FibroSURE and elastography poorly predict the severity of liver fibrosis in Fontan-associated liver disease

Objective: As the population of patients with Fontan circulation surviving into adult‐ hood increases, hepatic cirrhosis has grown to be a significant cause of morbidity and mortality. Early detection of advanced hepatic fibrosis is imperative for proper inter‐ vention and consideration for heart or combined heart/liver transplantation. Noninvasive biomarkers and elastography have been evaluated for their diagnostic utility with variable results in the Fontan population.
Design: The cohort included 14 patients age 26.4 SD 7.5 who underwent Fontan surgery. All patients were evaluated with FibroSURE, shear wave elastography (SWE), hepatic duplex sonography, and liver biopsy. Liver fibrosis on biopsy was evaluated according to the congestive hepatic fibrosis system.
Results: In our cohort, 100% of patients had fibrosis with 36% demonstrating ad‐ vanced fibrosis. FibroSURE agreed with liver biopsy in only 5 out of 14 cases (36%): underestimating in 7 and overestimating in 2 individuals. SWE agreed with liver bi‐ opsy in 0% of cases: overestimating in 10 and underestimating in 4 cases. None of the duplex sonography indices predicted the presence or severity of liver fibrosis.
Conclusion: This study demonstrates that children who have undergone a Fontan procedure universally develop some hepatic fibrosis and a significant number have advanced fibrosis by adulthood. The FibroSURE blood test, SWE, and hepatic duplex sonography were unable to accurately predict the presence or severity of hepatic fibrosis when compared with liver biopsy. Further studies are needed to investigate novel noninvasive methods and/or biomarkers that can adequately detect advanced hepatic fibrosis before the development of cirrhosis and hepatic decompensation.     

Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

Purpose: Patients who have undergone Fontan palliation of single ventricle physiol‐ ogy congenital heart disease are prone to developing focal liver lesions. In our experi‐ ence, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy‐ proven liver lesions in patients post–Fontan palliation of single ventricle physiology cared for at our institution.
Methods: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for lesion pathology, background liver findings, pertinent laboratory data, and demographic data. CT and MRI images were reviewed to describe imaging findings of the reported lesions.
Results: Twelve patients met inclusion criteria; 58% (7/12) of which were female. Fifteen lesions were biopsied including four macroregenerative/benign hepatocellu‐ lar hyperplastic nodules, two hepatocellular adenomas, two hepatocellular carcino‐ mas, two intrahepatic cholangiocarcinoma (in the same patient), one venous malformation, and one focus of vascularized scar tissue. Two additional lesions in patients postcardiac transplant were posttransplant lymphoproliferative disorder.
Conclusion: Patients who have undergone Fontan palliation of single ventricle physi‐ ology are prone to develop a variety of liver lesions, both benign and malignant.     

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
Results: We identified 118 children with T21 who underwent initial surgical SV pallia‐ tion. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital sur‐ vival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
Conclusions: Children with T21 and SV are at high risk for procedural and long‐term mortality related to their genetic condition and underlying CHD. Nevertheless, a se‐ lect group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.     

Assessing hepatic impairment in Fontan‐associated liver disease using the HepQuant SHUNT test

Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances adjusted for body mass) of orally and intra‐ venously administered cholates labeled with deuterium or 13C. The SHUNT param‐ eter of the test measures portal systemic shunting from the ratio of Systemic HFR to Portal HFR. Chart review included laboratory tests, imaging, and clinical findings. Data from FALD patients were compared with data from healthy controls.
Results: The average DSI and SHUNT values for the FALD patients were 17.5% and 36.1%, respectively, compared to 9.2% and 24.1%, respectively, for controls. Twelve (85.7%) FALD patients had a DSI >15 (upper limit of normal). Seven (50.0%) FALD patients had SHUNT values >30% (upper limit of normal), while three FALD patients (21.4%) had SHUNT values >49%. One FALD patient with preoperative SHUNT of 69%, who underwent a combined heart‐liver transplant, had confirmed cirrhotic mor‐ phology within the liver explant.
Conclusions: This pilot study demonstrated that most FALD patients had hepatic im‐ pairment detected by abnormal DSI, with a smaller number having markedly elevated SHUNT values >49% suggesting intrinsic liver disease. The HepQuant SHUNT test may be useful in detecting and quantifying liver disease severity in FALD patients.     

