Impact of Maternal-Fetal Environment on Outcomes Following the Hybrid Procedure in the Single Ventricle Population.

Treatment of infants with hypoplastic left heart syndrome (HLHS) remains challenging, and those affected remain with significant risks for mortality and morbidity throughout their lifetimes. The maternal-fetal environment (MFE) has been shown to affect outcomes for infants with HLHS after the Norwood procedure. The hybrid procedure, comprised of both catheterization and surgical components, is a less invasive option for initial intervention compared to the Norwood procedure. It is unknown how the MFE impacts outcomes following the hybrid procedure. This is a single-center, retrospective study of infants born with HLHS who underwent hybrid palliation from January 2009 to August 2021. Predictor variables analyzed included fetal, maternal, and postnatal factors. The primary outcome was mortality prior to Stage II palliation. We studied a 144-subject cohort. There was a statistically significant difference in mortality prior to stage II palliation in infants with prematurity, small for gestational age, and aortic atresia subtype (p < 0.001, p = 0.009, and p = 0.008, respectively). There was no difference in mortality associated with maternal diabetes, hypertension, obesity, smoking or illicit drug use, or advanced maternal age. State and national area deprivation index scores were associated with increased risk of mortality in the entire cohort, such that infants born in areas with higher deprivation had a higher incidence of mortality. Several markers of an impaired MFE, including prematurity, small for gestational age, and higher deprivation index scores, are associated with mortality following hybrid palliation. Individual maternal comorbidities were not associated with higher mortality. The MFE may be a target for prenatal counseling and future interventions to improve pregnancy and neonatal outcomes in this population.

Tricuspid Valve and Right Ventricular Function Throughout the Hybrid Palliation Strategy for Hypoplastic Left Heart Syndrome and Variants.

BACKGROUND: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy. METHODS: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed. RESULTS: The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, P = .5260; RV dysfunction: OR = 0.02-0.13, P = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, P = .0356; RV dysfunction: OR = 3.84, P = .0262). Transplant-free survival at 15 years was 44.8%. CONCLUSION: Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.

Incidence and impact of acute kidney injury in patients with hypoplastic left heart syndrome following the hybrid stage 1 palliation.

OBJECTIVE: Acute kidney injury leads to worse outcomes following paediatric cardiac surgery. There is a lack of literature focusing on acute kidney injury after the Hybrid stage 1 palliation for single ventricle physiology. Patients undergoing the Hybrid Stage 1, as a primary option, may have a lower incidence of kidney injury than previously reported. When present, kidney injury may increase the risk of post-operative morbidity and mortality. METHODS: A retrospective, single centre review was conducted in patients with hypoplastic left heart syndrome who underwent Hybrid Stage 1 from 2008 to 2018. Acute kidney injury was defined as a dichotomous yes (meeting any injury criteria) or no (no injury) utilising two different criteria utilised in paediatrics. The impact of kidney injury on perioperative characteristics and 30-day mortality was analysed. RESULTS: The incidence of acute kidney injury is 13.4-20.7%, with a severe injury rate of 2.4%. Patients without a prenatal diagnosis of hypoplastic left heart syndrome have a higher incidence of kidney injury than those prenatally diagnosed, (40% versus 14.5%, p = 0.024). Patients with acute kidney injury have a significantly higher incidence of 30-day mortality, 27.3%, compared to without, 5.6% (p = 0.047). DISCUSSION: The incidence of severe acute kidney injury after the Hybrid Stage 1 palliation is low. A prenatal diagnosis may be associated with a lower incidence of kidney injury following the Hybrid Stage 1. Though uncommon, severe acute kidney injury following Hybrid Stage 1 may be associated with higher 30-day mortality.

Pediatric Fontan Associated Liver Disease: Non-invasive Evaluation with Serologic Markers and Acoustic Radiation Force Impulse (ARFI) Elastography.

