High Acuity Therapy Variation Across Pediatric Acute Care Cardiology Units: Results from the Pediatric Acute Care Cardiology Collaborative Hospital Surveys.

We utilized the multicenter Pediatric Acute Care Cardiology Collaborative (PAC3) 2017 and 2019 surveys to describe practice variation in therapy availability and changes over a 2-year period. A high acuity therapies (ATs) score was derived (1 point per positive response) from 44 survey questions and scores were compared to center surgical volume. Of 31 centers that completed the 2017 survey, 26 also completed the 2019 survey. Scores ranged from 11 to 34 in 2017 and 11 to 35 in 2019. AT scores in 2019 were not statistically different from 2017 scores (29/44, IQR 27-32.5 vs. 29.5/44, IQR 27-31, p = 0.9). In 2019, more centers reported initiation of continuous positive airway pressure (CPAP) and Bi-level positive airway pressure (BiPAP) in Acute Care Cardiology Unit (ACCU) (19/26 vs. 4/26, p < 0.001) and permitting continuous CPAP/BiPAP (22/26 vs. 14/26, p = 0.034) compared to 2017. Scores in both survey years were significantly higher in the highest surgical volume group compared to the lowest, 33 ± 1.5 versus 25 ± 8.5, p = 0.046 and 32 ± 1.7 versus 23 ± 5.5, p = 0.009, respectively. Variation in therapy within the ACCUs participating in PAC3 presents an opportunity for shared learning across the collaborative. Experience with PAC3 was associated with increasing available respiratory therapies from 2017 to 2019. Whether AT scores impact the quality and outcomes of pediatric acute cardiac care will be the subject of further investigation using a comprehensive registry launched in early 2019.

Association of Interstage Home Monitoring With Mortality, Readmissions, and Weight Gain: A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative.

BACKGROUND: Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects. METHODS AND RESULTS: We performed a retrospective cohort study using prospectively collected data from the National Pediatric Cardiology Quality Improvement Collaborative from 2008 to 2012. We compared interstage mortality, unscheduled readmissions, and change in weight-for-age Z score for various home monitoring strategies of oxygen saturation (n=494) or weight (n=472), adjusting for sex, syndrome, tricuspid regurgitation, arch obstruction, and shunt type. Overall interstage mortality was 8.1%, and 47% had ≥1 unscheduled readmission. We did not find any associations of home oxygen saturation or weight monitoring with mortality or readmission. Although there was no difference in weight-for-age Z score for daily (0.33±0.12) versus weekly (0.34±0.18, P=0.98) weight monitoring, daily home weight monitoring was superior to no home weight monitoring (-0.15±0.18; P<0.01). CONCLUSIONS: Home weight monitoring is associated with improved weight gain during the interstage period, but we did not find any benefits in other clinical outcomes for either home oxygen saturation monitoring or home weight monitoring.

Is Clinic Visit Frequency Associated with Weight Gain During the Interstage Period? A Report from the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative (JCCHD-NPCQIC).

Growth problems are prevalent among infants with congenital heart disease. We sought to determine whether frequency of outpatient clinic visits correlated with weight gain in patients with hypoplastic left heart syndrome or variant during the interstage period between discharge from stage I palliation and presentation for stage II palliation (SIIP). Using prospectively collected data from the JCCHD NPC-QIC database from June 2008 to July 2013, we performed a retrospective cohort study assessing the association of days between clinic visits (DBV) with the change in weight-for-age z-score (WAZ) during the interstage period. Eligible subjects were those who survived to a SIIP performed at <270 days of age and had at least two outpatient clinic visits. There were 561 patients from 49 centers who fulfilled inclusion criteria. The average interstage change in WAZ was +0.22. The mean number of DBV was 16.1 days, and the average number of clinic visits was six. There was no correlation of change in WAZ with either DBV (r = 0.02, P = 0.62) or the number of visits (r = 0.03, P = 0.44). Subjects within this cohort are seen about every 2 weeks averaged over the interstage period. There is no correlation between interstage visit frequency and change in WAZ in this patient population. Further research is needed to describe differences in visit frequency as the patient progresses through the interstage period and to elucidate whether patient factors such as growth velocity are influencing visit frequency. The optimal visit frequency remains unknown.