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced expiratory volume in one second (FEV₁) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV₁ was <80%pred., and all of these patients had FEV₁/forced vital capacity (FVC) > 80%, suggestive of a restric‐ tive ventilatory pattern. Reduced FEV₁ was associated with a reduced peakVO₂ of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible con‐ founding factors (sex, BMI, age, years after palliation, number of interventions, sco‐ liosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV₁ was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV₁ was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long‐term complications.
Conclusions: This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.     

Detection of arrhythmias in adult congenital heart disease patients with LINQTM implantable loop recorder

Background: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhyth‐ mias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQ^TM, have been useful in long‐term monitoring for arrhythmias in adults with cryptogenic stroke.
Objective: We propose the Reveal LINQ^TM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients.
Methods: This is a single center retrospective review of Reveal LINQ^TM use in ACHD patients from 2014‐2017. Medical records were reviewed to determine cardiac diag‐ nosis, indication for implant, ILR findings, and changes in management.
Results: Twenty‐two patients, median age 25 years, underwent ILR implantation. ILR findings resulted in change in management in nine (41%) patients. One‐third (3/9) of the patients with clinically relevant events were asymptomatic. Patients with Fontan palliation had the highest number of pertinent positive events (57%). ACHD physi‐ ologic class D patients were more likely to have a positive finding (P = .034) compared to other physiologic classes. Majority (75%) of patients with positive events had ar‐ rhythmias documented on ILR which were not demonstrated on prior Holter/event monitors. Pertinent negative event occurred in one patient with Fontan palliation (5%) who had syncope corresponding to sinus rhythm.
Conclusion: ILRs are a useful adjunct for arrhythmia monitoring in the ACHD popula‐ tion with clinically relevant events in 41% of patients. A special consideration for ILRs could be made for high‐risk asymptomatic patients.     

Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization

Background: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anat‐ omy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.
Objectives: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation were analyzed.
Results: Type of feeding method remained relatively unchanged during hospitalization for stage 2 palliation. Gastrostomy tube fed only patients were the oldest at time of surgery (182.7 ± 57.7 days, P < .001) and had the lowest weight‐for‐age z scores at ad‐ mission (−1.6 ± 1.4, P < .001). Oral + gastrostomy tube groups had the longest median bypass times (172.5 minutes, P = .001) and longest length of stay (median 12 days, P < .001). Multivariable modeling revealed that feeding by tube only (P < .001), oral + tube feeding (P ≤ .001), reintubation (P < .001), and prolonged intubation (P < .001) were associated with increased length of stay. Neither age (P = .156) nor weight‐for‐age z score at admission (P = .066) was predictive of length of stay.
Conclusions: Feeding methods established at admission for stage 2 palliation are not likely to change by discharge. Length of stay is more likely to be impacted by tube feeding and intubation history than age or weight‐for‐age z score at admission. Better understanding for selection of feeding methods and their impact on patient out‐ comes is needed to develop evidence‐based guidelines to decrease variability in clini‐ cal practice patterns and provide appropriate counseling to caregivers.     

Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation

Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the pa‐ tient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less fre‐ quent in low‐risk Fontan patients compared to high‐risk Fontan patients. Counseling practices were similar for both low‐risk and high‐risk Fontan patients. Aspirin mono‐ therapy was recommended by 82% of providers for low‐risk Fontan patients, while anticoagulation regimens were more varied for the high‐risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low‐risk and high‐risk Fontan patients. There were no other major differences in test‐ ing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice.
Conclusion: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence‐based guidelines to streamline the approach to manage these complex patients.     

Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age 9 months (inter‐ quartile range, 5‐14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time‐to‐pallia‐ tion of 4 years (interquartile range, 3‐5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five‐ and 10‐year transplant‐free survival were 91% and 84%, respectively. At a median follow‐up of 6 years (interquartile range, 2‐8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3‐11], oxygen sat‐ uration 83% ± 4%). Preoperative moderate‐severe atrioventricular valve regurgita‐ tion (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80‐33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08‐6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for co‐ variates. Reaching further palliation was less likely in patients with preoperative moderate‐severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08‐0.59; P =.002).
Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circum‐ stances, these patients can have a favorable long‐term prognosis without reducing their suitability for further palliation.     

