Single Ventricle Infants Research Articles

Arginine-NO metabolites are associated with morbidity in single ventricle infants undergoing stage 2 palliation.

Infants with single ventricle heart disease (SVHD) suffer morbidity from insufficient pulmonary blood flow, which may be related to impaired arginine metabolism. No prior study has reported quantitative mapping of arginine metabolites to evaluate the relationship between circulating metabolite levels and outcomes. Prospective cohort study of 75 SVHD cases peri-Stage 2 and 50 healthy controls. We targeted pre- and post-op absolute serum quantification of 9 key members of the arginine metabolism pathway by tandem mass spectrometry. Primary outcomes were length of stay (LOS) and post-Stage 2 hypoxemia. Pre-op cases showed alteration in 6 metabolites including decreased arginine and increased asymmetric dimethyl arginine (ADMA) levels compared to controls. Post-op cases demonstrated decreased arginine and citrulline levels persisting through 48 h. Adjusting for clinical variables, lower pre-op and 2 h post-op concentrations of multiple metabolites, including arginine and citrulline, were associated with longer post-op LOS (p < 0.01). Increased ADMA at 24 h was associated with greater post-op hypoxemia burden (p < 0.05). Arginine metabolism is impaired in interstage SVHD infants and is further deranged following Stage 2 palliation. Patients with greater metabolite alterations experience greater post-op morbidity. Decreased arginine metabolism may be an important driver of pathology in SVHD. Interstage infants with SVHD have significantly altered arginine-nitric oxide metabolism compared to healthy children with deficiency of multiple pathway intermediates persisting through 48 h post-Stage 2 palliation. After controlling for clinical covariates and classic catheterization-derived predictors of Stage 2 readiness, both lower pre-operation and lower post-operation circulating metabolite levels were associated with longer post-Stage 2 LOS while increased post-Stage 2 ADMA concentration was associated with greater post-op hypoxemia. Arginine metabolism mapping offers potential for development using personalized medicine strategies as a biomarker of Stage 2 readiness and therapeutic target to improve pulmonary vascular health in infants with SVHD.

Are Parent Discharge Readiness Scores Effective for Patients With Congenital Heart Disease After Cardiac Surgery?

To assess discharge readiness and clinical engagement post-discharge in families of children undergoing congenital heart surgery. This prospective cross-sectional study was performed at a major tertiary pediatric cardiac referral center. Eligible parents and caregivers completed a discharge readiness tool, the Readiness for Hospital Discharge Scale for Parents of Hospitalized Children, via online survey on the day of discharge. Clinical engagement data included subsequent phone calls, clinic visits, emergency department visits, and hospital readmissions. Readiness for Hospital Discharge Scale for Parents of Hospitalized Children scores were measured as follows: very high (9-10), high (8-8.9), moderate (7-7.9), and low (<7). Descriptive statistics were used to describe demographic data. In total, 128 families enrolled between April and December 2021. Parent discharge readiness scores ranged from "high" to "very high." Families with lower socioeconomic status and younger patients (especially single-ventricle infants or "interstage") had a greater proportion of clinic visits, emergency department visits, and hospital readmissions within 30-days postdischarge compared with other groups. Discharge readiness scores were not associated with clinical engagement. We identified vulnerable populations as evidenced by a greater frequency of clinical engagement in the immediate postoperative period, particularly younger patients and first-time surgeries. Although these visits may be appropriate, novel programs could enhance education and emotional support to prevent delay in seeking care or creating excessive stress and anxiety after discharge.

Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study.

Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT. Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality. Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p=.004). While waitlist mortality was similar between groups (p=.09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p=.03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p=.03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival. Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.

Ability of Video Telemetry to Predict Unplanned Hospital Admissions for Single Ventricle Infants.

