Jeffrey B. Anderson

Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle

OBJECTIVE Poor growth has been described in infants with a single ventricle; however, little is known regarding its effect on surgical outcomes. We sought to assess the effect of nutritional status at the time of the bidirectional Glenn procedure on short-term outcomes. METHODS We performed a retrospective case series of children who underwent the bidirectional Glenn procedure at our institution between January 2001 and December 2007. Anthropometric measurements were recorded at the time of neonatal admission and the bidirectional Glenn procedure. Data from preoperative echocardiograms and cardiac catheterization were recorded. The primary outcome variable was length of hospital stay. RESULTS Data on 100 infants were included for analysis. Age at the time of the bidirectional Glenn procedure was 5.1 months (range, 2.4-10 months). The median weight-for-age z score at birth was -0.4 (range, -2.6 to 3.2), and by the time of the bidirectional Glenn procedure, it had decreased to -1.3 (range, -3.9 to 0.6). In multivariable modeling longer postoperative hospital stays were predicted by lower weight-for-age z score (P = .02), younger age (P < .001), being fed through a gastrostomy tube (P = .01), and undergoing concomitant aortic arch reconstruction (P < .001) at the time of the bidirectional Glenn procedure. CONCLUSIONS There is suboptimal weight gain between neonatal discharge and the bidirectional Glenn procedure. A lower weight-for-age z score and younger age at the time of the bidirectional Glenn procedure affects length of hospital stay independent of hemodynamic or echocardiographic variables.

Variation in interstage outpatient care after the Norwood procedure: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

OBJECTIVE The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) is the first quality improvement collaborative in pediatric cardiology, and its registry captures information on interstage care and outcomes of infants after the Norwood procedure. The purpose of this study was to evaluate variation in interstage outpatient clinical care practices for infants discharged home after the Norwood procedure. DESIGN Data for the first 100 infants enrolled in the NPC-QIC registry were evaluated. The care domains assessed for variation included: (1) discharge communication with outpatient cardiologist and primary care physician (PCP); (2) nutrition plan at hospital discharge; and (3) planned use of home surveillance strategies. RESULTS One hundred infants were discharged home between July 2008 and February 2010, from 21 participating US pediatric cardiac programs. Median age at discharge was 29 (11-188) days. Interstage outpatient care was provided at the Norwood center for 62 infants, at other centers for 25, and at a combination of centers for 13. Complete discharge communication (defined as written communication of medication list, nutrition plan, and red flag checklist) was relayed to only 45 outpatient cardiologists and to 26 PCPs. Nutrition route at discharge was exclusively oral in 49, combined oral and nasogastric (NG)/nasojejunal (NJ) in 38, exclusively NG/NJ in six, combined oral and gastrostomy tube (GT) in six, and exclusively GT in one infant. Home surveillance strategies were utilized for 81 infants (oximetry and weight monitoring in 77, oximetry alone in four), with no home surveillance in 19 infants. CONCLUSIONS Considerable variation exists in interstage outpatient care after the Norwood procedure in the care domains of discharge communication, nutrition, and home surveillance. Standardizing care around evidence-based practices may improve the outcomes for these very high-risk children.

Nutrition algorithms for infants with hypoplastic left heart syndrome; birth through the first interstage period.

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.

