart syndrome, were analyzed with logistic and multivariable mixed regression methods to determine relationships between standard hemodynamic measures, direct and NIRS measures of saturation, and outcome. The primary outcome measure was survival through hospital discharge and 30 days. Results From the entire cohort of 329 patients, complete data for comparative analysis of physiologic predictors were available from 194 patients. The early survival rate was 92.1%; extracorporeal membrane oxygenation was used in 8.8% of patients. The mean arterial pressure, arterial cerebral, and somatic cerebral NIRS saturation differences were significantly higher for survivors versus nonsurvivors. Multivariable analysis found cerebral and somatic NIRS saturations, heart rate, and arterial pressure as predictors of outcome. Bivariate analysis of mean arterial pressure and somatic saturation allowed early identification of low cardiac output and high mortality risk. Conclusions Continuous noninvasive measurement of regional cerebral and somatic NIRS saturations in the early postoperative period can predict outcomes of early mortality and extracorporeal membrane oxygenation use in hypoplastic left heart syndrome. Because outcomes were strongly determined by NIRS measures at 6 hours, early postoperative NIRS measures may be rational targets for goal-directed interventions.
The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively ...review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population.
A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms. The reference lists of identified articles were also searched. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. A management algorithm was devised that stratified children with CHD on the basis of established risk factors. For those deemed to be at high risk for developmental disorder or disabilities or for developmental delay, formal, periodic developmental and medical evaluations are recommended. A CHD algorithm for surveillance, screening, evaluation, reevaluation, and management of developmental disorder or disability has been constructed to serve as a supplement to the 2006 American Academy of Pediatrics statement on developmental surveillance and screening. The proposed algorithm is designed to be carried out within the context of the medical home. This scientific statement is meant for medical providers within the medical home who care for patients with CHD.
Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay. Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.
Objectives Neonates with hypoplastic left heart syndrome have significant hemodynamic threats to cerebral perfusion and are at risk of reduced neurodevelopmental performance. We hypothesized that ...cerebral hypoxia, detectable by near-infrared spectroscopy in the early postoperative period, would be related to later neurodevelopmental performance. Methods The study population was a sequential cohort of patients who had undergone stage 1 palliation of hypoplastic left heart syndrome under standard conditions, including neonatal perioperative monitoring with cerebral near-infrared spectroscopy, and who had undergone a neurodevelopmental assessment at age 4 to 5 years. The neonatal demographic and 48-hour perioperative hemodynamic parameters, including cerebral oxygen saturation, were tested for their relationship to 4 domains of neurodevelopmental performance, including visual-motor integration in childhood in univariate and multivariate models. The neurodevelopmental scores were classified as low if less than 85 (−1 standard deviation) and abnormal if less than 70 (−2 standard deviations). Results For the 51 patients in the surgical cohort, the early survival was 94%, the cumulative survival was 86%, and the neurodevelopmental assessment was completed by 21 (48%) of the survivors, without evidence of an ascertainment bias. At the test age of 56.3 ± 5.5 months, the composite neurodevelopmental index, constructed from equally weighted measures in 4 domains, was 97.6 ± 9.6, not different from the age-based norms, with 3 of 21 in the low range and none abnormal. The mean visual-motor integration was 93.4 ± 14, slightly less than the population norm ( P < .05), with 2 of 21 having low scores and 1 abnormal scores. In patients with low to abnormal visual-motor integration, the perioperative stage 1 palliation cerebral oxygenation saturation was significantly lower (63.6 ± 8.1 vs 67.8 ± 8.1, P < .05). Two patients had discrete embolic strokes after their initial hospitalization; the occurrence of late stroke reduced the visual-motor integration performance but was not related to the early cerebral oxygen saturation. Nonlinear