BackgroundOur Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA).Methods and ResultsSix candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home by CHAMP between March 2014 and March 2018 were included. Videos obtained within 48 hours before UHA were compared with videos obtained at baseline. We used binary logistic regression models and receiver operating characteristic curves to evaluate the parameters' performance in discriminating the outcome of interest.Thirty‐nine subjects with 64 UHA were included. We compared 64 pre‐admission videos to 64 paired baseline videos. Scoring was feasible for a mean of 91.6% (83.6%–98%) of all observations. Three different HVT score models were proposed, and a final model composed of respiratory rate, respiratory effort, color, and behavior exhibited an excellent discriminatory capability with an area under the receiver operating characteristic curve of 93% (89%–98%). HVT score of 5 was associated with specificity of 93.8% and sensitivity of 88.7% in predicting UHA.ConclusionsWe developed a feasible and reproducible HVT score that can serve as a tool to predict UHA in infants with single ventricle. Future directions involve prospective, multicenter validation of this tool.

Changes in Provider Prescribing Behavior for Infants with Single Ventricle Physiology After Evidence-Based Publications.

The impact of published evidence on clinical practice has been understudied in pediatric cardiology. We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.

Clinical Deterioration in Single Ventricle Infants Waiting for Heart Transplant

Ventricular assist device (VAD) support has drastically improved waitlist mortality for pediatric patients awaiting heart transplant (HT). However, VAD support for single ventricle (SV) patients remains challenging, particularly in smaller patients with non-Fontan physiology. Unfortunately, waitlist mortality and morbidity are also significant in this subset of SV patients, and the risks of VAD support should be examined in the context of waitlist risks. We examined clinical deterioration while on the waitlist for a subset of SV patients for whom VAD support remains challenging. We performed a retrospective review of all single ventricle patients listed for HT at a single pediatric center from 2012-2018. Patients <15 kg with non-Fontan physiology were included. A composite endpoint of waitlist clinical deterioration was defined as waitlist death, removal due to deterioration, or escalation of hemodynamic support (addition of inotropes, intubation, VAD or ECMO) before transplant. Kaplan Meier method was used to examine survival. Thirty-three SV patients were included. Median weight was 7.6 kg (IQR 5.1, 11.5), 58% were s/p Glenn, 21% Norwood, 18% hybrid or banding, and 3% unrepaired. Over a median 89 day (43, 206) of waitlist time, 21 (64%) were transplanted, 8 (24%) died or were removed for deterioration, and 4 (12%) were alive waiting or removed for improvement. Of the 21 transplanted, 7 had escalation of support by time of HT, for an overall waitlist deterioration of 45% (n=15/33, Figure 1A). From listing to HT, inotrope use increased from 48% to 71%, intubation 19% to 24%, and VAD 5% to 19%. At time of HT, 43% were in ICU and 29% were NPO for feeding intolerance. Survival to HT was similar between patients on VAD versus not on VAD support (67% vs 78%, p-value = 0.65, Figure 1B). Single ventricle infants are at high risk for death and clinical deterioration while waiting for HT. While VAD support remains challenging for this population, it can be a successful means of bridging to transplant.

Survival and Cardiopulmonary Resuscitation Hemodynamics Following Cardiac Arrest in Children With Surgical Compared to Medical Heart Disease.