Variation in feeding practices following the Norwood procedure.

OBJECTIVES: To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II. STUDY DESIGN: From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed. RESULTS: Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P < .01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications (R(2) = 0.65, P < .01). After adjusting for site, mean pre-stage II weight-for-age z-score was significantly higher in the oral-only group (-1.4) vs the N-tube-only (-2.2) and G-tube-only (-2.1) groups (P = .04 and .02, respectively). CONCLUSIONS: Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre-stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.

Severe aortopathy due to fibulin-4 deficiency: molecular insights, surgical strategy, and a review of the literature.

UNLABELLED: Mutations in the EFEMP2 (alias FBLN4) gene, which encodes the extracellular matrix protein fibulin-4, lead to severe aortopathy with aneurysm formation and vascular tortuosity. The disease phenotype, termed autosomal recessive cutis laxa type 1B (ARCL 1B), is rare among heritable connective tissue diseases but becomes more likely when noting family consanguinity and loose, inelastic skin in the patient. Our patient presented with an intercurrent illness exacerbating upper airway obstruction due to compression from a large aortic aneurysm. Genetic testing eventually revealed the causative mutation. She was initially treated with an angiotensin II receptor blocker and beta-blocker and eventually underwent total thoracic aortic replacement via a two-stage elephant trunk-type procedure. She recovered well and is currently asymptomatic but will require lifetime follow-up due to residual vascular tortuosity and aneurysm risk. CONCLUSION: Better understanding of the importance of transforming growth factor beta signaling in the pathophysiology of aortopathies such as ARCL 1B has led to targeted medical therapies. Specific surgical techniques can lead to optimal outcomes in these patients.

Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic-to-pulmonary artery shunt, including Norwood palliation, at Children's Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization's Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was -2.0 (IQR -2.7 to -1.2) representing an overall median WAZ score decrease of -1.3 (IQR -1.7 to -0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.

Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt.

BACKGROUND: On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process. METHODS: In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score. RESULTS: Single-ventricle patients had a higher weight-for-age z-score at neonatal surgery than two-ventricle patients (-0.4 ± 1.0 compared with -1.2 ± 0.9, with p < 0.001), but they had a greater drop in the weight-for-age z-score to the first outpatient visit (-1.1 ± 0.7 compared with -0.8 ± 0.7, with p = 0.02). After the first outpatient visit, the weight-for-age z-score was not significantly different between single-ventricle and two-ventricle patients. From multivariate analysis, a lower number of nutritional interventions by cardiologists was significantly associated with poor growth (p = 0.03). Poor growth was not associated with race, use of feeding tube, exclusive formula use, or proximity to surgical centre. CONCLUSION: The significant drop in the weight-for-age z-score from neonatal surgery to first outpatient visit suggests that these patients may receive inadequate nutrition. The poorest performers received the least number of outpatient changes to their diet. This finding underscores the critical role of the primary cardiologist in optimising weight gain through adjustments in nutrition.

Coarctation of the aorta in the setting of tetralogy of Fallot: an uncommon cause of myocardial dysfunction.

The combination of both right and left heart obstruction has only rarely been described in the medical literature. We present three cases of coarctation of the aorta in patients with variants of tetralogy of Fallot and hypothesise that this condition may be more common than previously suspected and could represent a hidden cause of morbidity in patients with pulmonary atresia/ventricular septal defect.

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

Intensive Care Unit and Acute Care Unit Length of Stay After Congenital Heart Surgery.

