Association of hepatitis delta virus infection and hepatocellular carcinoma, hepatic decompensation, all-cause and liver-related death in a national cohort.

BACKGROUND: Hepatitis Delta Virus (HDV) infection is the most severe form of chronic hepatitis. However, studies on outcomes and causes of death in a United States-born population, with primarily horizontal transmission of HDV, are lacking. The aim of this study was to conduct a national study of patients with hepatitis D to understand the natural history and outcomes compared to patients with hepatitis B virus (HBV). infection. METHODS: In a national cohort of 4,817 HBV infected veterans tested for HDV (99.6% US-born, 3.3% HDV positive) over a 23-year period, we used multivariable models to identify the factors associated with a composite outcome of hepatocellular carcinoma (HCC), decompensation, and liver-related mortality (LRM), as well as all-cause mortality of patients with HDV compared to HBV mono-infection. RESULTS: HDV coinfection (vs. HBV monoinfection) was associated with a significantly higher incidence of composite liver-related outcomes at both 5 (23.84 vs. 7.98, p<0.001), and 10 years (19.14 vs. 10.18, p<0.001) respectively. The most common cause of death was liver-related (33.8% for HDV vs. 24.7% for HBV), followed by non-hepatic malignancies, (15.6 vs. 14.8%),cardiac (11.7 vs. 15.2%), and lung disease (5.2 vs. 3.7%). In multivariable models, HDV was associated with an increased risk of composite liver outcomes (aHR 2.57, 95% CI 1.87-3.52, p<0.001), and all-cause mortality (aHR 1.52, 95% CI 1.20-1.93, p<0.001). CONCLUSION: In a predominantly U.S born cohort of Veterans, HDV co-infection was associated with an increased risk of liver-related outcomes and all-cause mortality. Our findings support widespread testing for early identification of HDV.

Success of the US Veterans Health Administration's Hepatitis C Virus Care Continuum in the Direct-acting Antiviral Era.

BACKGROUND: Estimated hepatitis C prevalence within the Veterans Health Administration is higher than the general population and is a risk factor for advanced liver disease and subsequent complications. We describe the hepatitis C care continuum within the Veterans Health Administration 1 January 2014 to 31 December 2022. METHODS: We included individuals in Veterans Health Administration care 2021-2022 who were eligible for direct-acting antiviral treatment 1 January 2014 to 31 December 2022. We evaluated the proportion of Veterans who progressed through each step of the hepatitis C care continuum, and identified factors associated with initiating direct-acting antivirals, achieving sustained virologic response, and repeat hepatitis C viremia. RESULTS: We identified 133 732 Veterans with hepatitis C viremia. Hepatitis C treatment was initiated in 107 134 (80.1%), with sustained virologic response achieved in 98 136 (91.6%). In those who achieved sustained virologic response, 1097 (1.1%) had repeat viremia and 579 (52.8%) were retreated for hepatitis C. Veterans of younger ages were less likely to initiate treatment and achieve sustained virologic response, and more likely to have repeat viremia. Stimulant use and unstable housing were negatively associated with each step of the hepatitis C care continuum. CONCLUSIONS: The Veterans Health Administration has treated 80% of Veterans with hepatitis C in care 2021-2022 and achieved sustained virologic response in more than 90% of those treated. Repeat viremia is rare and is associated with younger age, unstable housing, opioid use, and stimulant use. Ongoing efforts are needed to reach younger Veterans, and Veterans with unstable housing or substance use disorders.

Core implementation strategies for improving cirrhosis care in the Veterans Health Administration.

BACKGROUND AND AIMS: The Veterans Health Administration (VHA) provides care for more than 80,000 veterans with cirrhosis. This longitudinal, multimethod evaluation of a cirrhosis care quality improvement program aimed to (1) identify implementation strategies associated with evidence-based, guideline-concordant cirrhosis care over time, and (2) use qualitative interviews to operationalize strategies for a manualized intervention. APPROACH AND RESULTS: VHA providers were surveyed annually about the use of 73 implementation strategies to improve cirrhosis care in fiscal years 2018 (FY18) and 2019 (FY19). Implementation strategies linked to guideline-concordant cirrhosis care were identified using bivariate statistics and comparative configurational methods. Semistructured interviews were conducted with 12 facilities in the highest quartile of cirrhosis care to specify the successful implementation strategies and their mechanisms of change. A total of 106 VHA facilities (82%) responded at least once over the 2-year period (FY18, n = 63; FY19, n = 100). Facilities reported using a median of 12 (interquartile range [IQR] 20) implementation strategies in FY18 and 10 (IQR 19) in FY19. Of the 73 strategies, 35 (48%) were positively correlated with provision of evidence-based cirrhosis care. Configurational analysis identified multiple strategy pathways directly linked to more guideline-concordant cirrhosis care. Across both methods, a subset of eight strategies was determined to be core to cirrhosis care improvement and specified using qualitative interviews. CONCLUSIONS: In a national cirrhosis care improvement initiative, a multimethod approach identified a core subset of successful implementation strategy combinations. This process of empirically identifying and specifying implementation strategies may be applicable to other implementation challenges in hepatology.