To assess the association of diastolic blood pressure cutoffs (≥ 25 mm Hg in infants and ≥ 30 mm Hg in children) during cardiopulmonary resuscitation with return of spontaneous circulation and survival in surgical cardiac versus medical cardiac patients. Secondarily, we assessed whether these diastolic blood pressure targets were feasible to achieve and associated with outcome in physiology unique to congenital heart disease (single ventricle infants, open chest), and influenced outcomes when extracorporeal cardiopulmonary resuscitation was deployed. Multicenter, prospective, observational cohort analysis. Tertiary PICU and cardiac ICUs within the Collaborative Pediatric Critical Care Research Network. Patients with invasive arterial catheters during cardiopulmonary resuscitation and surgical cardiac or medical cardiac illness category. None. Hemodynamic waveforms during cardiopulmonary resuscitation were analyzed on 113 patients, 88 surgical cardiac and 25 medical cardiac. A similar percent of surgical cardiac (51/88; 58%) and medical cardiac (17/25; 68%) patients reached the diastolic blood pressure targets (p = 0.488). Achievement of diastolic blood pressure target was associated with improved survival to hospital discharge in surgical cardiac patients (p = 0.018), but not medical cardiac patients (p = 0.359). Fifty-three percent (16/30) of patients with single ventricles attained the target diastolic blood pressure. In patients with an open chest at the start of chest compressions, 11 of 20 (55%) attained the target diastolic blood pressure. In the 33 extracorporeal cardiopulmonary resuscitation patients, 16 patients (48%) met the diastolic blood pressure target with no difference between survivors and nonsurvivors (p = 0.296). During resuscitation in an ICU, with invasive monitoring in place, diastolic blood pressure targets of greater than or equal to 25 mm Hg in infants and greater than or equal to 30 mm Hg in children can be achieved in patients with both surgical and medical heart disease. Achievement of diastolic blood pressure target was associated with improved survival to hospital discharge in surgical cardiac patients, but not medical cardiac patients. Diastolic blood pressure targets were feasible to achieve in 1) single ventricle patients, 2) open chest physiology, and 3) extracorporeal cardiopulmonary resuscitation patients.

Non-Infant, Single Ventricle Patients Enjoy the Same Post-Transplant Survival as Other Congenital Heart Patients

Purpose Prior database studies have grouped pediatric transplant recipients broadly into “congenital heart disease” but have not distinguished between single ventricle (SV) patients and other diagnoses. By combining databases, we investigated whether SV patients could have similar post-transplant survival as biventricular (BiV) patients. Methods The UNOS database, providing patient outcomes, was combined with the Pediatric Hospital Information System (PHIS) database, used to elicit specific congenital diagnosis for patients transplanted at a PHIS hospital from 2006-2018. The most complex or diagnosis indicating SV physiology by ICD-9 diagnostic code was used to establish the primary diagnosis. Diagnoses were described and post-transplant outcomes compared by Kaplan-Meier analysis. Results There were 1,803 patients identified with congenital heart disease undergoing transplantation. Diagnoses were identified 1,031 (57%) identified as SV pathology, 706 (39%) as BiV pathology, and 66 (4%) as indeterminant. Only 78 (4%) patients were >18. Though 1-year survival was worse for SV diagnoses compared to biventricular (84% vs 91%; p-value 0.05. When stratified by age, SV infants (n=402) had worse long-term survival compared to BiV (log-rank p=0.038), however, non-infants (n=629) remained the same (p=0.405) as seen in Figure 1. Conclusion Although early survival is worse, overall SV patients are expected to enjoy equally excellent long-term survival after cardiac transplantation as other congenital heart disease patients. However, long-term survival for infant SV is inferior to infant BiV patients, primarily due to mortality within the six months.

ABILITY OF VIDEO TELEMEDICINE TO PREDICT UNPLANNED HOSPITAL READMISSIONS FOR SINGLE VENTRICLE INFANTS

Our Cardiac High Acuity Monitoring program (CHAMP) utilizes video telemetry to monitor inter-stage single ventricle infants (SVI) at home. Our objective is to develop a reliable video telemetry scoring system to predict unplanned hospital admissions (UHA) in this high-risk population. Videos from

Digoxin Use in Infants with Single Ventricle Physiology: Secondary Analysis of the Pediatric Heart Network Infant Single Ventricle Trial Public Use Dataset.

