Predictive value of General Movements Assessment for developmental delay at 18 months in children with complex congenital heart disease.

BACKGROUND: Infants with complex congenital heart disease are at increased risk of impaired fetal brain growth, brain injury, and developmental impairments. The General Movement Assessment (GMA) is a valid and reliable tool to predict cerebral palsy (CP), especially in preterm infants. Predictive properties of the GMA in infants with complex congenital heart disease (CCHD) are unknown. AIM: To evaluate predictive properties of the GMA to predict developmental outcomes, including cerebral palsy (CP), at 18-months corrected age (CA) in children with CCHD undergoing heart surgery in the first month of life. METHODS: A prospective cohort of 56 infants with CCHD (35 males, 21 females) was assessed with GMA at writhing age (0-6 weeks CA) and fidgety age (7-17 weeks CA) and the Bayley Scales of Infant Development at 18 months. GMA focused on markedly reduced GM-variation and complexity (definitely abnormal (DA) GM-complexity) and fidgety movements. Predictive values of GMA for specific cognitive, language and motor delay (composite scores <85th percentile) and general developmental delay (delay in all domains) were calculated at 18 months. RESULTS: At fidgety age, all infants had fidgety movements and no child was diagnosed with CP. DA GM-complexity at fidgety age predicted general developmental delay at 18 months (71 % sensitivity, 90 % specificity), but predicted specific developmental delay less robustly. DA GM-complexity at writhing age did not predict developmental delay, nor did it improve prediction based on DA GM-complexity at fidgety age. CONCLUSIONS: In infants with CCHD and fidgety movements, DA GM-complexity at fidgety age predicted general developmental delay.

Methylprednisolone for Heart Surgery in Infants - A Randomized, Controlled Trial.

BACKGROUND: Although perioperative prophylactic glucocorticoids have been used for decades, whether they improve outcomes in infants after heart surgery with cardiopulmonary bypass is unknown. METHODS: We conducted a multicenter, prospective, randomized, placebo-controlled, registry-based trial involving infants (<1 year of age) undergoing heart surgery with cardiopulmonary bypass at 24 sites participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database. Registry data were used in the evaluation of outcomes. The infants were randomly assigned to receive prophylactic methylprednisolone (30 mg per kilogram of body weight) or placebo, which was administered into the cardiopulmonary-bypass pump-priming fluid. The primary end point was a ranked composite of death, heart transplantation, or any of 13 major complications. Patients without any of these events were assigned a ranked outcome based on postoperative length of stay. In the primary analysis, the ranked outcomes were compared between the trial groups with the use of odds ratios adjusted for prespecified risk factors. Secondary analyses included an unadjusted odds ratio, a win ratio, and safety outcomes. RESULTS: A total of 1263 infants underwent randomization, of whom 1200 received either methylprednisolone (599 infants) or placebo (601 infants). The likelihood of a worse outcome did not differ significantly between the methylprednisolone group and the placebo group (adjusted odds ratio, 0.86; 95% confidence interval [CI], 0.71 to 1.05; P = 0.14). Secondary analyses (unadjusted for risk factors) showed an odds ratio for a worse outcome of 0.82 (95% CI, 0.67 to 1.00) and a win ratio of 1.15 (95% CI, 1.00 to 1.32) in the methylprednisolone group as compared with the placebo group, findings suggestive of a benefit with methylprednisolone; however, patients in the methylprednisolone group were more likely than those in the placebo group to receive postoperative insulin for hyperglycemia (19.0% vs. 6.7%, P<0.001). CONCLUSIONS: Among infants undergoing surgery with cardiopulmonary bypass, prophylactic use of methylprednisolone did not significantly reduce the likelihood of a worse outcome in an adjusted analysis and was associated with postoperative development of hyperglycemia warranting insulin in a higher percentage of infants than placebo. (Funded by the National Center for Advancing Translational Sciences and others; STRESS ClinicalTrials.gov number, NCT03229538.).

