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Keywords: advanced liver disease; HCV; HIV coinfection; late presentation; viral hepatitis EN advanced liver disease HCV HIV coinfection late presentation viral hepatitis 1660 1664 5 10/19/21 20211101 NES 211101 Abbreviations ALT Alanine-aminotransferase APRI Aspartate-aminotransferase-to-platelet-ratio-index ART Antiretroviral therapy BMI Body mass index DAA Direct acting antiviral DHC-R German Hepatitis C-Registry GECCO German Hepatitis C Cohort GT Genotype HBV Hepatitis B virus HCC Hepatocellular carcinoma HCV Hepatitis C virus HIV Human immunodeficiency virus IVDA intravenous drug abuse LP Late presentation for viral hepatitis MSM Men who have sex with men OST Opioid substitution therapy PrEP Pre-Exposure prophylaxis RNA Ribonucleic acid SVR Sustained virological response WHO World Health Organization INTRODUCTION Chronic liver disease due to hepatitis C virus (HCV) infection is a major public health burden with an estimated 71 million people worldwide suffering from replicative HCV infection.1 Even if diagnosed in an early stage of disease, missing linkage to care or reluctance to treat might defer treatment until progression to an advanced stage of liver disease. Has increased rollout of direct acting antiviral therapy decreased the burden of late presentation and advanced liver disease in patients starting hepatitis C virus therapy in Germany? We aimed to investigate the extent of LP since unrestricted access to DAA therapy in Germany for HCV monoinfected and HIV/HCV coinfected patients in the period 2014-2018. Treatment uptake was about 3 times higher in 2015 compared to 2014 (1100 patients vs. 3055 patients), which reflects most likely the effect of the implementation of DAAs for HCV treatment and unrestricted access in Germany. [Extracted from the article]

Background: Neonatal management of patients with hypoplastic left heart syndrome and complex remains a challenging task, whereby the "hybrid" palliation is often reserved for high‐risk patients as a "rescue" procedure. Aim: This study documents the anesthetic challenges and potential complications associated with the Giessen hybrid stage I approach. Methods: The Giessen hybrid stage I approach is focused on surgical bilateral pulmonary artery banding. Retrospective perioperative data were analyzed. Contrary to a stable group A, inotropic treatment before surgery for treatment of postnatal shock classified patients as unstable (Group B). Clinical outcomes considered were inhospital mortality, duration of postoperative mechanical ventilation, postoperative time at the intensive care unit, perioperative vasoactive medication requirements, and red blood cell transfusion. Results: From June 1998 to December 2015, 185 patients were allocated to Group A (n = 165) and Group B (n = 20). The inhospital mortality was 2.2% with no difference between the groups. There was also no difference in the postoperative time on mechanical ventilation and the time in the intensive care unit. Vasoactive medication was more often required in Group B (100%) compared to Group A (19%). In Group B, more red blood cells were transfused 6.0 ± 8.3 vs 2.0 ± 5.8 mL/kg in Group A (P <.05, 95% CI 0.0 ‐ 2.6). Conclusion: Considering a learning curve, anesthesia for surgical bilateral pulmonary artery banding palliating patients with hypoplastic left heart syndrome and complex can safely be performed, independent from the preoperative clinical status. [ABSTRACT FROM AUTHOR]

Background: An alloimmune response to red blood cell (RBC) transfusion in neonates is a rare event. Several guidelines recommend limited pretransfusion testing in neonates. The evidence for these recommendations is based on small studies with sample sizes of between 30 and 90 infants.Study Design and Methods: We conducted a retrospective cohort study among consecutive patients who received transfusions at a single university medical center. All non-alloimmunized patients who had received their first RBC transfusion between 1994 and 2013 and who underwent at least one antibody screening follow-up visit between 7 and 365 days after transfusion were included.Results: The incidence of alloimmunization in the control group of 17,084 adult patients age 45 years or older who had received a median of 5 RBC units (interquartile range, 2-12 RBC units) was 3.55% (n = 607 alloimmunized patients). After transfusion of 40 RBC units, the cumulative incidence of alloimmunization in adult controls was 10.24% (95% confidence interval, 7.71%-13.17%). In total, 1641 neonates and children up to age 3 years received a median of 4 RBC units (interquartile range, 2-7 RBC units) in a median of two RBC transfusion episodes (interquartile range, one to five RBC transfusion episodes). Two children developed anti-M and anti-E antibodies post-transfusion at the ages of 181 and 611 days, respectively.Conclusion: To our knowledge, this study presents the largest longitudinal cohort study of RBC alloimmunization in neonates. Antibodies against RBC antigens were not detected within the first 6 months of life. Repeat antibody screening and cross-matching during the first months of life can be safely omitted. [ABSTRACT FROM AUTHOR]