Fate of the Fontan connection: Mechanisms of stenosis and management

Background: Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percuta‐ neous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.
Methods: Retrospective review of all Fontan patients who had cardiac catheteriza‐ tion from January 2002 to October 2018 was performed. Hemodynamic and angio‐ graphic data that assessed extra‐ or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated.
Results: Twenty patients underwent catheter intervention because of conduit steno‐ sis, including calcified homografts, stenotic Gore‐Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revi‐ sion. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P < .0001]. Fontan conduit/connection di‐ ameter increased [10.5 mm (IQR 9; 12) vs 18 mm (IQR 14.9; 18); P < .0001] and New York Heart Association class [III (IQR II; III) vs I (IQR II; III); P = .03) with stent placement.
Conclusions: We demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, sten‐ otic Gore‐Tex conduits and lateral tunnels pathways can be safely and effectively stented to eliminate obstruction. Percutaneous stenting is associated with a decrease in connection gradients and improvement in functional capacity.     

Arteriovenous fistula creation for hypoxia after single ventricle palliation: A single‐institution experience and literature review

Background: Hypoxia is a common and sometimes severe morbidity of single ven‐ tricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connec‐ tion (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previ‐ ous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO₂) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution.
Methods: We conducted a retrospective chart review of patients with a history of single ventricle palliation and history of surgical AVF creation who were seen at our tertiary care center from 1996 to 2017.
Results: A total of seven patients were included in our study. SpO₂ for the overall co‐ hort did not significantly increase after AVF creation (pre‐AVF 79.1 ± 6.9%, post‐AVF 82.7 ± 6.0% [P = .23]). SpO₂ trended up for large shunts (>5 mm) (pre‐AVF 75.0 ± 7.6%, post‐AVF 84.0 ± 5.3% [P = .25]). SpO₂ did not improve for small shunts (≤5 mm) (pre‐ AVF 82.3 ± 6.5%, post‐AVF 81.0 ± 8.5% [P = .50]). The 12‐month overall and transplant‐ free survival were 85.7% and 71.4%, respectively. Freedom from AVF‐related compli‐ cation (cephalic edema, thrombotic occlusion) was 51.4% at 12 months.
Conclusion: Palliative AVF creation for patients with single ventricle CHD and hy‐ poxia does not universally improve SpO₂ and is prone to early complications. Despite a lack of durable benefit and known risks, AVF creation remains a reasonable pallia‐ tion for a subset of patients after SCPC who are not candidates for TCPC, or poten‐ tially as a bridge to heart transplantation.     

Body mass index in adults with congenital heart disease

Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise test.
Results: Adults with CHD had slightly lower BMI than the reference group (24.1 ± 4.3 vs 24.6 ± 4.3; P = .012). Men in the mild and severe group (23.9 ± 3.6; 23.3 ± 4.4 vs 25.1 ± 3.7; P = .007; P = .023) and women in the severe group (21.6 ± 3.3 vs 24.2 ± 4.7; P < .001) had lower BMI compared to the reference group. In the subgroups, men with ventricular septal defect, coarctation of aorta/ventricular septal defect and Fontan cir‐ culation and women with Fontan circulation had lower BMI than the reference group. Underweight was more prevalent in women with severe lesions compared to the refer‐ ence group (22.2% vs 3.8%; P < .001). BMI was associated with age and exercise ca‐ pacity in patients with mild and moderate lesions, while higher BMI was related to better ventricular function in women with Fontan circulation.
Conclusion: Underweight was more prevalent in ACHD patients with severe lesions. Special attention should be paid to the possible existence of underweight‐related comorbidities.     

Safety of contraceptive use among women with congenital heart disease: A systematic review

Objective: Women with congenital heart disease (CHD) are at increased risk of preg‐ nancy complications and need information on safe, effective contraceptive methods to avoid unintended pregnancy. This systematic review examines evidence regarding safety of contraceptive use among women with CHD.
Methods: The PubMed database was searched for any peer‐reviewed articles pub‐ lished through April 2018 that included safety outcomes associated with reversible contraceptive methods among women with CHD.
Results: Five articles met inclusion criteria: three studies comparing contraceptive users to nonusers and two noncomparative studies. Sample sizes ranged from 65 to 505 women with CHD. Two studies found a higher percent of thromboembolic com‐ plications among women with Fontan palliation or transposition of the great arteries using oral contraceptives. One study, among women with Fontan palliation, found no increased risk of thromboembolic complications between contraceptive users (not separated by type) and nonusers. Two studies found no endocarditis among intrau‐ terine device users.
Conclusions: There is a paucity of data regarding the safety of contraceptive meth‐ ods among women with CHD. Limited evidence suggests an increased incidence of thromboembolic complications with use of oral contraceptives. Further studies are needed to evaluate contraceptive safety and quantify risk in this growing population. There is also limited data regarding the safety of contraceptive methods among women with CHD. Further information is needed to assist practitioners counseling women with CHD on safety of contraceptive methods.     