Developmental outcomes after early surgery for complex congenital heart disease: a systematic review and meta-analysis.

AIM: (1) To systematically review the literature on developmental outcomes from infancy to adolescence of children with complex congenital heart disease (CHD) who underwent early surgery; (2) to run a meta-regression analysis on the Bayley Scales of Infant Development, Second Edition Mental Developmental Index and Psychomotor Developmental Index (PDI) of infants up to 24 months and IQs of preschool-aged children to adolescents; (3) to assess associations between perioperative risk factors and outcomes. METHOD: We searched pertinent literature (January 1990 to January 2019) in PubMed, Embase, CINAHL, and PsycINFO. Selection criteria included infants with complex CHD who had primary surgery within the first 9 weeks of life. Methodological quality, including risk of bias and internal validity, were assessed. RESULTS: In total, 185 papers met the inclusion criteria; the 100 with high to moderate methodological quality were analysed in detail. Substantial heterogeneity in the group with CHD and in methodology existed. The outcome of infants with single-ventricle CHD was inferior to those with two-ventricle CHD (respectively: average scores for PDI 77 and 88; intelligence scores 92 and 98). Perioperative risk factors were inconsistently associated with developmental outcomes. INTERPRETATION: The literature on children undergoing surgery in early infancy suggests that infants with a single ventricle are at highest risk of adverse developmental outcomes.

The quality of general movements in infants with complex congenital heart disease undergoing surgery in the neonatal period.

BACKGROUND: Advances in diagnostic technologies, surgical management, and perioperative care have increased survival for neonates with complex congenital heart disease (CCHD). The success of these advances exposed a heightened risk of brain injury and developmental disabilities. The General Movements Assessment, a non-invasive method, may detect early neurodevelopmental impairments in high-risk infants. AIMS: To examine whether infants with CCHD undergoing neonatal surgery have higher prevalence of atypical general movements (GMs) than a reference group, and whether single ventricle physiology with systemic oxygen saturations <90% increases risk for atypical GMs. METHODS: Serial General Movements Assessment (GMA) in a cohort of infants with CCHD (n = 74) at writhing (term-6 weeks) and fidgety (7-17 weeks) GM-age. GMA focused on the presence of definitely abnormal GM-complexity and absent fidgety movements. Single GMAs at 3 months were available from a reference sample of Dutch infants (n = 300). Regression analyses examined relationships between cardiac characteristics and definitely abnormal GM-complexity. RESULTS: Higher prevalence of definitely abnormal GM-complexity in infants with CCHD compared to reference infants (adjusted OR 5.938, 95% CI 2.423-14.355), single ventricle CCHD increased the risk. Occurrence of absent fidgety movements was similar in infants with CCHD and reference infants (adjusted OR 0.475, 95% CI 0.058-3.876). Systemic postoperative oxygen saturations <90% was associated with higher risk of definitely abnormal GM-complexity at fidgety (adjusted OR 16.445 95% CI 1.149-235.281), not at writhing age. CONCLUSIONS: Infants with CCHD, especially those with single ventricle CCHD, are at increased risk of definitely abnormal GM-complexity. GMA at fidgety age is recommended.

Prevalence of Sensorineural Hearing Loss in Children with Palliated or Repaired Congenital Heart Disease.

