Palliation Of Congenital Heart Disease Research Articles

Non-invasive assessment of severe liver fibrosis in patients with Fontan-associated liver disease: The VALDIG-EASL FONLIVER cohort

Background & AimsFontan-type surgery is used as a palliation for congenital heart disease with univentricular physiology but may, in the long term, lead to advanced chronic liver disease. This study assessed the accuracy of conventional non-invasive models in assessing liver fibrosis and introduces a new risk score employing non-invasive tools. MethodsA prospective, cross-sectional, observational study was conducted across five European centers and encompassing all consecutive adult patients with Fontan circulation, liver biopsy and non-invasive tests (elastography, APRI, FIB-4, Fibrosis score, Doha, GUCI, and AAR). The primary outcome was the identification of severe liver fibrosis on biopsy. Multivariable logistic regression identified non-invasive predictors of severe fibrosis, leading to the development and internal validation of a new scoring model named the FonLiver risk score. ResultsIn total, 217 patients (mean [standard deviation] age, 27.9 [8.9] years; 50.7% males) were included. Severe liver fibrosis was present in 47.9% (95% CI 41.2%–54.5%) and correlated with a lower functional class, protein-losing enteropathy, and compromised cardiopulmonary and systemic hemodynamics. The final FonLiver risk score incorporated liver stiffness measurement using transient elastography and platelet count and demonstrated strong discrimination and calibration (area under the receiver operating curve [AUROC] of 0.81). The FonLiver risk score outperformed conventional prediction models (APRI, FIB-4, Fibrosis score, Doha, GUCI, and AAR), which all exhibited worse performance in our cohort (AUROC < 0.70 for all). ConclusionSevere liver fibrosis is prevalent in adults following Fontan-type palliation and can be effectively estimated using with the novel FonLiver risk score. This scoring system can be easily incorporated into the routine assessment of patients with Fontan circulation.

A Comparison of Ductal Stenting and Surgical Shunts for Infants with Duct-Dependent Pulmonary Blood Flow: The Impact of Single Versus Biventricular Repair Pathways on Outcomes.

Ductal stenting (DS) is an alternative to the Blalock-Taussig-Thomas Shunt (BTTS) as initial palliation for congenital heart disease with duct-dependent pulmonary blood flow (DDBPF). We sought to analyze the impact of intended single ventricle (SV) and biventricular (BiV) repair pathways on the outcome of DS and BTTS in infants with DDPBF. A single-center, retrospective comparison of infants with DDPBF who underwent either DS (2012-2022) or BTTS procedures (2013-2017). Primary outcomes included all-cause mortality and risk of unplanned re-intervention. Participants were divided into four groups: 1.SV with DS, 2.SV with BTTS, 3.BiV with DS, and 4.BiV with BTTS. Fifty-one DS (SV 45%) and 86 BTTS (SV 49%) procedures were undertaken. For those who had DS, mortality was lower in the BiV compared to SV patients (BiV: 0/28, versus SV: 4/23, p = 0.04). Compared to BiV DS, BiV BTTS had a higher risk of combined death or unplanned re-intervention (HR 4.28; CI 1.25-14.60; p = 0.02). In SV participants, there was no difference for either primary outcome based on procedure type. DS was associated with shorter intensive care length of stay for SV participants (mean difference 5days, p = 0.01) and shorter intensive care and hospital stay for BiV participants (mean difference 11days for both outcomes, p = 0.001). There is a survival benefit for DS in BiV participants compared with DS in SV and BTTS in BiV participants. Ductal stenting is associated with a shorter intensive care and hospital length of stay.

Personalized Approach in Transcatheter Palliation of Congenital Heart Disease with Duct-Dependent Pulmonary Circulation: Right Ventricular Outflow Tract Stenting vs. Arterial Duct Stenting.

Despite significant improvements in techniques, the treatment of neonates and infants with congenital heart disease resulting in duct-dependent pulmonary circulation is still significantly challenging. Despite current trends toward early primary surgical repair, temporary palliation is still necessary in those patients who are at high surgical risk for complete correction due to unfavorable clinical or anatomic characteristics. Recent advances in interventional cardiology have led to the emergence of right ventricular outflow tract and arterial duct stenting as cost-effective alternatives to surgical palliation in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated. This review aims to explore the evolving landscape of these transcatheter approaches, highlighting their role, efficacy and potential complications in the context of duct-dependent pulmonary circulation anatomies.