BACKGROUND: Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals after congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on postoperative LOS. METHODS: Hospitalizations for congenital heart surgery within the Pediatric Cardiac Critical Care Consortium (PC4) registry (August 2014 to February 2018) were included. Models were developed for ICU, ACU, and postoperative LOS by adjusting for differences in case-mix across hospitals. PC4 hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC3) were also surveyed on ACU organizational factors and practice patterns. RESULTS: Overall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Postperative LOS appeared to be most closely related to ICU LOS; 75% (6 of 8) of hospitals with shorter than expected postoperative LOS also had shorter than expected ICU LOS. A clear relationship between postoperative and ACU LOS was not observed. Hospitals with an ACU able to provide higher-acuity care as indexed according to the PAC3 survey were more likely to have shorter postoperative LOS (P < .01). CONCLUSIONS: For hospitals that achieve shorter than expected postoperative LOS after congenital heart surgery, ICU LOS appears to be the primary driver. Higher-acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing postoperative LOS.

Myocarditis in the pediatric population: A review.

Myocarditis has a variable clinical presentation and there is still debate regarding accurate diagnostic criteria. Adding to the controversy surrounding this diagnosis, there is no clear consensus for the treatment or ongoing follow-up of patients with myocarditis. All of this makes the diagnosis and management of myocarditis a particular challenge in the pediatric population. Furthermore, the literature with respect to this topic is dynamic and ever-changing. In this review article, we aim to review and summarize the common clinical presentations of myocarditis, along with the latest recommendations for diagnostic criteria, treatment, and follow-up of patients with myocarditis.

Variation in care practices across pediatric acute care cardiology units: Results of the Pediatric Acute Care Cardiology Collaborative (PAC3 ) hospital survey.

BACKGROUND: The Pediatric Acute Care Cardiology Collaborative (PAC3 ) was established in 2014 to improve the quality, value, and experience of hospital-based cardiac acute care outside of the intensive care unit. An initial PAC3 project was a comprehensive survey to understand unit structure, practices, and resource utilization across the collaborative. This report aims to describe the previously unknown degree of practice variation across member institutions. METHODS: A 126-stem question survey was developed with a total of 412 possible response fields across nine domains including demographics, staffing, available resources and therapies, and standard care practices. Five supplemental questions addressed surgical case volume and number of cardiac acute care unit (CACU) admissions. Responses were recorded and stored in Research Electronic Data Capture (REDCap). RESULTS: Surveys were completed by 31 out of 34 centers (91%) with minimal incomplete fields. A majority (61%) of centers have a single dedicated CACU, which is contiguous or adjacent to the intensive care unit in 48%. A nurse staffing ratio of 3:1 is most common (71%) and most (84%) centers employed a resource nurse. Centralized wireless rhythm monitoring is used in 84% of centers with 54% staffed continuously. There was significant variation in the use of noninvasive respiratory support, vasoactive infusions, and ventricular assist devices across the collaborative. Approximately half of the surveyed centers had lesion-specific postoperative pathways and approximately two-thirds had protocols for single-ventricle patients. CONCLUSIONS: The PAC3 hospital survey is the most comprehensive description of systems and care practices unique to CACUs to date. There exists considerable heterogeneity among unit composition and variation in care practices. These variations may allow for identification of best practices and improved quality of care for patients.

Factors Associated With Interstage Mortality Following Neonatal Single Ventricle Palliation.

BACKGROUND: Several advances have led to improved hospital survival following neonatal palliation (NP) of single ventricle (SV) anomalies. Nonetheless, a number of patients continue to suffer from interstage mortality (ISM) prior to subsequent Glenn. We aim to study patients' characteristics and anatomic, surgical, and clinical details associated with ISM. METHODS: A total of 453 SV neonates survived to hospital discharge following NP. Competing risk analysis modeled events after NP (Glenn, transplantation, or death) and examined variables associated with ISM. RESULTS: Competing risk analysis showed that one year following NP, 10% of patients had died, 87% had progressed to Glenn, 1% had received heart transplantation, and 2% were alive without subsequent surgery. On multivariable analysis, factors associated with ISM were as follows: weight ≤2.5 kg (hazard ratio, HR = 2.4 [1.2-4.6], P = .013), premature birth ≤36 weeks (HR = 2.0 [1.0-4.0], P = .05), genetic syndromes (HR = 3.2 [1.7-6.1], P < .001), unplanned cardiac reoperation (HR = 2.1 [1.0-4.4], P = .05), and prolonged intensive care unit (ICU) stay >30 days following NP (HR = 2.5 [1.4-4.5], P < .001). Palliative surgery type (shunt, Norwood, band) was not associated with ISM, although aortopulmonary shunt circulation after Norwood was (HR = 5.4 [1.5-19.2] P = .01). Of interest, underlying SV anatomy was not associated with ISM (HR = 1.1 [0.6-2.2], P = .749). CONCLUSIONS: In our series, ISM following NP occurred in 10% of hospital survivors. As opposed to hospital death, underlying SV anomaly was not associated with ISM. Conversely, several patient factors (prematurity, low weight, and genetic syndromes) and clinical factors (unplanned reoperation and prolonged ICU stay following NP) were associated with ISM. Vigilant outpatient management that is individualized to specific clinical and social needs, taking into account all associated factors, is warranted to improve survival in high-risk patients.