Variation in Growth of Infants with a Single Ventricle

OBJECTIVE The study goal was to evaluate interstage growth variation among sites participating in the National Pediatric Cardiology Quality Improvement Collaborative registry caring for infants with hypoplastic left heart syndrome and to identify nutritional practices common among sites achieving best growth outcomes. STUDY DESIGN This was a retrospective analysis of infants in the registry who had presented due to their superior cavopulmonary connection (SCPC) and whose surgical site had enrolled ≥ 4 eligible patients in the registry. The primary outcome variable was weight-for-age z-score (WAZ) change between Norwood discharge and presentation for SCPC (interstage period). Blinded, structured interviews were performed with each site regarding site-specific nutritional practices. Practices common among sites with positive interstage WAZ changes were identified. RESULTS Sixteen centers enrolled 132 infants from December 2008 through December 2010. Median age at SCPC was 5 months (2.6-12.6), and median interstage WAZ change was -0.29 (-3.2 to 2.3). Significant variation in WAZ changes among sites was demonstrated (P < .001). Sites that used standard feeding evaluation prior to Norwood discharge and that closely monitored for specific weight gain/loss red flags in the interstage period demonstrated significantly better patient growth than those that did not use these practices (P = .002). CONCLUSIONS Considerable variation exists in interstage growth among patients receiving care at these 16 surgical sites. Standardization of interstage nutritional management with focus on best nutritional practices may lead to improved growth in this high-risk population of infants.

Improvement in Interstage Survival in a National Pediatric Cardiology Learning Network

Infants with univentricular congenital heart disease (CHD), including those with hypoplastic left heart syndrome (HLHS), regularly pose dilemmas in decision-making because their anatomy and physiology are often unique and variable. The typical staged surgical course for infants with complex univentricular anatomy with systemic outflow obstruction begins with the Norwood (stage 1) operation or variant shortly after birth, followed several months later by superior cavopulmonary anastomosis (stage 2 palliation) with an ultimate goal of a Fontan-type operation several years later.1–3 Improvement in surgical and postoperative management has led to considerable improvement in early post-Norwood survival in the recent era.4–7 However, after the Norwood procedure and before stage 2 palliation, a high-risk time period termed interstage, mortality has been previously been reported at 10% to 15%.8–10 The rare nature of this disorder has limited robust learning about successful strategies to improve survival undertaken by single-surgical centers, and a gap exists in our ability to further improve mortality in this population. The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), the first multicenter learning network within pediatric cardiology,11 was established with the goal of improving care and outcomes for children with univentricular heart after the Norwood operation and specifically to (1) improve interstage mortality, (2) decrease interstage growth failure, and (3) reduce interstage hospital readmissions for major medical events. There were several perceived challenges to success in changing clinical outcomes before starting the NPC-QIC collaborative. A primary challenge in collaboration among multiple sites can be agreement on best practices that should be implemented. This is especially true for rare diseases, such as univentricular heart disease, where evidence-based clinical guidelines are not available to clinicians. As noted above, major variation persists in management practices among individuals and institutions caring for children with HLHS and other …

Utility of ambulatory monitoring in patients with congenital heart disease.

Patients with congenital heart disease (CHD) are at increased risk of cardiac arrhythmias. The utility of ambulatory (Holter) monitoring in predicting these arrhythmias remains unclear. We sought to evaluate the clinical utility and cost effectiveness of Holter monitoring in patients with CHD. A retrospective study of patients with CHD who had undergone Holter monitoring was performed. The Holter data from patients with tetralogy of Fallot (TOF), d-transposition of the great arteries (d-TGA) after an atrial switch operation, and patients with a single ventricle after Fontan palliation were reviewed. The Holter indication included evaluation of clinical symptoms or as a part of routine screening. The Holter results were deemed clinically significant if they resulted in a change in patient treatment. Sudden cardiac events included death or aborted sudden death and appropriate defibrillator therapies. A total of 589 Holter studies were performed in 189 patients (100 with TOF, 38 with d-TGA, and 51 with Fontan). The results of Holter monitoring performed for clinical symptoms had a low positive predictive value (0.08) for clinically significant changes in management. On routine monitoring, the sensitivity was low (0.40) but the negative predictive value was high (0.96) for future clinically significant arrhythmias. The frequency of clinically significant findings and associated cost-effectiveness improved with older patient age and Fontan and d-TGA CHD type. Nonsustained ventricular tachycardia was associated with sudden cardiac events in patients with TOF but not in those with d-TGA or Fontan palliation. In conclusion, Holter monitoring is generally inefficient for symptomatic evaluation; however, within specific age and CHD type subgroups, such as patients with repaired TOF >25 years old, it could be useful in clinical management and risk assessment as a part of routine care.