Trends in Treatment Uptake and Provider Specialty for Hepatitis C Virus (HCV) Infection in the Veterans Affairs Healthcare System: Results From the Electronically Retrieved Cohort of HCV-Infected Veterans (ERCHIVES).

Between 2001 and 2017, 108133 persons (45.7% of diagnosed cases) were initiated on anti-hepatitis C virus treatment in the Veterans Affairs healthcare system. In 2017, nonphysician clinicians accounted for 22.2% of prescriptions, infectious diseases specialists for 14.9%, and gastroenterologists/hepatologists for 10.3%. In the pre-direct-acting antiviral era, they accounted for 7.2%, 26.7%, and 11.6%, respectively.

Curing Hepatitis C Virus Infection: Best Practices From the U.S. Department of Veterans Affairs.

The U.S. Department of Veterans Affairs (VA) is the nation's largest care provider for hepatitis C virus (HCV)-infected patients and is uniquely suited to inform national efforts to eliminate HCV. An extensive array of delivery of services, policy guidance, outreach efforts, and funding has broadened the reach and capacity of the VA to deliver direct-acting antiviral (DAA) HCV therapy, supported by an infrastructure to effectively implement change and informed by extensive population health data analysis. The VA has treated more than 92 000 HCV-infected veterans since all-oral DAAs became available in January 2014, with cure rates exceeding 90%; only 51 000 veterans in VA care are known to remain potentially eligible for treatment. Key actions advancing the VA's aggressive treatment of HCV infection that are germane to non-VA settings include expansion of treatment capacity through the use of nonphysician providers, video telehealth, and electronic technologies; expansion of integrated care to address psychiatric and substance use comorbidities; and electronic data tools for patient tracking and outreach. A critical component of effective implementation has been building infrastructure through the creation of regional multidisciplinary HCV Innovation Teams, whose system redesign efforts have produced innovative HCV practice models addressing gaps in care while providing more efficient and effective HCV management for the populations they serve. Financing for HCV treatment and infrastructure resources coupled with reduced drug prices has been paramount to the VA's success in curing HCV infection. The VA is poised to share and extend best practices to other health care organizations and providers delivering HCV care, contributing to a concerted effort to reduce the overall burden of HCV infection.

Postoperative Complications and Outcomes Associated With a Transition to 24/7 Intensivist Management of Cardiac Surgery Patients.

OBJECTIVES: Nighttime intensivist staffing does not improve patient outcomes in general ICUs. Few studies have examined the association between dedicated in-house 24/7 intensivist coverage on outcomes in specialized cardiac surgical ICUs. We sought to evaluate the association between 24/7 in-house intensivist-only management of cardiac surgical patients on postoperative complications and health resource utilization. DESIGN: Before-and-after propensity matched cohort study. SETTING: Tertiary care cardiac surgical ICU. PATIENTS: Patients greater than 18 years old who underwent cardiac surgery between January 1, 2006, and April 30, 2013 (nighttime resident model), were propensity-matched (1:1) to patients from August 1, 2013, to December 31, 2014 (24/7 in-house intensivist model). INTERVENTIONS: Cardiac surgical ICU coverage change from a nighttime resident physician coverage model to a 24/7 in-house intensivist staffing model. MEASUREMENTS AND MAIN RESULTS: The primary outcome of interest was a composite of postoperative major complications. Secondary outcomes included duration of mechanical ventilation, all-cause cardiac surgical ICU readmissions, and surgical postponements attributed to lack of cardiac surgical ICU bed availability. A total of 1,509 patients during the nighttime resident model were matched to 1,509 patients during the intensivist model. The adjusted risk of major complications (26.3% vs 19.3%; odds ratio, 0.73; 95% CI, 0.36-0.85; p < 0.01), mean mechanical ventilation time (25.2 vs 19.4 hr; p < 0.01), cardiac surgical ICU readmissions (5.3% vs 1.6%; odds ratio, 0.31; 95% CI, 0.19-0.48; p < 0.01), and surgical postponements (3.4 vs 0.3 per mo; p < 0.01) were lower with the intensivist model. CONCLUSIONS: A transition to a 24/7 in-house intensivist care model was associated with a reduction in postoperative major complications, duration of mechanical ventilation, cardiac surgical ICU readmissions, and surgical postponements. These findings suggest that 24/7 intensivist physician care models may improve patient outcomes and health resource utilization in specialized cardiac surgical ICUs.