Hepatic fibrosis is a significant complication in adult Fontan patients suggesting development as a function of time since the surgery. Children with Fontan circulation are not routinely assessed for development of liver disease. We aimed to evaluate the effectiveness of serologic biomarkers and acoustic radiation force impulse (ARFI) elastography to detect liver disease in pediatric Fontan patients. Patients ≥ 1 year after Fontan operation prospectively had hepatic US with acoustic radiation force impulse and laboratory testing. Clinical cardiac data (echocardiograms, cardiac catheterizations) were reviewed. Statistical analysis was performed using Pearson's correlation coefficient, Wilcoxon rank-sum test and Kruskal-Wallis test. Forty patients were enrolled with median age of 11 years and median time since Fontan of 6.5 years. Platelet count negatively correlated with years since Fontan (p < 0.000). Thrombocytopenia was noted in 15% of patients with the lowest platelet count of 78 K/cu mm, in a patient >10 years from the Fontan (DORV) operation. Alanine transaminase (ALT, p = 0.034) and aspartate aminotransferase (AST, p = 0.009) were higher in patients with Extracardiac Conduit Fontan and not in other Fontan operations. Heterogeneous echotexture on liver ultrasound correlated with years since Fontan (p = 0.022), however all acoustic radiation force impulse values were elevated (> 1.34 m/s) and did not correlate with age, years since Fontan, labs or imaging. FibroSure values did not correlate with years since Fontan. This suggests that ARFI may be elevated due to passive hepatic congestion, limiting its value in this patient population. Additional testing is necessary to identify reliable noninvasive screening modalities for hepatic fibrosis in Fontan patients. Our study is the largest pediatric study to evaluate ARFI in patients after the Fontan operation and showed increased shear wave speed for all patients with no correlation with time since palliation. Decreasing platelet count may indicate the development of liver fibrosis.

Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation.

OBJECTIVE: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period. DESIGN: Retrospective analysis. SETTING: The Heart Center at Nationwide Children's Hospital, Columbus, Ohio. PATIENTS: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution. INTERVENTIONS: All patients underwent hybrid stage 1 palliation. OUTCOME MEASURES: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality. RESULTS: Study group comprised 57 patients. Five patients only admitted once during the interstage period for scheduled cardiac catheterization were included in the no readmission group. Therefore, 43 patients (75%) had a total of 87 interstage readmissions. Fourteen patients had 15 major interstage adverse events accounting for 17% of total readmissions. Stroke (n = 1); sepsis (n = 1); pericardial effusion requiring drainage (n = 1); mesenteric ischemia (n = 1); shock (n = 1); and cardiac catheterization requiring intervention (n = 11)-ductal stent balloon angioplasty (n = 3), enlargement of atrial septal defect/stent placement (n = 3), retrograde aortic arch stenosis (n = 4). Thirty-three readmissions were secondary to gastrointestinal/feeding issues; 15 cyanosis; 15 work of breathing; and 9 asymptomatic patients. Four patients suffered interstage deaths (7%). Five patients (9%) spent >30 days in the hospital during the interstage period. Of the 47 newborns (82%) discharged exclusively orally feeding, 74% remained all orally feeding throughout interstage period. No patient discharged with tube feedings learned to eat during the interstage period. CONCLUSION: Interstage readmissions are common in the hybrid patient population. Seventeen percent were secondary to major adverse events. Interstage mortality was 7%. Future studies to identify interventions aimed at decreasing feeding issues and viral bronchiolitis in this tenuous patient population will hopefully improve quality outcomes, reduce readmissions, and lessen health care costs.

Pediatric marginal donor hearts: Trends in US national use, 2005-2014.