Background Children with congenital heart disease (CHD) are at increased risk of neurodevelopmental deficits, and the presence of sensorineural hearing loss (SNHL) may further lead to poor language skills acquisition and speech delays. Prevalence of SNHL in the general pediatric population is estimated to be 0.2% at birth to 0.35% during adolescence. Very few studies have attempted to estimate SNHL prevalence in children who have undergone congenital heart surgery. Methods This retrospective study aimed to estimate SNHL prevalence in children who underwent congenital heart surgery in our institution and were followed up in our high-risk pediatric cardiology clinics for four years from 2009 to 2013. Data were collected on demographics, preoperative variables, surgical variables, and post-operative variables. Results SNHL prevalence in asymptomatic, palliated/repaired CHD patients followed in our high-risk clinics and undergoing routine surveillance was 11.6% (20 of 172 patients with hearing impairment). SNHL prevalence was not statistically higher in single-ventricle patients (17.2%) compared to biventricular patients (14.7%). Inotropic score in the first 24 hours of postoperative period (p=0.05), lowest arterial PaO2 (p=0.003), duration of Lasix drip (p=0), and bolus dose in days (p=0.03) were all found to be statistically significant in the hearing-impaired group. However, using logistic regression, we identified no statistically significant predictors for hearing loss. Conclusion The results suggest the need for routine audiology screening of all patients with complex CHD, especially those who have undergone neonatal cardiac repair/palliation at less than one year of age, irrespective of risk factors.

Power of a Learning Network in Congenital Heart Disease.

BACKGROUND: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) formed to improve outcomes in infants with hypoplastic left heart syndrome. The collaborative sought to (1) decrease mortality, (2) reduce growth failure, and (3) reduce hospital readmissions due to major medical problems during the interstage period between discharge following stage 1 palliation (S1P) and admission for stage 2 palliation (S2P). METHODS: The NPC-QIC is a learning network, coproduced by parents and clinicians, of 65 pediatric cardiology centers that contribute clinical data on care processes and outcomes to a shared registry. The adapted Breakthrough Series Model structure brings teams together regularly to review data, share lessons, and plan improvements. Outcomes are monitored using statistical process control methods. RESULTS: Between 2008 and 2016, interstage mortality decreased by >40%, from 9.5% to 5.3%. Identification and use of a nutrition bundle led to improved infant growth, with a 28% reduction in interstage growth failure. The rate of serious hospital readmissions was low and did not significantly change. Importantly, a formed partnership with the parent group Sisters by Heart fostered the coproduction of tools and strategies and an emphasis on data transparency and outcomes. CONCLUSIONS: The NPC-QIC's initial efforts led to improvements in interstage growth and mortality. The NPC-QIC has modeled the use of data for improvement and research, the value of coproduction with parents, and the concept "all teach, all learn," demonstrating the power of the learning network model.

Utilization of Neurally Adjusted Ventilatory Assist (NAVA) Mode in Infants and Children Undergoing Congenital Heart Surgery: A Retrospective Review.

We assessed the feasibility and the impact of NAVA compared to conventional modes of mechanical ventilation in ventilatory and gas exchange parameters in post-operative children with congenital heart disease. Infants and children (age < 18 years) that underwent congenital heart surgery were enrolled. Patients were ventilated with conventional synchronized intermittent mechanical ventilation (SIMV) and subsequently transitioned to NAVA during their cardiovascular intensive care unit (CVICU) stay. The ventilatory and gas exchange parameters for the 24 h pre- and post-transition to NAVA were compared. Additional parameters assessed included pain scores and sedation requirements. Eighty-one patients met inclusion criteria with a median age of 21 days (interquartile range 13 days-2 months). The majority of patients enrolled (75.3%) had complex congenital heart disease with high surgical severity scores. The transition to NAVA was tolerated by all patients without complications. The mean peak inspiratory pressure (PIP) was 1.8 cm H2O lower (p < 0.001) and mean airway pressure (Paw) was 0.5 cm H2O lower (p = 0.009) on NAVA compared to conventional modes of mechanical ventilation. There was no significant difference in patients' respiratory rate, tidal volume, arterial pH, pCO2, and lactate levels between the two modes of ventilation. There was a decreased sedation requirement during the time of NAVA ventilation. Comfort scores did not differ significantly with ventilator mode change. We concluded that NAVA is safe and well-tolerated mode of mechanical ventilation for our cohort of patients after congenital heart surgery. Compared to conventional ventilation there was a statistically significant decrease in PIP and Paw on NAVA.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.

Significance of low peak Doppler velocity in the proximal sano conduit in hypoplastic left heart syndrome.