relationships of cerebral oxygen saturation to the neurodevelopmental measures found cerebral oxygen saturation thresholds of 49% to 62%. The hours at a cerebral oxygen saturation less than 45% and 55% were related to low visual-motor integration and neurodevelopmental index scores in the univariate and multivariate models. A multivariate model of age and weight at stage 1 palliation, cerebral oxygen saturation, arterial oxygen saturation, cardiopulmonary bypass and deep hypothermic circulatory arrest times, and later stroke predicted visual-motor integration to an important degree (R2 = 0.53, P < .001). The actual and predicted visual-motor integration and neurodevelopmental index were normal when a cerebral oxygen saturation less than 45% and other risk conditions were avoided. Conclusions Neurodevelopmental performance was related to demographic, neonatal perioperative physiologic, and later factors. Perioperative cerebral oxygenation assessed by near-infrared spectroscopy can detect hypoxic-ischemic conditions associated with injury and reduced neurodevelopmental performance and was the most significant physiologic factor identified. These data suggest that efforts to avoid cerebral hypoxia are likely to improve the outcomes in this high-risk population.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Objective To describe preschool neurodevelopmental outcomes of children with complex congenital heart disease (CHD), who were evaluated as part of a longitudinal cardiac neurodevelopmental follow-up ...program, as recommended by the American Heart Association and the American Academy of Pediatrics, and identify predictors of neurodevelopmental outcomes in these children. Study design Children with CHD meeting the American Heart Association/American Academy of Pediatrics high-risk criteria for neurodevelopmental delay were evaluated at 4-5 years of age. Testing included standardized neuropsychological measures. Parents completed measures of child functioning. Scores were compared by group (single ventricle 1V; 2 ventricles 2V; CHD plus known genetic condition) to test norms and classified as: normal (within 1 SD of mean); at risk (1-2 SD from mean); and impaired (>2 SD from mean). Results Data on 102 patients were analyzed. Neurodevelopmental scores did not differ based on cardiac anatomy (1V vs 2V); both groups scored lower than norms on fine motor and adaptive behavior skills, but were within 1 SD of norms. Patients with genetic conditions scored significantly worse than 1V and 2V groups and test norms on most measures. Conclusions Children with CHD and genetic conditions are at greatest neurodevelopmental risk. Deficits in children with CHD without genetic conditions were mild and may not be detected without formal longitudinal testing. Parents and providers need additional education regarding the importance of developmental follow-up for children with CHD.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Children with congenital heart disease (CHD) are at risk for developmental delay (DD). Changes in cognitive, language, and motor skills in early childhood have not been described. We report the ...results of a structured approach using longitudinal testing to identify problems and ensure early intervention in accordance with published guidelines.
Bayley Scales of Infant Development, Third Edition, were used to assess cognitive, language, and motor skills in 99 children with CHD. Subjects were evaluated 3 to 6 times in the first 3 years of life. DD was defined as scores >1 SD below the population mean.
Cardiac anatomy was single ventricle (1V) in 34 subjects and 2 ventricles (2V) in 65. Medical comorbidities were present in 21% and genetic syndromes in 19%. Most subjects (75%) had DD in ≥1 area at ≥1 assessments. Subjects with 1V anatomy had equivalent outcomes to those with 2V. Cognitive and language scores declined in subjects with genetic syndromes but were stable and within the average range for subjects with 1V and 2V. Motor scores improved for subjects with 1V and 2V but remained low for those with genetic syndromes. In addition to age, need for supplemental tube feeding, longer cardiopulmonary bypass time, and shorter time since last hospitalization were significant predictors of developmental outcomes.
DDs in young children with CHD are both common and dynamic. Providers should encourage longitudinal surveillance for children with CHD because exposure to risk and prevalence of DD change over time.
Children with congenital heart disease are at risk for developmental delay. This study sought to identify early risk factors for abnormal developmental trajectories in children with congenital heart ...disease.