Pacemaker treatment after Fontan surgery—A Swedish national study

Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indica‐ tions in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.
Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).
Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower preva‐ lence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atre‐ sia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventri‐ cle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively).
Conclusions: Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV.     

Reintervention following stage 1 palliation: A report from the NPC‐QIC Registry

Background: Single ventricle heart disease with aortic arch hypoplasia has high mor‐ bidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter‐based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re‐intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC‐QIC) registry.
Methods: The NPC‐QIC registry, consisting of patients discharged after stage 1 pal‐ liation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was re‐intervention.
Results: Of 1156 patients, (50%) had re‐intervention. There was no difference in total rate of re‐intervention by shunt type (BT shunt 52% vs. RVPA shunt 48%; P = .17). Patients with a BT shunt had increased re‐intervention during stage 1 hospi‐ talization (P =.002). During the interstage period, following discharge from stage 1 palliation, patients with a BT shunt had increased aortic arch re‐intervention (P < .005), while patients with an RVPA shunt had increased re‐intervention on the shunt and the pulmonary arteries (P = .02). Postoperative mechanical ventilation >14 d (P < .01) was the only risk factor associated with re‐intervention by multivariable analysis, regardless of shunt type.
Conclusions: Re‐intervention between stage I and stage 2 palliation is common. There is no difference in cumulative frequency of re‐intervention between shunt types, though types and timing of re‐intervention varied between shunt types. Longitudinal assessment of the NPC‐QIC database is important to identify long term outcomes of patients requiring re‐intervention.     

More than the heart: Hepatic,renal, and cardiac dysfunction in adult Fontan patients

Setting: Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI.
Objective: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores.
Design: Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD‐XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort.
Results: Fifty‐seven patients had congruent lab and catheterization data for analysis. Sixty‐three and sixty‐nine patients had congruent lab and echocardiogram data for MELD and MELD‐XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD‐XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD‐XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD‐XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02).
Conclusions: In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD‐XI scores.     

Comparison of the diagnostic quality of aspiration and core-biopsy needles for transjugular liver biopsy

BackgroundLiver biopsy remains essential for the diagnostic work-up of patients with liver disease.AimsTo evaluate aspiration vs. core-biopsy needles for transjugular liver biopsy (TJLB) in patients undergoing hepatic venous pressure gradient (HVPG) measurements.Methods84 patients undergoing TJLB between 06/2017 and 12/2018 were prospectively included. Liver biopsy specimens were systematically evaluated for quantitative and qualitative criteria such as number of portal tracts, sample length and fragmentation.ResultsIn direct comparison of paired TJLB specimens (n=35), core-biopsy samples were significantly longer (median 12 vs. 9mm, p=0.012), tended to contain more portal tracts (median 8 vs. 6, p=0.064) and were less fragmented (p<0.001), which resulted in better confidence for liver fibrosis assessment (p=0.035). However, a superior quality in terms of less fragmentation of core-biopsy specimens (p<0.05) was only confirmed in patients with HVPG ≥10mmHg or liver stiffness measurement >40kPa. In contrast, the aspiration needle provided significantly longer samples in patients with HVPG <10mmHg (median 21 vs. 12mm, p=0.007) or with liver stiffness measurement <20kPa (median 21 vs. 11mm, p=0.025).ConclusionIn patients with HVPG ≥10mmHg, we recommend to performed TJLB using core-biopsy needles, while the aspiration needle provides high quality liver biopsy specimens in patients with HVPG <10mmHg.     