Post-operative Outcomes in Children Undergoing Fontan Palliation in a Regionalized Surgical System.

Evidence suggests that outcomes in pediatric cardiac surgery are improved by consolidating care into centers of excellence. Our objective was to determine if outcomes are equivalent in patients across a large regional referral base, or if patients from centers without on-site surgery are at a disadvantage. Since 1996, all pediatric cardiac surgery has been offered at one of two centers within the region assessed, with the majority being performed at Stollery Children's Hospital. All patients who underwent a Fontan between 1996 and 2016 were included. Follow-up data including length of stay (LOS), repeat surgical interventions, and transplant-free survival were acquired for each patient. The association between post-operative outcomes and home center was assessed using Kaplan-Meier survival analysis and Cox proportional Hazards models. 320 children (median age 3.3 years, IQR 2.8-4.0) were included; 120 (37.5%) had the surgical center as their home center. Cardiac anatomy was hypoplastic left heart syndrome in 107 (33.4%) subjects. Median LOS was 11 days (IQR, 8-17), and there were 8 in-hospital deaths. There were 17 deaths and 11 transplants over the course of follow-up. Five-year transplant-free survival was 92.5%. There was no difference in hospital re-intervention, late re-intervention, or survival by referral center (all p > 0.05). In multivariable analysis, home center was not predictive of either LOS (R 2 = -0.40, p = 0.87) or transplant-free survival (1.52, 95%CI 0.66, 3.54). In children with complex congenital heart disease, a regionalized surgical care model achieves good outcomes, which do not differ according to a patient's home base.

Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation.

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan-Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood-Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.

Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention.

OBJECTIVE: To compare the proportion of developmental delay in early complex cardiac surgery (CCS) survivors with and without gastrostomy tube feeding (GTF). To explore acute care predictors of GTF that might help improve care in CCS survivors. STUDY GROUP: This comparison study of 2 groups within an inception cohort included 334 CCS survivors after cardiopulmonary bypass at ≤6 weeks of age (2005-2012) who did not require extracorporeal membrane oxygenation or heart transplantation. Children were assessed at 21 ± 3 months with the Bayley Scales of Infant and Toddler Development-Third Edition and the Adaptive Behavior Assessment System-Second Edition: general adaptive composite score. Delay was determined by scores >2 SD below mean. The χ(2) test compared groups. Predictors of GTF were analyzed using multiple logistic regression analysis, results expressed as OR with 95% CI. RESULTS: Of the survivors, 67/334 (20%) had GTF any time before the 21-month assessment. Developmental delays in children with GTF were cognitive in 16 (24%), motor in 18 (27%), language in 24 (36%) vs without GTF in 7 (3%), 8 (3%), and 32 (12%), respectively (P < .001). Gastrostomy group had almost 8 times the number of children delayed on the general adaptive composite score. Independent OR for GTF are presence of a chromosomal abnormality, OR 4.6 (95% CI 1.8, 12.0) (P = .002), single ventricle anatomy, OR 3.4 (95% CI 1.7, 6.8) (P < .001), total postoperative days of open sternum, OR 1.15 (95% CI 1.1, 1.3) (P = .031), and total number of hospital days at CCS, OR 1.03 (95% CI 1.1, 1.04) (P = .002). CONCLUSIONS: GTF identifies CCS survivors at risk for delay, who would benefit from early developmental intervention. The described mostly nonmodifiable predictors may guide counseling of these children's families.

Cascade of Care for Hepatitis C Virus Infection Within the US Veterans Health Administration.