De novo heterozygous mutations changing R179 to histidine, leucine, or cysteine in the ACTA2 gene are associated with Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS). Characteristic hallmarks of this condition, caused only by these specific ACTA2 mutations, are congenital mydriasis (mid-dilated, non-reactive pupils), a large persistent ductus arteriosus (PDA), aortic aneurysms evolving during childhood, and cerebrovascular anomalies. We describe two patients, a 3-day-old newborn and a 26-year-old woman, with this unique mutation in association with a huge PDA and an aorto-pulmonary window. In addition, one showed a coarctation of the aortic arch and the other a complete interruption of the aortic arch type A; thereby expanding the spectrum of cardiac congenital heart defect of this syndrome. Each patient displayed a huge PDA and an extra-cardiovascular phenotype consistent with MSMDS. These observations exemplify that a functional alpha 2 smooth muscle actin is necessary for proper cardiovascular organ development, and demonstrate that a very exceptional congenital heart defect (aortopulmonary window) can be caused by a mutation in a gene encoding a contractile protein of vascular smooth muscle cells. © 2017 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH.Methods: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC).Results: Of initially 155 patients, 128 were liveborn (82.6%). Ten neonates (7.8%) were lost to follow-up, in three (2.5%) neonates, parents decided for compassionate care. Overall survival after prenatal diagnosis of functionally UVH was 67.1%, and 90.4% on an intention-to-treat basis. Survival after surgery reached 93.7%. The majority of deaths occurred within the group of dominant RV (10/74, 13.5%). High risk HLHS with restrictive foramen ovale was associated with the lowest survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p < 0.05).Conclusion: These results should be explained to parents to ensure informed decisions and counselling. © 2016 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Noonan syndrome with multiple lentigines (NSML) frequently manifests with hypertrophic cardiomyopathy (HCM). Recently, it was demonstrated that mTOR inhibition reverses HCM in NSML mice. We report for the first time on the effects of treatment with a rapamycin analog in an infant with LS and malignant HCM. In the boy, progressive HCM was diagnosed during the first week of life and a diagnosis of NSML was established at age 20 weeks by showing a heterozygous Q510E mutation in PTPN11. Immunoblotting with antibodies against pERK, pAkt, and pS6RP in fibroblasts demonstrated enhanced Akt/mTOR pathway activity. Because of the patient's critical condition, everolimus therapy was started at age 24 weeks and continued until heart transplantation at age 36 weeks. Prior to surgery, heart failure improved from NYHA stage IV to II and brain natriuretic peptide values decreased from 9,600 to <1,000 pg/ml, but no reversal of cardiac hypertrophy was observed. Examination of the explanted heart revealed severe hypertrophy and myofiber disarray with extensive perivascular fibrosis. These findings provide evidence that Akt/mTOR activity is enhanced in NSML with HCM and suggest that rapamycin treatment could principally be feasible for infantile NSML. The preliminary experiences made in this single patient indicate that therapy should start early to prevent irreversible cardiac remodelling. © 2015 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective We aimed to assess the feasibility of using a percutaneous transhepatic cardiac catheterization technique to perform fetal pulmonary valvuloplasty and valvulotomy under ultrasound guidance at mid-gestation. Method In 13 mid-gestation fetal lambs without cardiac pathology, percutaneous transhepatic cardiac catheterization was used to position a coronary angioplasty catheter within the pulmonary valve. The balloon was inflated/deflated several times, simulating pulmonary valvuloplasty. In another two fetal lambs, a guidewire tip was positioned against the pulmonary valve, and unipolar diathermy was applied to simulate perforation of an atretic valve. Results Percutaneous access followed by right heart catheterization was successful in all cases. One fetus died following right ventricle perforation. Simulated pulmonary valvuloplasty was successful in nine cases using catheters with 6-mm-long balloons but unsuccessful in two cases (both survived) using 12-mm-long balloons. In one case, the catheter could not be inserted as the cannula became dislodged. Diathermy of the pulmonary valve was successful in both attempts. Conclusion We successfully simulated in utero perforation and dilation of the pulmonary valve using percutaneous transhepatic access in fetal lambs. The technique has potential for clinical translation into treatment for human fetuses with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. © 2014 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Behnke-Hall K, Bauer J, Thul J, Akintuerk H, Reitz K, Bauer A, Schranz D. Renal function in children with heart transplantation after switching to CNI-free immunosuppression with everolimus. Pediatr Transplantation 2011: 15: 784-789. © 2011 John Wiley & Sons A/S. Abstract: Renal impairment because of CNI contributes to long-term morbidity. Therefore, CNI avoiding or sparing treatment strategies are important. In this article, we describe the results of a CNI-free treatment protocol with regard to recovery of renal function. Twenty-eight patients with heart transplantation were switched from CNI regimen to everolimus and mycophenolate, when cGFR was <75 mL/min/1.73 m2. In all patients, CNI was stopped, when everolimus trough levels of 5-8 ng/L were achieved. Serum creatinine and cGFR were determined before and after 6 and 12 months. Median serum creatinine decreased from 1.2 mg/dL (range 0.7-3.7) before everolimus to 1.0 (range 0.6-1.8) and 1.0 (range 0.5-1.9) mg/dL after 6 and 12 months. Median cGFR was 47.81 (range 18.3-72.6) mL/min/1.73 m2 before everolimus and 63.1 (range 37.8-108.7) mL/min/1.73 m2 at six months and 64.8 (range 37.7-106.6) mL/min/1.73 m2 after 12 months. All changes from baseline to six and 12 months were statistically significant (p < 0.05). Adverse events were infections (n = 3) and rejections (n = 3). Therapy was discontinued in four patients. Conversion to CNI-free immunosuppression resulted in significant improvements of renal function within six months of CNI withdrawal. Side effects are common. However, more studies are required to demonstrate the effectiveness in children. [ABSTRACT FROM AUTHOR]