Feasibility of Digital Stethoscopes in Telecardiology Visits for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program. In addition to standard home-monitoring practice for TCV, caregivers received training on use of a DS (Eko CORE attachment assembled with Classic II Infant Littman stethoscope). Sound quality of the DS and comparability to in-person auscultation were evaluated based on two providers' subjective assessment. We also evaluated provider and caregiver acceptability of the DS. From 7/2021 to 6/2022, the DS was used during 52 TCVs in 16 patients (median TCVs/patient: 3; range: 1-8), including 7 with hypoplastic left heart syndrome. Quality of heart sounds and murmur auscultation were subjectively equivalent to in-person findings with excellent inter-rater agreement (98%). All providers and caregivers reported ease of use and confidence in evaluation with the DS. In 12% (6/52) of TCVs, the DS provided additional significant information compared to a routine TCV; this expedited life-saving care in two patients. There were no missed events or deaths. Use of a DS during TCV was feasible in this fragile cohort and effective in identifying clinical concerns with no missed events. Longer term use of this technology will further establish its role in telecardiology.

PO-03-201 VIRTUAL REALITY-BASED METHOD FOR TEACHING COMPLEX CONGENITAL HEART DISEASE ANATOMY TO EP TRAINEES – A PILOT STUDY

Developing an accurate and detailed 3D mental model of cardiac anatomy is critical for electrophysiology (EP) trainees. This can be challenging in certain forms of palliated congenital heart disease. Surgical repair of d-TGA via a Mustard or Senning procedure requires a complex baffling technique that is particularly difficult to visualize from a 3D perspective and creates a propensity for arrhythmias. Traditional teaching methods may be sub-optimal for visualizing the interaction between the conduction system and this unusual anatomic substrate. Due to its immersive, stereoscopic nature, virtual reality (VR) may provide a better learning platform in a collaborative environment. The purpose of this pilot study is to compare the experience of a collaborative, immersive VR-based educational environment with a traditional PowerPoint (PPT) method for teaching the 3D cardiac anatomy of patients who have undergone Mustard and Senning procedures to EP trainees. In collaboration with the Visible Heart Lab at the University of Minnesota and DOPL Technologies, LLC, we created an immersive VR environment that allows remotely shared viewing, manipulation, and virtual dissection of high-resolution digital 3D models of wax-infiltrated heart specimens (d-TGA s/p Mustard and Senning procedures) that were provided by Boston Children’s Hospital. Participants viewed the environment using Oculus Quest2 head-mounted displays (Meta, Menlow Park, CA, USA). Three EP trainees were recruited for the pilot study, which entailed a traditional PPT lecture followed by an interactive viewing of the models in the VR learning environment. Participants were asked to complete a pre/post-test based on the material taught then a Likert satisfaction survey adapted from previously validated usability instruments. The study participants’ mean pre-test score was 46.7% and post-test score was 76.7%, a 64.3% improvement. They globally rated the VR learning environment favorably on the Likert satisfaction survey and reported that they would like to use the VR learning environment in both didactic lecture series and for self-directed study (Figure 1). We demonstrate the feasibility of using a remote, shared, immersive VR-based education platform for teaching EP trainees cardiac anatomy related to Mustard and Senning procedures. This learning environment was favorably received. Further efforts are needed to expand the library of CHD defects and provide additional features to the learning environment.