Digoxin has been associated with reduced interstage mortality after Norwood procedure. We sought to determine its association with survival and change in weight-for-age Z-score (WAZ) before the superior cavopulmonary connection (SCPC) surgery and at 14months in a heterogeneous group of single ventricle infants. We performed a post-hoc analysis of the Pediatric Heart Network Infant Single Ventricle public use dataset to determine associations between digoxin and survival, transplant-free survival, and change in WAZ pre-SCPC and at 14 months. Sub-analyses of survival and transplant-free survival were performed for subjects who underwent Damus-Kaye-Stansel (DKS)/Norwood. Propensity score weighting was used in Cox hazard-proportion models. Of 229 subjects, 82 (36%) received digoxin and 147 (64%) received no digoxin. Pre-SCPC and 14-month survival and transplant-free survival were not significantly different between the digoxin and no digoxin groups for the main cohort and DKS/Norwood sub-group. However, in DKS/Norwood subjects there was a trend towards improved interstage transplant-free survival in the digoxin group (95.7 vs. 89.6%, p = 0.08). Digoxin was associated with a greater decrease in WAZ from birth to pre-SCPC (- 1.96 ± 0.19 vs. - 1.31 ± 0.18, p < 0.001) and birth to 14 months (- 0.64 ± 0.15 vs. - 0.19 ± 0.15, p = 0.03). Digoxin was not associated with improved survival during the interstage or at 14 months in a mixed single ventricle cohort, but there was a trend towards improved interstage transplant-free survival in post-Norwood infants. As digoxin was associated with poorer weight gain, further research is needed to identify the risks/benefits for anatomic subtypes of infants with single ventricles.

Hemoglobin Level at Stage 1 Discharge has No Impact on Inter-stage Growth and Stability in Single Ventricle Infants.

Hemoglobin levels (Hgb) of infants with a single ventricle (SV) are traditionally maintained high to maximize oxygen-carrying capacity during stage 1 palliation (S1P), stage 2 palliation (S2P), and between stages (IS). A single-center observational cohort study was performed to determine if red blood cell transfusion during the convalescent phase of the S1P (late S1P transfusion) to achievehigher Hgb is associated with benefits during the IS including improved growth and decreased acute medical events. 137 infants <1year with SV with SIP undergoing care from January 2008 to June 2015 were retrospectively evaluated. 78 (57%) infants received a late S1P transfusion. Median Hgb at S1P discharge was 15.9g/dL (IQR 14.7-17.1) and median Hgb S2P at admission was 15.3g/dL (IQR 14-16.3). Median daily weight gain was 22g/day during IS (IQR 17-26) and median daily length gain was 0.09cm (IQR 0.06-0.11). Hgb at SIP discharge was not associated with IS growth or fewer IS acute events. However, late S1P transfusions were associated with illness severity at S1P and more complicated S1P care. Our data suggest that SV infants after S1P, who are steadily recovering, do not benefit from late transfusion to raise their hemoglobin level at discharge.

Translating clinical trials into clinical practice: a survey assessing the potential impact of the Pediatric Heart Network Infant Single Ventricle Trial.

A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice. A web-based survey was distributed via e-mail to over 2000 paediatric cardiologists, intensivists, cardiothoracic surgeons, and cardiac advance practice nurses during three distribution periods. The results were analysed using McNemar's test for paired data and Fisher's exact test. The response rate was 31.5% (69% cardiologists and 65% with >10 years of experience). Among respondents familiar with trial results, 74% reported current practice consistent with trial findings versus 48% before trial publication (p<0.001); 19% used angiotensin-converting enzyme inhibitor in this population "almost always" versus 36% in the past (p<0.001), and 72% reported a change in management or improved confidence in treatment decisions involving this therapy based on the trial results. Respondents familiar with trial results (78%) were marginally more likely to practise consistent with the trial results than those unfamiliar (74 versus 67%, p=0.16). Among all respondents, 28% reported less frequent use of angiotensin-converting enzyme inhibitor over the last 3 years. Within 5 years of publication, the majority of respondents was familiar with the Infant Single Ventricle Trial results and reported less frequent use of angiotensin-converting enzyme inhibitor in single-ventricle infants; however, 28% reported not adjusting their clinical decisions based on the trial's findings.