Children with congenital heart disease at high risk for developmental delay, without known genetic abnormality, and with ≥3 assessments by the use of the Bayley Scales of Infant and Toddler Development, Third Edition, were studied. Logistic regression was used to assess the impact of patient and clinical factors on cognitive, language, and motor score trajectories; classified as: average or improved if all scores were ≥85 (<1 standard deviation below the mean) or increased to ≥85 and never decreased; or abnormal if all scores were <85, fell to <85 and never improved, or fluctuated above and below 85. Data on 131 children with 527 Bayley Scales of Infant and Toddler Development, Third Edition assessments were analyzed. Subject age was 5.5 to 37.4 months. Overall, 56% had cognitive, language, and motor development in the average range. Delays occurred in single domains in 23%. Multiple domains were delayed in 21%. More cardiac surgeries, longer hospital stay, poorer linear growth, and tube feeding were associated with worse outcomes in all domains (P<0.05). In the multivariable model, the need for tube feeding was a risk factor for having an abnormal developmental trajectory (odds ratio, 5.1-7.9). Minority race and lack of private insurance had significant relationships with individual domains.
Longitudinal developmental surveillance identified early factors that can help quantify the risk of developmental delay over time. Strategies to improve modifiable factors and early therapeutic intervention can be targeted to children at highest risk.
Objective Infants who undergo Norwood stage 1 palliation (S1P) continue with high-risk circulation until stage 2 palliation (S2P). Routine care during the interstage period is associated with 10% to ...20% mortality. This report illustrates the sustained reduction of interstage mortality over 10 years associated with use of home monitoring. Methods Daily monitoring of oxygen saturation and weight was done for all patients discharged to home after S1P. Notification of the care team occurred for oxygen saturation <75% or >90%, weight gain < 20 g over 3 days, weight loss > 30 g, or intake < 100 cc/kg/d. Breach of these criteria marked an interstage event. Interstage outcomes are reported. Patient characteristics and perioperative variables were compared between patients with and without interstage events. Results Over 10 years, 157 patients were discharged after S1P with home monitoring. Interstage survival was 98%. Breach of home criteria occurred in 59% (93 out of 157), with violation of oxygen saturation <75% the most common event. Patient characteristics, operative data, and early postoperative morbidity did not differ between patients with and without events. Conclusions Home monitoring after S1P is associated with excellent interstage survival. Although a breach of monitoring criteria occurred in more than half of patients, our analysis failed to identify independent predictors of interstage events. Analysis of variables predicting mortality could not be assessed due to the low frequency of death in this cohort. Failure to identify specific variables for interstage events suggests that home monitoring, as part of an interstage surveillance program, should be applied to all S1P hospital survivors.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Background Advances in management of the Fontan patient include interval superior cavopulmonary shunt, total cavopulmonary connection, either lateral tunnel or extracardiac conduit, and the use of a ...fenestration. Coincident with these improvements, Fontan palliation has been applied to a wider ranger of anatomic subgroups. Methods A cross-sectional analysis of 256 consecutive patients undergoing a total cavopulmonary connection Fontan after superior cavopulmonary shunt between January 1, 1994, and June 30, 2007 were studied. Fenestration was used selectively. Fontan failure was defined as death, transplant, or takedown. Event-free survival was defined as freedom from death, transplant, Fontan takedown, functional class III to IV, pacemaker, antiarrhythmic medication, protein-losing enteropathy, stroke, or thrombus. Results Survival was 97% ± 1%, 96% ± 1%, and 94% ± 2%, respectively, at 1, 5, and 10 years. Event-free survival was 96% ± 1%, 87% ± 3%, and 64% ± 6%, respectively, at 1, 5, and 10 years. Factors predicting worse event-free survival included longer cross-clamp time ( p = 0.003), fenestration ( p = 0.014), and longer hospital length of stay ( p = 0.016). Ventricular morphology did not predict outcome. Left ventricle (n = 113, 44%) versus right ventricle (n = 142, 56%) failure-free survival (death, transplant, or Fontan takedown) at 10 years was 92% ± 4% versus 91% ± 3%, respectively ( p = 0.19). Left ventricle versus right ventricle event-free survival at 10 years was 75% ± 7% versus 67% ± 9%, respectively ( p > 0.1). Conclusions Survival for patients undergoing a completion Fontan in the current era is excellent, but patients remain at risk for morbid events. In the intermediate follow-up period, we could not identify a difference in outcome between dominant left and right ventricle morphology.
In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, ...survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary ...bypass.
In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean PDI (74 ± 19) and MDI (89 ± 18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R(2)=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R(2)=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy.
Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room.
URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.
PDF