Electroencephalographic changes in neurologically free patients with tetralogy of Fallot after surgical repair: a cross section study in Egyptian children

BackgroundSurgical correction of tetralogy of Fallot (TOF) is still one of the most frequently performed intervention in pediatric cardiac surgery. The occurrence of seizures after repair or palliation of congenital heart disease (CHD) is a marker for a central nervous system (CNS) injury and has been associated with adverse neurodevelopmental sequelae. The prognostic value of electroencephalogram (EEG) for outcome prediction is glowing in children with CHD undergoing open-heart surgery who are at risk for subsequent neurodevelopmental deficits. To our knowledge, this is the first study to detect the EEG changes in neurologically free TOF patients after surgical repairResultsOur study included 68 TOF cases and 32 sex- and age-matched control group; they were 66.0% males and 34.0% females. The mean age of the studied children was 11.41 ± 4.23 years. There was statistically significant difference between TOF patients and control group as regards alpha power and Delta% in left occipital region and Alpha% in right occipital region, with dominant alpha waves in patients’ right occipital region. None of our patients had epileptic waves. The duration after operation showed negative correlation with Delta% at left occipital region and positive correlation with Alpha% in right occipital region (p = 0.002, p = 0.044 respectively).ConclusionTOF cases showed changes in EEG parameters chiefly dominant alpha power and Delta% in left occipital region and dominant Alpha% in right occipital region. Duration after surgery correlated negatively with Delta% at left occipital region, and positively with Alpha% in the right occipital region. Subsequent assessment is recommended to study long-term hazards of these varied dominance of EEG waves in our patients, e.g., evaluation of the cognitive functions.

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75–264) and median number of TM visits/patient was 2 (range 1–5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0–2.5) vs. 0.4 (0–3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease

Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes. Sixteen patients underwent dilatable PAB-median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication-Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients-allowing for interval growth or even serving as the definitive palliation.

Cangrelor PK/PD analysis in post‐operative neonatal cardiac patients at risk for thrombosis

Unlabelled Box AbstractBackgroundSystemic‐to‐pulmonary artery shunt thrombosis is a significant cause of early postoperative mortality in neonates after palliation of congenital heart disease. In the context of thromboprophylaxis, an optimal therapeutic strategy has yet to be established before aspirin administration. Cangrelor, a fast‐acting, reversible P2Y12 inhibitor, may fill this unmet need. ObjectivesTo evaluate the pharmacokinetics (PK), pharmacodynamics (PD), and safety of cangrelor in neonates undergoing stage 1 palliation. MethodsThis prospective, open‐label, single‐arm study evaluated two cangrelor dosing cohorts following placement of a systemic‐to‐pulmonary artery shunt, right ventricle‐to‐pulmonary artery shunt, or ductal stent. Drug concentrations and platelet reactivity, assessed by light transmission aggregometry and in microfluidic assays (MF), were measured. ResultsTwenty‐two patients were consented and 15 received a 1‐hour infusion of cangrelor at either 0.5 µg/kg/min (cohort 1) or 0.25 µg/kg/min (cohort 2). Whereas the primary PD endpoint was achieved at the higher dose (ie, reduction in maximal platelet aggregation by ≥90% in 60% of participants), only 29% of those in cohort 2 attained this goal. Comparable and statistically significant results were obtained in MF assays (P < .0001 vs. baseline). Drug levels during infusion were 3‐fold higher in cohort 1 vs. cohort 2 (P < .001). Most participants (70%) had undetectable drug levels by 10 minutes postinfusion with full recovery in platelet function at 1 hour. No drug‐related bleeding events occurred. ConclusionsFavorable PK/PD properties of cangrelor 0.5 µg/kg/min dosing and safety profile warrant further evaluation in neonates following palliative cardiac procedures.

Abstract 14356: Initial Experience With Telemedicine for Interstage Monitoring in Infants With Palliated Congenital Heart Disease