A Novel Electrocardiogram Algorithm Utilizing ST-Segment Instability for Detection of Cardiopulmonary Arrest in Single Ventricle Physiology: A Retrospective Study

We evaluated ST-segment monitoring to detect clinical decompensation in infants with single ventricle anatomy. We proposed a signal processing algorithm for ST-segment instability and hypothesized that instability is associated with cardiopulmonary arrests. Retrospective, observational study. Tertiary children's hospital 21-bed cardiovascular ICU and 36-bed step-down unit. Twenty single ventricle infants who received stage 1 palliation surgery between January 2013 and January 2014. Twenty rapid response events resulting in cardiopulmonary arrests (arrest group) were recorded in 13 subjects, and nine subjects had no interstage cardiopulmonary arrest (control group). None. Arrest data were collected over the 4-hour time window prior to cardiopulmonary arrest. Control data were collected from subjects with no interstage arrest using the 4-hour time window prior to cardiovascular ICU discharge. A paired subgroup analysis was performed comparing subject 4-hour windows prior to arrest (prearrest group) with 4-hour windows prior to discharge (postarrest group). Raw values of ST segments were compared between groups. A 3D ST-segment vector was created using three quasi-orthogonal leads (II, aVL, and V5). Magnitude and instability of this continuous vector were compared between groups. There was no significant difference in mean unprocessed ST-segment values in the arrest and control groups. Utilizing signal processing, there was an increase in the ST-vector magnitude (p = 0.02) and instability (p = 0.008) in the arrest group. In the paired subgroup analysis, there was an increase in the ST-vector magnitude (p = 0.05) and instability (p = 0.05) in the prearrest state compared with the postarrest state prior to discharge. In single ventricle patients, increased ST instability and magnitude were associated with rapid response events that required intervention for cardiopulmonary arrest, whereas conventional ST-segment monitoring did not differentiate an arrest from control state.

Perioperative Feeding Approaches in Single Ventricle Infants: A Survey of 46 Centers.

Background Feeding dysfunction occurs commonly in infants with single ventricle heart disease and impacts growth and long-term outcomes. Little evidence exists to guide safe feeding in this population. This study surveyed centers participating in the National Pediatric Cardiology Quality Improvement Collaborative to assess prevailing feeding practices amongthose caring for single ventricle neonates. Methods Web-based survey of 56 pediatric cardiac surgical centers was conducted. Questions addressed peri-operative feeding approaches and responses were presented and analyzed descriptively. Results Of 56 centers, 46 (82%) completed a survey. Preoperative feeding was common in single ventricle infants (30/46; 65%), routes varied. Centers who did not feed infants preoperatively cited the risk of necrotizing enterocolitis (16/16; 100%), presence of umbilical artery catheter (12/16; 75%), and prostaglandin infusion (9/16; 56%) as main concerns. 67% of centers reported no specific vital sign thresholds for withholding enteral feedings. In the postoperative period, most centers used an "internal guideline" (21/46; 46%) or an "informal practice" (15/46; 33%) to determine feeding readiness. Approaches to findings were significantly different among centers. About 40% of centers did not send patients home with feeding tubes, and there was no clear consensus between preferred feeding tube modality at discharge. Conclusion Considerable variation exists in feeding practices for infants with single ventricle congenital heart disease among 46 centers participating in a quality improvement collaborative. Although most centers generally feed infants preoperatively, feeding practices remain center-specific. Variability continues in the immediate post-operative and interstage periods. Further opportunities exist for investigation, standardization and development of best-practice feeding guidelines.

Improving Outcomes for Infants with Single Ventricle Physiology through Standardized Feeding during the Interstage.

Congenital heart disease is identified as the most common birth defect with single ventricle physiology carrying the highest mortality. Staged surgical palliation is required for treatment, with mortality historically as high as 22% in the four- to six-month period from the first- to second-stage surgical palliation, known as the interstage. A standardized postoperative feeding approach was implemented through an evidence-based protocol, parent engagement, and interprofessional team rounds. Five infants with single ventricle physiology preprotocol were compared with five infants who received the standardized feeding approach. Mann-Whitney U tests were conducted to evaluate the hypotheses that infants in the intervention condition would consume more calories and have a positive change in weight-to-age z-score (WAZ) and shorter length of stay (LOS) following the first and second surgeries compared to infants in the control condition. After the protocol, the change in WAZ during the interstage increased by virtually one standard deviation from 0.05 to 0.91. Median LOS dropped 32% after the first surgery and 43% after the second surgery. Since first- and second-stage palliative surgeries occur within the same year of life, this represents savings of $500,000 to $800,000 per year in a 10-infant model. The standardized feeding approach improved growth in single ventricle infants while concurrently lowering hospital costs.