Siahi-Benlarbi R, Nies SM, Sziegoleit A, Bauer J, Schranz D, Wetzel W-E. Caries-, Candida- and Candida antigen/antibody frequency in children after heart transplantation and children with congenital heart disease. Pediatr Transplantation 2010: 14:715–721. © 2008 Wiley Periodicals, Inc. The aim of this study was to determine the incidence of oral/intestinal Candida colonization and Candida-antigen/antibody in immunosuppressed children after HTx (group III, n = 31), in children with CHD (group II, n = 24) and in children with healthy hearts (comparison group, group I, n = 23) aged 2–16 yr according to their dental status between 2004 and 2007. Candida species in saliva, dental plaque, carious lesions and stool were detected with Sabouraud-/CHROMagar and Auxacolor system. Candida-specific-antigen/antibody assays were used for serological diagnosis. Dental status was determined on the basis of the DMF/dmf(T/t)-index. We found significant group differences in fecal Candida colonization (p = 0.027). In relation to dental status, oral Candida colonization increased within group III from 28.5% [DMF/dmf(T/t) = 0] to 66.7% [DMF/dmf(T/t) ≥ 1] up to 100.0% [D/d(T/t) ≥ 1], similar in groups I and II. Candida-mannan-antigen was determined to be positive in 16.1% (HTx), 5.5% (CHD) and 13.0% (comparison group). We show correlation between oral Candida colonization and (carious) dental status. We assume that high oral Candida and their descending/resorption through the gastrointestinal tract may lead to serologic Candida accumulation or rather candidiasis. Therefore, healthy oral cavity (especially before/after HTx) is an important precondition to prevent Candida infections. [ABSTRACT FROM AUTHOR]

DCM is the most common cardiomyopathy in childhood. Effectiveness of anticongestive therapy is limited in most cases and about one-third of children diagnosed with DCM die or receive heart transplantation within the first year after diagnosis. Cardiac stem cell transplantation has become a promising therapy to treat heart failure in adult patients. Based on these promising results, the cardiac stem cell therapy might also represent a new therapeutic option particularly in young children. The present case documents for the first time intracoronary administration of autologous bone marrow-derived progenitor cells in a critically ill two-yr-old child with severe heart failure caused by DCM. Because of progressive worsening of the clinical condition despite maximal anticongestive treatment, the decision to perform autologous stem cell therapy was made. Cardiac stem cell therapy proved to be technically feasible, was associated with improvement in cardiac function, and might represent an option before heart transplantation in children with severe heart failure. [ABSTRACT FROM AUTHOR]