Transfusion‐related acute hepatic injury following postoperative platelets administration in pediatric patients undergoing the Fontan procedure

Objective: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.
Design: Single center retrospective cohort study.
Setting: Pediatric Cardiac Intensive Care Unit at a tertiary medical center.
Patients: Ninety‐nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.
Interventions: None.
Measurements and Main Results: Out of the four types of blood products, transfusion of platelets was found to significantly affect postoperative levels of ALT and AST. Additional factors included postoperative administration of sodium bicarbonate, decreased flow through the Fontan canal and decreased urine output. Preoperative pulmonary artery pressure and pulmonary vascular resistance, cardiopulmonary bypass time, aortic cross‐clamp time, amount of postoperative bleeding, and vasoactive‐ inotropic score did not influence liver enzymes levels.
Conclusions: In pediatric Fontan patients, platelets transfusions contribute to an acute hepatic injury. The relation between platelets and transfusion‐related acute lung injury (TRALI) has been well described, but this is the first time it is being described in regard to acute hepatic injury (TRAHI). Changing platelet transfusion strategy could decrease morbidity in Fontan patients but further research is needed. KEYWORDS acute hepatic injury, congenital heart defect, Fontan, pediatric cardiac surgery, platelets, transfusion‐related acute hepatic injury     

S100B and its relation to cerebral oxygenation in neonates and infants undergoing surgery for congenital heart disease

Objectives: Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic‐ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astro‐ cyte protein S100B and its relation to cerebral oxygenation.
Methods: Serum S100B was measured before and 0, 12, 24, and 48 hours after sur‐ gery. Cerebral oxygen saturation was derived by near‐infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th per‐ centile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in the normal range. Neonates (≤28 days) and infants (>28 and ≤365 days) were analyzed separately due to age‐dependent release of S100B.
Results: Seventy‐four patients underwent 94 surgical procedures (neonates, n = 38; infants, n = 56). S100B concentrations were higher in neonates before and after sur‐ gery at all time points (P ≤ .015). Highest values were noticed immediately after sur‐ gery. Postoperative S100B was elevated after 15 (40.5%) surgeries in neonates. There was no difference in pre‐, intra‐, or postoperative cerebral oxygenation. In in‐ fants, postoperative S100B was elevated after 23 (41.8%) procedures. Preoperative cerebral oxygen saturations tended to be lower (53 ± 12% vs 59 ± 12%, P = .069) and arterial‐cerebral oxygen saturation difference was higher (35 ± 11% vs 28 ± 11%, P = .018) in infants with elevated postoperative S100B. In the early postoperative course, cerebral oxygen saturation was lower (54 ± 13% vs 63 ± 12%, P = .011) and arterial‐cerebral oxygen saturation difference was wider (38 ± 11% vs 30 ± 10%, P = .008). Cerebral oxygen saturation was also lower for the entire postoperative course (62 ± 18% vs 67 ± 9%, P = .047).
Conclusions: Postoperative S100B was elevated in about 40% of neonates and in‐ fants undergoing cardiac surgery. Infants with elevated postoperative S100B had impaired perioperative cerebral tissue oxygenation. No relation between S100B and cerebral oxygenation could be demonstrated in neonates.     

The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data set

Objective: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.
Design: Retrospective study design.
Setting: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
Patient: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
Intervention: None.
Outcome measures: Mortality was defined as death, listed for transplant, or transplanted after stage 2 palliation. Morbidity was evaluated as hospital length of stay and duration of intubation.
Results: A total of 283 patients met criteria for analysis. Of those, only 18 patients failed stage 2. Right ventricular fractional area change was less in those who failed (30% vs 34%, P = .039) and right ventricular indexed end‐diastolic volume and end‐ systolic volume were larger in those who failed (142.74 mL/ BSA^1.3 vs 111.29 mL/ BSA^1.3, P = .023, 88.45 mL/ BSA^1.3 vs 62.75 mL/ BSA^1.3, P = .025, respectively). Larger right ventricular indexed end‐diastolic and systolic volumes were associated with failure (OR 1.17 [1.01‐1.35] P = .021, OR 1.25 [1.03‐1.52] P = .021, respectively). Every 10% increase in RV ejection fraction had a 63% decrease in length of stay and a 68% decrease in duration of intubation (P = .014, and P = .039, respectively).
Conclusion: Patients with decreased right ventricular fractional area change and larger right ventricular indexed end‐diastolic and systolic volumes were more likely to fail stage 2 palliation. Those with preserved right ventricular function had a shorter hospital length of stay and duration of intubation. Echocardiographic measurements of right ventricular indices during the interstage period can be utilized to determine the prognosis following stage 2 palliation.