Pediatric patients awaiting heart transplant face high mortality rates due to donor organ shortages, including non-use of marginal donor hearts. We examined national trends in pediatric marginal donor heart use over time. UNOS data were queried for heart donors <18 years from 2005 to 2014. The proportion of donor hearts considered marginal was determined using previously cited marginal characteristics: left ventricular ejection fraction (LVEF) <50%, use of ≥2 inotropes, cerebrovascular death, CDC high-risk status, and eGFR < 30 mL/min/1.73 m2 . Disposition of donor hearts was determined and stratified by marginal donor status. Of 6778 pediatric hearts offered from 2005 to 2014, 2373 (35.0%) were considered marginal. Non-use of marginal donor hearts was significantly higher than that of donor hearts without any marginal characteristics (59.5% vs 20.3%, P < .001). In particular, LVEF < 50% and donor inotropes were associated with high rates of organ non-use among pediatric donors. Yet, non-use of marginal donor organs decreased from 67% to 48% from 2005 to 2014 (P < .001). Although the proportion of pediatric donor hearts used for pediatric patients has increased, more than half of donor hearts are declined for use in pediatric recipients due, in part, to perceived marginal status.

Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results.

INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving outcomes and quality of life for children with single ventricle heart defects, all functioning within the medical home of our heart center. Standards of care were developed and implemented in five target areas to standardize medical management and patient and family support. Under the team 100 patients have been cared for. Since 2014 a decrease in interstage mortality for HLHS were seen. Using a team approach and the tools of Quality Improvement they have been successful in reaching high protocol compliance for each of these areas. CONCLUSIONS: This article describes the process of building a successful Single Ventricle team, our initial results, and lessons learned. Additional study is ongoing to demonstrate the effects of these interventions on patient outcomes.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

BACKGROUND: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC). METHODS AND RESULTS: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow-up. Two analyses were performed: (1) propensity-score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site-size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post-S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04). CONCLUSIONS: Among SVD infants in the NPCQIC database discharged post-S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Management strategies after cardiac surgery in an infant with human rhinovirus.

This report presents management strategies after cardiopulmonary bypass surgery for an infant with community-acquired rhinovirus bronchiolitis. The case report emphasizes human rhinovirus as a lower respiratory pathogen, the difficulty treating the complications of human rhinovirus for an infant with cyanotic congenital heart disease after cardiac surgery, and the importance of preoperative evaluation for this respiratory infection in high-risk infants before cardiac surgery.

Acute kidney injury after cardiac surgery in infants and children: evaluation of the role of angiotensin-converting enzyme inhibitors.

Children with congenital heart disease who undergo cardiac surgery are vulnerable to acute kidney injury (AKI). This study sought to evaluate the role of angiotensin-converting enzyme (ACE) inhibitors and other nephrotoxic medications in the risk for the development of AKI in neonates and children undergoing cardiac surgery. A retrospective review of all patients younger than 2 years admitted to the cardiac intensive care unit after cardiac surgery from March 2007 to September 2008 was conducted. Patients were included in the review if they received furosemide alone or in combination with an ACE inhibitor. Creatinine clearance was calculated, and the patient's maximal degree of AKI was classified by pRIFLE. A P value less than 0.05 was considered significant. Of the 319 patients who met the inclusion criteria, 149 (47%) received furosemide therapy alone and 170 (53%) received a combination of furosemide and an ACE inhibitor. Patients in the furosemide-only group (age, 5 months) were older than the patients who received both furosemide and an ACE inhibitor (age, 3.8 months; P = 0.024). Despite statistically higher Aristotle scores in the ACE-inhibitor group, the intraoperative variables did not differ between the two groups. Postoperatively, the ACE-inhibitor group had a decreased creatinine clearance (55.3 ml/min/1.73 m(2)) compared with the furosemide group (64.4 ml/min/1.73 m(2); P = 0.015) and an increased incidence of a pRIFLE maximal score of "F" (odds ratio [OR], 1.75; P = 0.033). However, after adjustment for additional risk factors, no difference in the occurrence of AKI resulted (OR, 0.939; P = 0.85) when patients received an ACE inhibitor. More than half of the study population received ACE inhibitors, but this treatment was not associated with an increase in AKI.

A curious isolated cystic lesion of the membranous atrioventricular septum.

We present the case of an isolated cystic lesion of the atrioventricular component of the membranous septum of unclear aetiology, but responsible for cardiomegaly and benign disturbances of cardiac rhythm. As far as we are aware, this type of lesion has not previously been documented.