BACKGROUND: The Sano modification of the Norwood operation is a well-established first step palliation for hypoplastic left heart syndrome (HLHS). Theoretically, the first point of resistance to pulmonary flow should be in the proximal Sano, generating high Doppler flow velocity. Paradoxically, however, some patients have low gradients in the proximal Sano conduit. The objective of this study was to determine the hemodynamic and anatomic significance of low proximal Sano Doppler flow velocity and its clinical implications. METHODS: Doppler-derived peak gradients in the proximal Sano conduits were measured in HLHS patients after Norwood-Sano surgery over a 4-year period and confirmed by cardiac catheterization within 2 to 4 weeks. Clinical outcomes of patients with proximal Sano gradients of 30 mm Hg or less (group 1) were compared with patients whose gradient was greater than 30 mm Hg (group 2). RESULTS: Of the 53 patients, 21 (40%) belonged to group 1. Patients in group 1 had smaller ostial right and left pulmonary artery (PA) diameter (3.2 ± 1.2 mm versus 4.5 ± 1.8 mm, p = 0.03; and 3.4 ± 1.2 mm versus 5.6 ± 2.1 mm, p = 0.01) when compared with patients in group 2. Patients (7 of 10) who needed either balloon angioplasty of a distal Sano or proximal branch PA were from group 1 (p = 0.01). Patients in group 1 had higher rates of unintended PA interventions (33% versus 9%) and complications (48% versus 16%) compared with group 2. CONCLUSIONS: Low peak Doppler flow velocity in the proximal Sano correlates with the presence of either distal Sano stenosis or proximal branch PA stenosis. These patients require close follow-up in the interstage period and may need earlier intervention.

Transesophageal echocardiography in critically ill acute postoperative infants: comparison of AcuNav intracardiac echocardiographic and microTEE miniaturized transducers.

BACKGROUND: Multiple barriers to transthoracic echocardiography are present in critically ill infants immediately after surgery. Transesophageal echocardiography (TEE) is sometimes needed to obtain specific important information that transthoracic echocardiography fails to demonstrate. Formerly, the investigators used the AcuNav intracardiac echocardiographic (ICE) intravascular ultrasound transducer (8 Fr, 2.5 mm, 64-element crystal array, multifrequency [5.5-10 MHz], single longitudinal plane, linear phased array [Siemens Medical Solutions USA, Inc., Mountain View, CA]). Recently, the investigators have also used the microTEE transducer (8-mm transducer tip, 5.2-mm shaft, multifrequency [3-8 MHz], multiplane phased array, 32-element probe [Philips Medical Systems, Andover, MA]). Both transducers have two-dimensional, M-mode, color Doppler, and pulsed-wave and continuous-wave Doppler capabilities. The aim of this study was to compare the efficacy, safety, ease of insertion, capabilities, utilization, and cost of the AcuNav ICE transducer versus those of the microTEE transducer. METHODS: A retrospective review of all 50 postoperative critically ill infants who underwent TEE using the AcuNav and microTEE in the past 5 years was conducted. TEE was performed as ordered by the attending physician to answer a specific question not answered by transthoracic echocardiography. RESULTS: In all cases, the clinical information sought was obtained. The AcuNav ICE transducer was safe, easy to insert through the transnasal route, and did not require paralysis; however, it had a limited number of echocardiographic views and had greater sterilization cost. The microTEE transducer had greater echocardiographic capabilities and lower sterilization cost; however, it was slightly more difficult to insert, had a few manageable complications, and required more sedation and paralysis. CONCLUSIONS: TEE in this setting has increased because of demonstrated efficacy and safety. Both the AcuNav ICE and microTEE transducers are useful and effective in this critical clinical scenario.

Coronary artery-to-pulmonary artery fistula in a case of pulmonary atresia with ventricular septal defect.

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.

Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation.

BACKGROUND: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. METHODS: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. RESULTS: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). CONCLUSIONS: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.