Near-infrared spectroscopy after high-risk congenital heart surgery in the paediatric intensive care unit

To establish whether the use of near-infrared spectroscopy is potentially beneficial in high-risk cardiac infants in United Kingdom paediatric intensive care units. A prospective observational pilot study. An intensive care unit in North West England. A total of 10 infants after congenital heart surgery, five with biventricular repairs and five with single-ventricle physiology undergoing palliation. Cerebral and somatic near-infrared spectroscopy monitoring for 24 hours post-operatively in the intensive care unit. Overall, there was no strong correlation between cerebral near-infrared spectroscopy and mixed venous oxygen saturation (r=0.48). At individual time points, the correlation was only strong (r=0.74) 1 hour after admission. The correlation was stronger for the biventricular patients (r=0.68) than single-ventricle infants (r=0.31). A strong inverse correlation was demonstrated between cerebral near-infrared spectroscopy and serum lactate at 3 of the 5 post-operative time points (1, 4, and 12 hours: r=-0.76, -0.72, and -0.69). The correlation was stronger when the cerebral near-infrared spectroscopy was <60%. For cerebral near-infrared spectroscopy <60%, the inverse correlation with lactate was r=-0.82 compared with those cerebral near-infrared spectroscopy >60%, which was r=-0.50. No correlations could be demonstrated between (average) somatic near-infrared spectroscopy and serum lactate (r=-0.13, n=110) or mixed venous oxygen saturation and serum lactate. There was one infant who suffered a cardiopulmonary arrest, and the cerebral near-infrared spectroscopy showed a consistent 43 minute decline before the event. We found that cerebral near-infrared spectroscopy is potentially beneficial as a non-invasive, continuously displayed value and is feasible to use on cost-constrained (National Health Service) cardiac intensive care units in children following heart surgery.

Impact of Pharmacotherapy on Interstage Outcomes in Single Ventricle Infants

To characterize the pharmacotherapeutic regimens used in infants with single ventricle heart disease and determine the influence of outpatient medications on interstage weight gain. Retrospective review. Tertiary care pediatric hospital. All patients discharged from our institution with single ventricle heart disease that underwent neonatal first stage surgical palliation between 2002 and 2009 were included. Patients who died prior to second stage palliation or underwent orthotopic heart transplantation were excluded. Outpatient medication regimens during the interstage period were reviewed. Medication regimens were compared between surgical eras and between patient groups experiencing different outcomes. A logistic regression model was developed to determine independent factors for an interstage increase in weight-for-age z-score (WAZ) and a linear regression model to determine medications significant for an increase in weight gain per day. The study cohort consisted of 161 patients (58% male). Most patients in this cohort had either hypoplastic left heart syndrome (51%) or unbalanced complete atrioventricular canal (29%). Patients were placed on a median of four medications (range 1-9) at discharge from first surgical palliation, with aspirin (79%), furosemide (79%), and angiotensin converting enzyme inhibitors (ACE-I) (73%) most commonly prescribed. A median of six medication doses per day (range 2-18) were prescribed at discharge. Most patients (71%) had a decrease in WAZ during the interstage period. Use of digoxin (P < 0.01) and high-dose furosemide (P = .02) were associated with a decrease in WAZ score during the interstage period. Additionally, the use of ACE-I, ranitidine, proton-pump inhibitors, or promotility agents was not associated with improved somatic growth during the interstage period. Infants with single ventricle heart disease have a high-medication burden during the interstage period. Despite the focused and intensified use of medications to improve feeding tolerance and somatic growth, current pharmacotherapeutic regimens appear to have little effect on interstage weight gain.