Introduction: Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. We have previously described the mediated risk for this patient population through close home monitoring. Given high rates of intercurrent illness, geographic and sociodemographic disparities in access to care, and significant family stressors, we instituted a telecardiology (TC) program in 2019. Methods: All consecutive patients discharged home were monitored from neonatal operation or intervention to subsequent surgical palliation. All had home scales and pulse oximeters. TC visits (TCVs) were held monthly, and increased to bimonthly during the COVID-19 pandemic. TCVs were launched through the electronic medical record (EMR) through a mobile device, with a full-version EMR available for access to full records and efficient documentation. We tracked how TCVs identified clinical concerns, prevented unnecessary emergency room visits (ERV) and expedited in-person assessment. Results: Between 8/2019-5/2020, we conducted 60 TCV for 29 patients (16 had stage 1 palliation, 6 with aortopulmonary shunt or stent and 7 with biventricular physiology with shunt or stents. Initial median weight was 3.4 kg (range 2.3-5.4 kg). Median monitoring time was 199 days (75-264). Median visits per patient were 2 (1-5 V) with average length per visit of 20 minutes. Most scheduled visits were completed (90%); 5% declined, and 5% canceled. Parents reported satisfaction for 97% of visits. In 6 visits (10%) significant clinical findings were identified which avoided ERV or hospital admissions, 4 TCVs (7%) visits led to expedited outpatient assessments, 1 required hospitalization. There were no missed events nor deaths. Additional issues identified included changes to medication (23%), feedings (15%) and equipment malfunction (5%). Frequency of EDV decreased from median 0.4 (0-3.7) V/patient/month the prior year to 0 (0-2.5), p=0.0004]. Conclusions: TC in these high-risk patients is feasible and effective to identify clinical concerns, no missed events and decrease in ERV per patient. TC was particularly effective during the COVID-19 pandemic allowing for easy adaptation of care to ensure patient safety in this fragile cohort.

Medical and Invasive Management of Cardiac Thrombosis in Children with Congenital and Acquired Heart Disease

In this manuscript, we review the diagnosis, causes, and management of cardiac thrombosis in children with congenital and acquired heart disease. Indications for invasive management and surgery are discussed. The differential diagnostic considerations and management of cardiac thromboses will be reviewed, including those that occur as a consequence of repair and palliation of congenital heart disease. The definition of venous thromboembolism has recently been expanded to include thromboses within both the right and left heart chambers. Direct thrombin inhibitors (DTIs) and direct oral anticoagulants (DOACs) are emerging as treatment alternatives to the heparins and vitamin K antagonists for the management of thrombosis in pediatric heart disease. Thrombosis within the heart occurs as a consequence of abnormalities and alterations in blood flow resulting in stasis and turbulence. Children with acquired and congenital heart disease carry a number of risk factors in Virchow’s triad. Thrombosis can occur throughout any part of the cardiovascular system in congenital heart disease and after surgery for repair or palliation of defects.

Percutaneous liver biopsy in Fontan patients.

Patients who have undergone the Fontan operation for palliation of congenital heart disease with single-ventricle pathophysiology are at high risk for developing progressive liver fibrosis. Pathological assessment from percutaneous liver biopsy is central to the management of Fontan-associated liver disease, but liver biopsy in this vulnerable population poses unique challenges and potential risks. This retrospective study describes our experience with percutaneous liver biopsy performed to assess changes of Fontan-associated liver disease, with particular regard to procedural outcomes. Data from liver biopsy procedure reports, pathology reports, cardiac angiography pressure measurements and laboratory values of patients with single ventricle heart disease after the Fontan operation who underwent ultrasound-guided percutaneous liver biopsy performed in interventional radiology at a pediatric tertiary care center during a 3-year period were retrospectively analyzed. Sixty-eight liver biopsies were performed in 67 patients (mean age: 20.2years, range: 7.2-39years). The technical success rate was 100%, and tissue was adequate for assessing liver disease in 100% of the procedures, including biopsies performed with a single pass. Anticoagulation was routinely suspended before biopsy, and no cardiac complications were encountered due to this suspension. A coaxial biopsy system using an 18-gauge (G) full-core instrument through a 17-G introducer trocar was most commonly used, in 41/68 cases (60%). The most common trough length was 2.3cm, used in 37 cases (54%). One pass was made in 27 procedures (40%) and two passes in 30 (44%); tract embolization with gelatin sponge was performed in 52 (76%). The only complication was hemorrhage, which occurred in 5/68 (7.4%) of the biopsies, minor in four (5.9%) and major in one (1.5%) -- similar to rates reported for liver biopsy in non-Fontan patients. Hemorrhage had a delayed presentation in three of these five cases. Immediate post-biopsy hemoglobin decrease of ≥2mg/dL showed a low sensitivity for hemorrhage. The mean Fontan pressure measured during cardiac angiography was 13.8mmHg, and shunt pressures were not associated with an increased risk of hemorrhage. Percutaneous liver biopsy in Fontan patients can be performed safely with high technical success rates and without increased complication rates. Meticulous technique and close observation are recommended to reduce post-biopsy complications. The degree of right heart pressure elevation was not associated with hemorrhage.

MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation.

Focal liver lesions are common in children and adults after Fontan procedures. To explore the relationship between liver shear stiffness, using magnetic resonance (MR) elastography, and the presence of focal liver lesions in patients after Fontan procedures (total cavopulmonary anastomosis). The retrospective study was approved by the institutional review board and the requirement for informed consent was waived. By searching institutional electronic medical records, we identified all patients with a history of Fontan palliation of congenital heart disease who had undergone same-day liver MR elastography and liver MRI without and with intravenous contrast material between January 2012 and December 2017. Using imaging reports, patients were placed into two groups: 1) no focal liver lesions and 2) one or more focal liver lesions. Patient age, sex, mean liver shear stiffness (kPa) and maximum single anatomical level liver shear stiffness (kPa) were recorded. The Mann-Whitney U test was used to compare age and liver stiffness between groups, while the Fisher exact test was used to assess the impact of gender on liver lesions. Forty-eight patients met study inclusion criteria; 33 (69%) had one or more focal liver lesions. The median age was 20.0years (IQR [interquartile range]: 10.8-29.1years) for patients without liver lesions and 19.9years (IQR: 17.2-27.0years) for patients with liver lesions (P=0.49). Eleven of 21 male patients (52.4%) had liver lesions compared to 22 of 27 female patients (81.5%) (P=0.058). Mean (4.62kPa [IQR: 4.10-5.59kPa] vs. 4.10kPa [IQR: 3.44-4.80kPa]; P=0.02) and maximum (5.53kPa [IQR: 4.64-6.56kPa] vs. 4.50kPa [IQR: 3.82-5.35kPa]; P=0.009) liver stiffness were significantly higher in patients without liver lesions as compared to patients with liver lesions. Our study demonstrated a significant negative association between focal liver lesions and increased liver stiffness in patients following Fontan procedures.

Heart Transplant in Children: What a Primary Care Provider Needs to Know.

Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant. Many pediatric practitioners are faced with caring for these patients before as well as after the heart transplant and, therefore, they should be knowledgeable about basic principles related to heart transplant. There are certain complications that are unique to this population, and medication side-effects, evaluation and management of a febrile illness, opportunistic infections, vaccination, pregnancy, and exercise recommendations are areas that require special consideration. [Pediatr Ann. 2018;47(4):e172-e178.].

Diagnostic Performance of MR Elastography for Liver Fibrosis in Children and Young Adults with a Spectrum of Liver Diseases.

Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease. MR elastography examinations were reprocessed by a single observer, blinded to pathologic findings. Pathology specimens were reviewed by a single pathologist who scored steatosis (lipid in ≥ 5% of hepatocytes) and staged fibrosis. Receiver operating characteristic (ROC) curves were used to assess diagnostic performance. Results A total of 86 patients, 49 (57%) male with a median age of 14.2 years (range, 0.3-20.6 years), were included. Fifty-one patients (59.3%) had Ludwig stage 2 or higher fibrosis; 44 patients (51.2%) had hepatic steatosis. The area under the ROC curve for Ludwig stage 0-1 versus stage 2 or higher fibrosis was 0.70 (95% confidence interval [CI]: 0.59, 0.81) for the whole population and was significantly lower for patients with steatosis versus those without (0.53 [95% CI: 0.35, 0.71] vs 0.82 [95% CI: 0.67, 0.96], P = .014). Optimal stiffness cut-offs for the entire population were 2.27 kPa with 68.6% sensitivity (95% CI: 57.2%, 80.1%) and 74.3% specificity (95% CI: 63.5%, 85.1%) or 1.67 kPa with 35.3% sensitivity (95% CI: 23.5%, 47.1%) and 91.4% specificity (95% CI: 84.5%, 98.3%). Conclusion In children and young adults, MR elastography performs significantly better for distinguishing stage 0-1 versus stage 2 or higher fibrosis in patients without steatosis than in those with steatosis. This suggests a confounding effect of steatosis or inflammation in the population with nonalcoholic fatty liver disease. © RSNA, 2018.