Background: Patients who require extracorporeal membrane oxygenation (ECMO) postsurgery for congenital heart disease (CHD) frequently experience severe bleeding episodes. Whereas recombinant-activated factor VII (rFVIIa) has proven efficacy in counteracting intractable hemorrhage in various scenarios, its use in patients on ECMO is limited by the increased risk for thrombotic events. Methods: Between December 2004 and January 2006, ECMO was used in 10 pediatric patients following cardiac surgery, of whom seven were treated with rFVIIa because of intractable hemorrhage. Their medical records were reviewed with respect to variations in chest tube output and transfusion requirements, occlusion of or thrombus formation in the ECMO circuit and the occurrence of thromboembolic events. Outcome and rate of ECMO circuit occlusion were compared with historic controls. Results: Three patients died, and four survived (none of the deaths was attributable to thrombus formation or bleeding). All patients were treated with aprotinin prior to and during rFVIIa therapy. Two patients developed an occlusion of the oxygenator, one after receiving co-medication with a FXIII concentrate, another after RBC transfusion in the ECMO system. In two patients, thrombus formation was observed in the ECMO system on inspection after discontinuation. Thromboembolic events were not observed. Conclusions: Recombinant-activated factor VII in a median dosage of 90 μg·kg−1 was used in seven pediatric patients on ECMO. Rates of ECMO system occlusions and mortality did not differ from historic controls. Neither the reduction of chest tube output nor the blood product transfusion requirements did reach statistical significance. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion Trial Registration Patients with hypoplastic left heart syndrome undergo the comprehensive stage 2 procedure as the second stage in the hybrid approach toward Fontan circulation. The complexity of comprehensive stage 2 procedure is considered a potential limitation, and limited information is available on its anesthetic management. This study aims to address this gap.A single‐center retrospective cohort study analyzed 148 HLHS patients who underwent comprehensive stage 2 procedure, divided into Group A (stable condition, n = 116) and Group B (requiring preoperative intravenous inotropic therapy, n = 32). Demographic data, intraoperative hemodynamics, anesthetic management, and postoperative outcomes were collected.Etomidate (40%) was the most common induction agent, followed by esketamine (24%), midazolam (16%), and propofol (13%). Inhaled induction was rarely necessary (2%), occurring only in Group A patients. No statistical differences were found between groups for induction drug choice. Post‐cardiopulmonary bypass management included moderate hypoventilation, inhaled nitric oxide (100%), and hemodynamic support with milrinone (97%) and norepinephrine (77%). Group B patients more frequently required additional levosimendan (20%) and epinephrine (18%). Extracorporeal membrane oxygenation was necessary in 8 patients (5%) with no between‐group differences. Switching from fentanyl to remifentanil reduced postoperative ventilation time overall. However, Group B experienced significantly longer ventilation (6.3 vs. 3.5 h) and ICU stay (22 vs. 14 days). In‐hospital mortality was 5% overall (Group A: 4%, Group B: 9%). Long‐term survival analysis revealed a significant advantage for Group A.The use of short‐acting opioids and adjusted ventilation modes enables optimal pulmonary blood flow and rapid transition to spontaneous breathing. Differentiated hemodynamic support with milrinone, norepinephrine, supplemented by levosimendan and epinephrine in high‐risk patients, can mitigate the effects on the preoperatively volume‐loaded right ventricle. However, differences in long‐term survival probability were observed between groups.Local ethics committee, Medical Faculty, Justus‐Liebig‐University‐Giessen (Trial Code Number: 216/14). [ABSTRACT FROM AUTHOR]

LUEDEMANN, M., et al.: Implantable Cardioverter Defibrillator in a Child Using a Single Subcutaneous Array Lead and an Abdominal Active Can. This report describes the case of an 8-year-old boy with hypertrophic cardiomyopathy (HCM) who underwent ICD implantation for recurrent syncope. To avoid vascular complications and to minimize the surgical approach in this small child, a nonthoracotomy ICD system was chosen using a single subcutaneous array lead with only one finger, an abdominally placed active can, and epicardial dual chamber pacing and sensing electrodes. During an 8-month follow-up, DFT was confirmed and there were no ventricular tachycardia or complications. It appears to be a safe device for the prevention of sudden cardiac death in infants and small children. (PACE 2004; 27:117–119) [ABSTRACT FROM AUTHOR]