OBJECTIVES: We measured the quality of HCV care using a cascade of HCV care model. METHODS: We estimated the number of patients diagnosed with chronic HCV, linked to HCV care, treated with HCV antivirals, and having achieved a sustained virologic response (SVR) in the electronic medical record data from the Veterans Health Administration's Corporate Data Warehouse and the HCV Clinical Case Registry in 2013. RESULTS: Of the estimated 233,898 patients with chronic HCV, 77% (181,168) were diagnosed, 69% (160,794) were linked to HCV care, 17% (39,388) were treated with HCV antivirals, and 7% (15,983) had achieved SVR. CONCLUSIONS: This Cascade of HCV Care provides a clinically relevant model to measure the quality of HCV care within a health care system and to compare HCV care across health systems.

Endothelin axis is upregulated in human and rat right ventricular hypertrophy.

RATIONALE: Right ventricular (RV) function is the most important determinant of morbidity and mortality in pulmonary arterial hypertension (PAH). Endothelin (ET)-1 receptor antagonists (ERAs) are approved therapies for PAH. It is not known whether ERAs have effects on the RV, in addition to their vasodilating/antiproliferative effects in pulmonary arteries. OBJECTIVE: We hypothesized that the ET axis is upregulated in RV hypertrophy (RVH) and that ERAs have direct effects on the RV myocardium. METHODS AND RESULTS: RV myocardial samples from 34 patients with RVH were compared with 16 nonhypertrophied RV samples, and from rats with normal RV versus RVH attributable to PAH. Confocal immunohistochemistry showed that RVH myocardial ET type A (but not type B) receptor and ET-1 protein levels were increased compared with the nonhypertrophied RVs and positively correlated with the degree of RVH (RV thickness/body surface area; r(2)=0.838 and r(2)=0.818, respectively; P<0.01). These results were recapitulated in the rat model. In modified Langendorff perfusions, ERAs (BQ-123 and bosentan 10(-7,-6,-5) mol/L) decreased contractility in the hypertrophied, but not normal RV, in a dose-dependent manner (P<0.01). CONCLUSIONS: Patients and rats with PAH have an upregulation of the myocardial ET axis in RVH. This might be a compensatory mechanism to preserve RV contractility, as the afterload increases. ERAs use might potentially worsen RV function, and this could explain some of the peripheral edema noted clinically with these agents. Further studies are required to evaluate the effects of ERAs on the RV in patients with RVH and PAH.

Refractory cardiogenic shock in a patient with ß-thalassemia major requiring mechanical circulatory support: Case report and literature review.

Iron overload cardiomyopathy secondary to ß-thalassemia major is a potentially reversible condition managed with chelation and medical hemodynamic support, as bridge-to-recovery or transplant. We describe our experience, and challenges faced, in a pediatric patient with iron overload cardiomyopathy secondary to ß-thalassemia major, requiring biventricular MCS.

The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital.

Health-related quality of life in pediatric cardiac extracorporeal life support survivors.

OBJECTIVE: To assess the health-related quality of life of children who received cardiac extracorporeal life support. We hypothesized that extracorporeal life support survivors have lower health-related quality-of-life scores when compared with a healthy sample, with children with chronic conditions, and with children who had surgery for congenital heart disease and did not receive extracorporeal life support. DESIGN: Prospective cohort study. SETTING: Stollery Children's Hospital and Complex Pediatric Therapies Follow-up Program clinics. PATIENTS: Children less than or 5 years old with diagnosis of cardiac disease (congenital or acquired) who received extracorporeal life support at the Stollery Children's Hospital from 1999 to 2009. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Health-related quality of life was assessed using the PedsQL 4.0 Generic Core Scales completed by the children's parents at the time of follow-up. Forty-seven cardiac extracorporeal life support survivors had their health-related quality of life assessed at a median age of 4 years. Compared with a healthy sample, children who received venoarterial extracorporeal life support have significantly lower PedsQL (64.9 vs 82.2; p < 0.0001). The PedsQL scores of children who received extracorporeal life support were also significantly lower than those of children with chronic health conditions (64.9 vs 73.1; p = 0.007). Compared with children with congenital heart disease who underwent cardiac surgery early in infancy and who did not receive extracorporeal life support, extracorporeal life support survivors had significantly lower PedsQL scores (64.9 vs 81.1; p < 0.0001). Multiple linear regression analysis found an independent association between both higher inotrope score in the first 24 hours of extracorporeal life support and longer hospital length of stay, with lower PedsQL scores. CONCLUSIONS: Pediatric cardiac extracorporeal life support survivors showed lower health-related quality of life than healthy children, children with chronic conditions, and children with congenital heart disease who did not receive extracorporeal life support.