Fate of Duct-Dependent, Discontinuous Pulmonary Arteries After Arterial Duct Stenting.

Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA. Five patients showed discontinuity of one PA, while two patients had both PAs served by bilateral ducts. PA growth was evaluated as per the Nakata index, McGoon ratio as well as dPA (n=9) versus heart-dependent PA (hPA; n=5) size and z-score changes. AD stabilization was performed using coronary stents dilated to 3.2±0.3mm (median 3.4), with significant increase of O2 saturation (from 83±11 to 95±5%, p<0.02). Control angiography was performed 5.1±2.8months (median 6 months) after duct stenting, showing significant growth of the dPA (from 3.7±1.0 to 7.6±2.7mm, p<0.001; z-score from -0.7±1.4 to 1.7±2.2, p<0.01). A trend toward better growth of the dPA as compared with the hPA was found (117±87 vs. 54±34%, p=NS). The final vessel size was still significantly different between the groups (dPA 7.6±2.7 vs. hPA 11.9±3.4mm, p=0.02), although the final z-score value did not significantly differ (dPA 1.7±2.2 vs. hPA 3.8±0.9mm, p=NS). In conclusion, percutaneous AD stenting is effective in promoting a significant catch-up growth of duct-dependent dPA, being, therefore, advisable as a reliable alternative to surgical palliation.

Use of Tolvaptan in a Patient With Palliated Congenital Heart Disease

Heart failure is a common problem in the ever growing population of patients with palliated congenital heart disease. It is frequently complicated by hyponatremia that has been associated with increased morbidity and mortality. Tolvaptan is a vasopressin receptor antagonist that has been effective in improving hyponatremia and congestive symptoms in adults with chronic heart failure. We describe the short-term use of tolvaptan to treat hyponatremic hypervolemia in an adolescent patient with chronic heart failure in the setting of palliated congenital heart disease prior to definitive surgical intervention. In this case, the patient had improvement in hyponatremia and a decrease in body weight, without any adverse effects.

Longer RBC storage duration is associated with increased postoperative infections in pediatric cardiac surgery.

Infants and children undergoing open heart surgery routinely require multiple RBC transfusions. Children receiving greater numbers of RBC transfusions have increased postoperative complications and mortality. Longer RBC storage age is also associated with increased morbidity and mortality in critically ill children. Whether the association of increased transfusions and worse outcomes can be ameliorated by use of fresh RBCs in pediatric cardiac surgery for congenital heart disease is unknown. One hundred and twenty-eight consecutively transfused children undergoing repair or palliation of congenital heart disease with cardiopulmonary bypass who were participating in a randomized trial of washed versus standard RBC transfusions were evaluated for an association of RBC storage age and clinical outcomes. To avoid confounding with dose of transfusions and timing of infection versus timing of transfusion, a subgroup analysis of patients only transfused 1-2 units on the day of surgery was performed. Mortality was low (4.9%) with no association between RBC storage duration and survival. The postoperative infection rate was significantly higher in children receiving the oldest blood (25-38 d) compared with those receiving the freshest RBCs (7-15 d) (34% vs 7%; p = 0.004). Subgroup analysis of subjects receiving only 1-2 RBC transfusions on the day of surgery (n = 74) also demonstrates a greater prevalence of infections in subjects receiving the oldest RBC units (0/33 [0%] with 7- to 15-day storage; 1/21 [5%] with 16- to 24-day storage; and 4/20 [20%] with 25- to 38-day storage; p = 0.01). In multivariate analysis, RBC storage age and corticosteroid administration were the only predictors of postoperative infection. Washing the oldest RBCs (> 27 d) was associated with a higher infection rate and increased morbidity compared with unwashed RBCs. Longer RBC storage duration was associated with increased postoperative nosocomial infections. This association may be secondary in part, to the large doses of stored RBCs transfused, from single-donor units. Washing the oldest RBCs was associated with increased morbidity, possibly from increased destruction of older, more fragile erythrocytes incurred by washing procedures. Additional studies examining the effect of RBC storage age on postoperative infection rate in pediatric cardiac surgery are warranted.

The Edwards Valeo lifestents in the treatment and palliation of congenital heart disease in infants and small children.