Predictors of poor weight gain in infants with a single ventricle

OBJECTIVE To assess growth from the time of neonatal discharge to the time of performance of the bidirectional Glenn (BDG) procedure in infants with a single ventricle and determine predictors of poor growth. STUDY DESIGN We performed a retrospective case series of infants who underwent the BDG procedure at our institution between January 2001 and December 2007 (n=102). Anthropometric and clinical data were recorded during neonatal hospitalization and before BDG. Outcome variables included weight-for-age z-score (WAZ) at the time of BDG and average daily weight gain between neonatal discharge and BDG. RESULTS Median age at the time of BDG was 5.1 months (range, 2.4-10 months), and median WAZ was -0.4 (range, -2.6 to 3.2) at neonatal admission and -1.3 (range, -3.9 to 0.6) at the time of BDG. Non-Caucasian infants (P=.03) and those with lower WAZ at neonatal discharge (P<.0001) had a lower WAZ at BDG. Being formula-fed at neonatal discharge (P=.04), and having higher mean pulmonary arterial pressure (P=.04) and systemic oxygen saturation (P=.006) were associated with lower average daily weight gain between neonatal discharge and BDG. CONCLUSIONS Infants with a single ventricle have poor weight gain between neonatal discharge and BDG. Non-Caucasian infants and those with evidence of increased pulmonary blood flow are at particular risk for growth failure.

Low Weight-for-Age Z-Score and Infection Risk After the Fontan Procedure

BACKGROUND Poor growth is common in infants with a single ventricle. Lower weight-for-age z-score (WAZ) is associated with worse short-term outcome after bidirectional Glenn procedure. We sought to assess growth status at the time of the Fontan procedure and the effect of poor growth status on surgical outcomes. METHODS This retrospective case series examined children who underwent Fontan at our institution between January 2003 and December 2008. Weight and height were obtained at the time of admission for Fontan. Data from preoperative echocardiogram and cardiac catheterization were abstracted to document cardiac function and hemodynamic measurements. Outcome variables included ventilator time, chest tube duration, postoperative infections (bacteremia, mediastinitis, urinary tract infection, gastroenteritis, or culture-positive pneumonia), and length of hospital stay. RESULTS Fifty-five patients were included for analysis. The median age at Fontan was 46 months (range, 18 to 72); median WAZ was -1.0 (-3.8 to +2.0), and height for age z-score was -1.1 (-3.7 to +1.5). The WAZ was less than -2.0 in 19% of patients. Multivariable modeling revealed that patients with a WAZ less than -2.0 (p=0.006) had a greater incidence of serious postoperative infections. The only factor predicting longer length of hospital stay was presence of a serious postoperative infection (p<0.0001). Ventilator time was predicted only by length of cardiopulmonary bypass (p=0.01). No factors were associated with longer chest tube duration. CONCLUSIONS Growth failure in children with a single ventricle persists through presentation for Fontan. A WAZ less than -2.0 at Fontan is associated with a higher rate of serious postoperative infections, which are associated with longer length of hospital stay.

A KCNQ1 Mutation Causes a High Penetrance for Familial Atrial Fibrillation

Atrial fibrillation (AF) is the most common cardiac arrhythmia, and its incidence is expected to grow. A genetic predisposition for AF has long been recognized, but its manifestation in these patients likely involves a combination of rare and common genetic variants. Identifying genetic variants that associate with a high penetrance for AF would represent a significant breakthrough for understanding the mechanisms that associate with disease.

Use of a learning network to improve variation in interstage weight gain after the Norwood operation

BACKGROUND Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth. METHODS A previously identified Nutritional Bundle was spread among NPC-QIC sites. PRIMARY OUTCOME interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth. RESULTS Four hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008-December 2010) and 249 in Period 2 (December 2010-April 2013). Median age at S2 was 4.9 months (2.6-12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ <0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was <0 in six sites while in Period 2 no site had median interstage ΔWAZ <0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively). CONCLUSIONS Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.