Neurotoxicity of sedative and analgesia drugs in young infants with congenital heart disease: 4-year follow-up.

OBJECTIVES/AIM: To determine whether sedation/analgesia drugs used before, during, and after infant cardiac surgery are associated with neurocognitive and functional outcomes. BACKGROUND: Some animal models suggest neurotoxic effects of anesthetic drugs on the developing brain; however, potential human effects are unknown. Whether these results can be extrapolated to humans is unknown. METHODS/MATERIALS: Prospective follow-up project of all infants ≤6 weeks old having surgery for congenital heart disease between 04/03 and 12/06. Demographic, perioperative, and sedation/analgesia variables were collected. Outcomes at kindergarten age were Wechsler Preschool and Primary Scale of Intelligence-III, Beery-Buktenica Developmental Test of Visual Motor Integration (VMI-V), and General Adaptive Composite (GAC) of the Adaptive Behavior Assessment System-II. Multivariable linear regression was used to identify predictor variables. RESULTS: From 135 infants who underwent heart surgery, 19 died, 17 were excluded, 8 were lost to follow-up, leaving 91 children for analysis. Multiple linear regression found days on chloral hydrate [3.5 (3.7) days] was associated with lower performance intelligence quotient (PIQ) (Effect size -1.03; 95% CI -1.96, -0.10; P = 0.03), and cumulative dose [54.2 (60.3) mg·kg(-1) ] of benzodiazepines was associated with lower VMI scores (Effect size -0.07; 95% CI -0.12, -0.01; P = 0.026). No other associations were found between sedation/analgesia variables and full-scale IQ, PIQ, Verbal IQ, VMI, or GAC. CONCLUSION: Assessment of this cohort at kindergarten age found a small statistically significant association between days on chloral hydrate and PIQ, and benzodiazepine cumulative dose and lower VMI. No other association between sedation/analgesia drugs and outcomes was found.

Comparison of two surgical techniques for complete atrioventricular septal defect repair using two- and three-dimensional echocardiography.

Different surgical techniques for complete atrioventricular septal defect (CAVSD) repair have been described, with the double-patch technique being most frequently employed. More recently a newer technique using a modified single-patch repair has been advocated. We hypothesized that the modified single-patch technique would result in an increased incidence of the two major post-repair comorbidities, namely, distortion of the left AV valve (LAVV) leaflets and narrowing of the left-ventricular outflow tract (LVOT). We studied 14 patients with CAVSD who underwent either traditional double-patch technique [group 1 (n = 7)] or modified single-patch technique [group 2 (n = 7)]. Preoperative and immediate postoperative two-dimensional (2D) echocardiograms, as well as follow-up 2D and three-dimensional (3D) studies, were reviewed. For group 1, the median age at repair was 4.1 months with a median duration from surgical repair and last echocardiogram of 44 months. For group 2, the median age at repair was 3 months with a median duration from surgical repair and last echocardiogram of 28 months. The two groups had similar demographics and ventricular septal defect size before surgery. For the LAVV, no significant difference was observed with respect to LAVV annulus size, tenting height, and the size of the vena contracta. Furthermore, there was no significant difference in the 2D echocardiographic areas and volumes of the LVOT between pre-repair and immediate post-repair studies for both groups. At the last evaluation, although there had been growth of the LVOT in both groups, no significant difference between areas and volumes were observed. Areas of the LVOT measured by 3D echocardiography on the final study showed no significant statistical difference between both groups. There was good correlation of the areas measured by 2D and 3D echocardiography within each group. In this small group, modified single-patch technique does not appear to tether the LAVV or promote an increase in regurgitation. In the short term, LVOT growth is unaffected, and the repair does not promote LVOT obstruction. 3D echocardiography is useful for area measurements of the LVOT and showed good correlation with areas measured by assumption of the LVOT shape as determined using 2D techniques.

Health-related quality of life experienced by children with chromosomal abnormalities and congenital heart defects.