Intravascular stenting is the procedure of choice in the treatment of vascular stenoses. However, in infants and small children large sheaths are needed, and adult-size stents cannot be implanted. The Valeo Biliary Lifestent (Edwards Lifesciences Irvine, CA) is low profile and can be dilated up to 18-20 mm. We aimed to report on early and mid-term results with the use of Valeo stents in infants and children with congenital heart disease. Twenty-five subjects were treated in our experience. Mean age and weight were 39 ± 35 months (range 1-132) and 10.4 ± 6.7 kg (range 3-30), respectively. Two groups of patients were: Group A: patients with pulmonary artery stenosis (21 subjects); Group B subjects in whom stenting was applied in other vessel or heart sites (four subjects). A total of 35 stents were successfully used. Fluoroscopy time was 32 ± 11 min. No intra-operative death or hospital mortality was recorded. Stent post dilatation was performed in nine patients. The incidence of complication was 12% (3/25) (two subjects developed transient hypotension and bradycardia which required inotropic treatment, 1 patient developed mild lung bleeding). There was a significant improvement of angiographic appearance and RV pressure (RV/AO systolic pressure ratio before 1 ± 02 (range 0.8-1.5) versus after the procedure 0.6 ± 0.2 (range 0.4-0.9) P < 0.001). In group B stents were successfully implanted in aortic recoarctation, interatrial septum and in two modified BT shunts. At a median follow-up of 18 months (range 1-24 months) results remained stable and no complications occurred. In particular no stent fractures were seen. Furthermore, redilation was performed safely and successfully in three subjecs up to 18 months after the first implantation. In our series, Valeo Lifestents have proven to be effective and with low incidence of complication in various anatomical settings, in low weight infants and in early post-operative course. Large series and longer follow-up are mandatory.

Clinical characteristics associated with postoperative intestinal epithelial barrier dysfunction in children with congenital heart disease.

Children with congenital heart disease have loss of intestinal epithelial barrier function, which increases their risk for postoperative sepsis and organ dysfunction. We do not understand how postoperative cardiopulmonary support or the inflammatory response to cardiopulmonary bypass might alter intestinal epithelial barrier function. We examined variation in a panel of plasma biomarkers to reflect intestinal epithelial barrier function (cellular and paracellular) after cardiopulmonary bypass and in response to routine ICU care. Prospective cohort. University medical center cardiac ICU. Twenty children aged between newborn and 18 years undergoing repair or palliation of congenital heart disease with cardiopulmonary bypass. We measured baseline and repeated plasma intestinal fatty acid-binding protein, citrulline, claudin 3, and dual sugar permeability testing to reflect intestinal epithelial integrity, epithelial function, paracellular integrity, and paracellular function, respectively. We measured baseline and repeated plasma proinflammatory (interleukin-6, tumor necrosis factor-α, and interferon-γ) and anti-inflammatory (interleukin-4 and interleukin-10) cytokines, known to modulate intestinal epithelial barrier function in murine models of cardiopulmonary bypass. All patients had abnormal baseline intestinal fatty acid-binding protein concentrations (mean, 3,815.5 pg/mL; normal, 41-336 pg/mL). Cytokine response to cardiopulmonary bypass was associated with early, but not late, changes in plasma concentrations of intestinal fatty acid-binding protein 2 and citrulline. Variation in biomarker concentrations over time was associated with aspects of ICU care indicating greater severity of illness: claudin 3, intestinal fatty acid-binding protein 2, and dual sugar permeability test ratio were associated with symptoms of feeding intolerance (p < 0.05), whereas intestinal fatty acid-binding protein was positively associated with vasoactive-inotrope score (p = 0.04). Citrulline was associated with larger arteriovenous oxygen saturation difference (p = 0.04) and had a complex relationship with vasoactive-inotrope score. Children undergoing cardiopulmonary bypass for repair or palliation of congenital heart disease are at risk for intestinal injury and often present with evidence for loss of intestinal epithelial integrity preoperatively. Greater severity of illness requiring increased cardiopulmonary support rather than the inflammatory response to cardiopulmonary bypass seems to mediate late postoperative intestinal epithelial barrier function.