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

OBJECTIVES: The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. METHODS: Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes. RESULTS: SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041]. CONCLUSIONS: Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

Results of palliation with an initial modified Blalock-Taussig shunt in neonates with single ventricle anomalies associated with restrictive pulmonary blood flow.

BACKGROUND: We report our current-era results after placement of a modified Blalock-Taussig shunt (mBTS) as the first surgery in the multistage palliation strategy of neonates born with single ventricle (SV) anomalies associated with restrictive pulmonary blood flow. METHODS: Between 2002 and 2012, 174 neonates with SV underwent mBTS. Competing risks analysis modeled events after mBTS (death or transplantation, transition to Glenn) and subsequently after Glenn (death or transplantation, transition to Fontan) and examined risk factors affecting outcomes. RESULTS: Competing risks analysis showed that 2 years after mBTS 27% of patients had died or received transplantation and 73% had undergone Glenn. Five years after Glenn, 6% had died, 73% had undergone Fontan, and 21% were alive awaiting Fontan. Overall survival 8 years after mBTS was 68%. On multivariable analysis, risk factors for mortality were increased shunt size to weight ratio (hazard ratio [HR]:1.2 per 0.1 ratio increase [1.0 to 1.4], p = 0.015), postoperative extracorporeal membrane oxygenation (HR: 4.0 [2.2 to 7.4], p < 0.001), chromosomal and extracardiac malformations (HR: 2.5 [1.3 to 5.0], p = 0.008), cardiopulmonary bypass use (HR: 2.5 [1.3 to 4.5], p = 0.004), and underlying pulmonary atresia with intact ventricular septum (PAIVS) or atrial isomerism (HR: 2.1 [1.1 to 4.2], p = 0.035). CONCLUSIONS: Palliation outcomes with mBTS in SV patients trail behind the generally improved results of congenital heart surgery. Several anatomic and patient-related factors continue to affect survival. Mortality is high in low weight neonates in whom a balance between overcirculation due to large shunt and low pulmonary blood flow and risk of occlusion due to small shunt is difficult to attain. Patients with chromosomal and extracardiac malformations, atrial isomerism, and PAIVS continue to be the most challenging and are associated with higher hospital and interstage mortality.

Is there a benefit to postoperative fluid restriction following infant surgery?

OBJECTIVE: Fluid restriction is often employed immediately following cardiac surgery in children. The goal of this approach is to achieve an early negative fluid balance, which theoretically should lead to less interstitial edema and earlier extubation. The purpose of this study was to determine whether time to negative fluid balance in infants after undergoing systemic-to-pulmonary artery shunt palliation impacts duration of mechanical ventilation and hospital length of stay. DESIGN: This is a retrospective study of neonates who underwent a modified systemic-to-pulmonary artery shunt at a single institution. SETTING: University hospital pediatric cardiac intensive care unit (CICU). PATIENTS: Neonates who underwent a modified systemic-to-pulmonary artery shunt between January 1, 2009 and June 1, 2011. OUTCOME MEASURES: Information collected included time to negative fluid balance (in hours), CICU and hospital length of stay (in days), and the number of patients who had delayed sternal closure and/or underwent cardiopulmonary bypass. RESULTS: Data were available for 65 subjects. Median fluid administration in the 24 hours postoperatively was 43.9 cc/kg/day (interquartile range: 32.9-61.0). Mean time to negative fluid balance was 25.0 ± 12.8 hours. Time to negative fluid balance was not associated with time to extubation, CICU and hospital length of stay, or change in weight-for-age z-score at intensive care unit discharge. CONCLUSION: Time to negative fluid balance is not associated with duration of mechanical ventilation, CICU, and hospital length of stay in patients after undergoing systemic-to-pulmonary artery shunt palliation. The utility of a restricted fluid strategy immediately following infant heart surgery is questionable.

Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat.

PURPOSE: Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management. METHODS: After IRB approval, a retrospective review of all patients treated for HS at a large tertiary care children's hospital between January 2008 and June 2012 was performed. For the purpose of this paper, malrotation was defined as an operative note that described the presence of Ladd's bands and a narrow mesentery. RESULTS: Thirty-eight patients with HS were identified, including 18 who underwent abdominal exploration. Left atrial isomerisation (LAI) was identified in 13 individuals, and right atrial isomerisation (RAI) was noted in 25. The rate of surgical intervention did not vary between the 2 groups (54%). Malrotation was found in 8 patients: one with LAI and 7 with RAI. This difference in incidence was statistically significant (p=0.04). CONCLUSION: These data suggest that the direction of atrial isomerisation influences the likelihood of true malrotation, where RAI patients are more likely to be malrotated. Given the inherent risk of surgery on this medically fragile patient population, surgeons should consider expectant management for asymptomatic LAI patients.

Longitudinal assessment of growth in hypoplastic left heart syndrome: results from the single ventricle reconstruction trial.

BACKGROUND: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. METHODS AND RESULTS: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients <37 weeks gestation (N=498). We determined length-for-age z score (LAZ) and weight-for-age z score (WAZ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth (P<0.001, P=0.05, respectively) and age 3 years (P<0.001 each). The largest decrease in WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were <-2 in 6% and 18%, respectively. Factors associated with change in WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock-Taussig shunt had a greater decline in WAZ than those with a right ventricle-pulmonary artery shunt (P=0.002). CONCLUSIONS: WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (>2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient- and stage-specific targets. CLINICAL TRIAL REGISTRATION URL: http://clinicaltrials.gov/. Unique identifier: NCT00115934.

Association of feeding modality with interstage mortality after single-ventricle palliation.

OBJECTIVE: Interstage mortality has been reported in 10% to 25% of hospital survivors after single-ventricle palliation. The purpose of this study was to examine the impact of feeding modality at discharge after single-ventricle palliation on interstage mortality. METHODS: We conducted a retrospective review of all neonates undergoing single-ventricle palliation from January 2003 to January 2010. A total of 334 patients (90%) survived to hospital discharge, comprising the study group. Preoperative, operative, and postoperative variables were examined, including feeding method at discharge. Multivariate Poisson regression models were constructed to estimate the relative risk of interstage mortality. RESULTS: Of 334 patients, 56 (17%) underwent gastrostomy tube ± Nissen. There was a statistically significant increase in interstage mortality for patients who underwent gastrostomy tube ± Nissen compared with patients who did not (relative risk, 2.38; 95% confidence interval, 1.05-5.40; P = .04]). Of the 278 patients who were not fed via a gastrostomy tube ± Nissen, 190 (68%) were fed with nasogastric feedings and 88 (32%) were fed entirely by mouth. There was no difference in interstage mortality between these 2 groups (relative risk, 0.92; 95% confidence interval, 0.31-2.73; P = .89). CONCLUSIONS: Neonates undergoing single-ventricle palliation who require gastrostomy tube ± Nissen are at an increased risk of interstage mortality. The need for gastrostomy tube ± Nissen in this population may be a marker for other unmeasured comorbidities that place them at an increased risk of interstage mortality. Discharge with nasogastric feeds does not increase the risk of interstage mortality.

Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial.

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.