Long-term outcomes are fundamental in advising parents about the potential future of their children with congenital heart disease (CHD). No published reports have described the health-related quality of life (HRQL) experienced by children with chromosomal abnormalities who had surgery in early infancy for CHD. A study was undertaken to assess HRQL among children with chromosomal abnormalities and CHD. The authors hypothesized that these children have a worse HRQL than healthy children or a cohort of children matched for CHD diagnosis. Infants with chromosomal abnormalities undergoing cardiac surgery for CHD at 6 weeks of age or younger at the Stollery Children's Hospital between July 2000 and June 2005 were included in the study. The HRQL of these infants was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales completed by their parents at a 4-year follow-up evaluation. The study compared the scores for 16 children with normative data. The children with chromosomal abnormalities and CHD had significantly lower mean total PedsQL (71.3 vs. 87.3; p < 0.0001), Psychosocial Summary (70.3 vs. 86.1; p < 0.0001), and Physical Summary (74.3 vs. 89.2; p = 0.0006) scores. Compared with the matched children, those with chromosomal abnormalities had a significantly lower median total PedsQL (75.0 vs. 84.6; p = 0.03), Physical Summary (79.5 vs. 96.9; p = 0.007), and School Functioning (68.5 vs. 83.0; p = 0.03) scores. A better understanding of the mechanisms and determinants of HRQL in these children has the potential to yield important implications for clinical practice including clarity for treatment decision making as well as determination of targeted supports and services to meet the needs of these children and their families differentially.

Risk factors for and outcomes of acute kidney injury in neonates undergoing complex cardiac surgery.

OBJECTIVE: To characterize the epidemiology of and identify risk factors for neonatal cardiac surgery-associated acute kidney injury (CS-AKI) and determine its impact on clinical outcomes. STUDY DESIGN: Using secondary analysis of data from an ongoing multiprovincial prospective cohort study, we studied 264 neonates undergoing complex cardiac repair. CS-AKI was defined based on the Acute Kidney Injury Network (AKIN) definition. We used regression modeling and survival analysis (adjusting for covariates) to evaluate associations. RESULTS: CS-AKI occurred in 64% of the neonates in our study cohort. Lower age, longer cardiopulmonary bypass time, hypothermic circulatory arrest, type of repair, lower preoperative serum creatinine (SCr) level, lower gestational age, and preoperative ventilation were independent risk factors for developing CS-AKI. Neonates with CS-AKI had longer times to extubation, intensive care discharge, and hospital discharge, after adjusting for covariates. Mortality was significantly increased in neonates with AKIN stage 2 or higher CS-AKI. The neonates with CS-AKI had a lower z-score for height at 2-year follow-up and were seen by more specialists. CONCLUSION: Neonatal CS-AKI is common and independently predicts important clinical outcomes, including mortality. Many risk factors are similar to those in older children, but some are unique to neonates. The observation that lower baseline SCr predicts CS-AKI merits further study. The AKIN definition, based on preoperative SCr value, is a reasonable method for defining CS-AKI in neonates. Many previous studies of CS-AKI have excluded neonates; we suggest that future intervention studies on approaches to reducing CS-AKI incidence and improving outcomes should include neonates.

Right ventricular and tricuspid valve remodeling after bidirectional cavopulmonary anastomosis.

BACKGROUND: There are few investigations of the changes in tricuspid valve (TV) and right ventricular (RV) morphology following bidirectional cavopulmonary anastomosis (BCPA). METHODS AND RESULTS: The 2-D echocardiograms of 35 children (male, n=23; female, n=12; median age, 6 months; range, 3-10 months) with hypoplastic left heart syndrome, 1 month before and after BCPA performed between 2005 and 2011, were retrospectively reviewed. Patients who underwent TV repair at BCPA were excluded. From the 4-chamber view, the coaptation length, vena contracta width and RV end-diastolic area before and after BCPA were measured and indexed to surface area. The severity of tricuspid regurgitation was graded qualitatively. After BCPA, RV end-diastolic area decreased from 2,951 ± 584 to 2,580 ± 591 mm(2)/m(2) (P<0.001). The coaptation length of the anterior leaflet (8.8 ± 5.8 vs. 11.0 ± 6.2 mm/m(2), P=0.0014) and of the septal leaflet (13.5 ± 5.3 vs. 15.8 ± 5.4mm/m(2), P=0.0072) increased after BCPA. The vena contracta width decreased (5.8 ± 4.9 vs. 4.3 ± 4.2 mm/m(2), P=0.035), although there was no change in tricuspid regurgitation grade after BCPC (1.4 ± 0.7 vs. 1.4 ± 0.9, P=0.234). CONCLUSIONS: In children with hypoplastic left heart syndrome after BCPA, the coaptation length of the anterior and septal leaflets of the TV improved concomitantly with vena contracta width and RV end-diastolic area despite unchanged tricuspid regurgitation grade. This suggests that favorable RV and TV remodeling accompanies the reduction in RV volume load following BCPA.