Publications by authors named "Ina Michel-Behnke"

71 Publications

Percutaneous treatment of a huge patent ductus venosus and severe portal vein hypoplasia using a Figulla Flex II atrial septal defect occluder in a 2-year-old infant: a case report.

Eur Heart J Case Rep 2021 Dec 11;5(12):ytab455. Epub 2021 Nov 11.

Division of Pediatric Cardiology, University Hospital for Children and Adolescents, Pediatric Heart Centre, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.

Background: Intra- or extrahepatic porto-caval shunts (PCSs) can account for multiorgan dysfunction with pulmonary arterial hypertension and portosystemic encephalopathy as the most serious consequences of bypass of the hepatic circulation. The ductus venosus (DV) represents a rare foetal PCS and might be persistently patent in newborns after birth. Treatment strategies include surgical ligation and percutaneous device closure. The degree of portal vein hypoplasia limits therapy making liver transplantation the only option in some of them.

Case Summary: In a newborn female patient a huge persistently patent DV, known already prenatally, resulted in severe secondary portal vein hypoplasia. She presented with hyperammonaemia, elevated liver enzymes, and pulmonary hypertension. With only diminutive portal venous branches and exceedingly high portal venous pressures during test-occlusion of the DV, shunt closure was not possible. At the age of 2 years more favourable portal venous pressures allowed transcatheter device closure with a nitinol atrial septal defect occlusion device. Pulmonary artery pressures and ammonia levels normalized after the procedure without any signs of portal hypertension.

Discussion: The case highlights the importance of meticulous imaging using balloon occlusion angiography of PCSs like the DV, to search for intrahepatic portal veins. Moreover, portal vein pressure during test-occlusion can identify patients amenable for surgical or endovascular shunt closure. Occlusion devices licensed for other indications like atrial septal defect closure can be used safely in huge PCS vessels in a one-step or staged procedure. Optimal timing of the intervention should be tailored to the patient's needs.
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http://dx.doi.org/10.1093/ehjcr/ytab455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8684806PMC
December 2021

Initial Angiotensin Receptor Blocker Response in Young Marfan Patients Decreases After 3 Years of Treatment.

Pediatr Cardiol 2021 Nov 10. Epub 2021 Nov 10.

Division of Pediatric Cardiology, Department of Pediatric Cardiology, Pediatric Heart Center Vienna, University Children's Hospital, Pediatric and Young Adult Marfan Syndrome Outpatient Clinic Vienna, Medical University Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.

Marfan syndrome is caused by mutations of the fibrillin-1 gene, which weakens the connective tissue integrity. Since 2003, bioavailability regulations of TGF-ß through fibrillin alterations have been presumed of being the culprit mechanisms for aortic aneurysm development. We present the analysis of our single-center Marfan children and adolescents cohort to assess the influence of age, sex, degree of cardiovascular involvement and dosage on losartan effectivity. This prospective longitudinal registered echocardiographical investigation (EudraCT 2009-016139-36) of 49 patients with an average follow-up of 72 months focused on aortic root z-scores, elasticity, and yearly progression rates. The 33 patients under medication with losartan showed an aortic root z-score reduction during the first 36 months compared to 22 patients without medication presenting constant mild progression. Yet, results diminished under losartan thereafter, adding up to similar progressions over 72 months in both groups (0.07 ± 0.10/year versus 0.04 ± 0.11/year). Although male patients exhibited higher root z-scores, progression with and without medication was comparable to females and not age-dependent. In conclusion, losartan evoked a significant aortic root z-score regression in young Marfan patients over the first 3 years, but this effect mitigated thereafter. The initial improvement concurred with ameliorated elasticity; lower stiffness levels predicted better clinical outcome, but likewise only up to 36 months. Sex differences in dilatation severity were observed but neither age nor sex had significant influence on progression rates. Losartan dosages were gradually increased in more severely affected patients and provided an equal rate of root progression over 72 months in comparison to patients under losartan treatment with lesser baseline dilatation severity.
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http://dx.doi.org/10.1007/s00246-021-02761-4DOI Listing
November 2021

A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension.

Cardiovasc Diagn Ther 2021 Aug;11(4):1160-1177

Division of Pediatric Cardiology and Pediatrics, Medical University Graz, Graz, Austria.

While the current definition of pulmonary hypertension (PH) is still based on haemodynamic variables, transthoracic echocardiography is the most important diagnostic clinical tool for the first assessment and evaluation of a patient, in whom PH is suspected. In addition, it is the most important clinical modality in long term follow-up and the utility of echocardiography has widely been demonstrated in patients with PH. Echocardiography not only reveals the underlying cardiac morphology and diagnosis of any associated cardiac defects. In most patients with PH right ventricular (RV) pressure estimation is feasible. In addition, ventricular systolic and diastolic function, as well as ventricular-ventricular interactions of both ventricles can be assessed by using echocardiography. Maximizing the use of echocardiography by reporting several measures to gain information and quantitatively describe the parameters, that are linked to prognosis, seem particularly appealing in these children, in whom other advanced imaging modalities requiring anaesthesia is associated with a considerable risk. Herein we provide a practical approach and a concise and clinically applicable echocardiographic guidance and present basic variables, which should be obtained at any assessment. Moreover, we present additional advanced echocardiographic measures, that can be applied in a research or clinical setting when progressive PH needs a deeper insight to assess heart function, estimation of pulmonary artery pressures among others, by echocardiography. Finally, clinically relevant studies in view of the prognostic properties with a focus on the most important echocardiographic variables in pediatric PH are summarized.
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http://dx.doi.org/10.21037/cdt-21-119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8410502PMC
August 2021

Characteristics, management, and outcome of pediatric patients with post-transplant lymphoproliferative disease-A 20 years' experience from Austria.

Cancer Rep (Hoboken) 2021 10 23;4(5):e1375. Epub 2021 Mar 23.

Department of Pediatric Hematology and Oncology, St. Anna Children's Hospital, Vienna, Austria.

Background: Management of pediatric post-transplantation lymphoproliferative disorder (PTLD) after hematopoietic stem cell (HSCT) and solid organ transplantation (SOT) is challenging.

Aim: This study of 34 PTLD patients up to 19-years old diagnosed in Austria from 2000 to 2018 aimed at assessing initial characteristics, therapy, response, and outcome as well as prognostic markers of this rare pediatric disease.

Methods And Results: A retrospective data analysis was performed. Types of allografts were kidney (n = 12), liver (n = 7), heart (n = 5), hematopoietic stem cells (n = 4), lungs (n = 2), multi-visceral (n = 2), small intestine (n = 1), and vessels (n = 1). Eighteen/34 were classified as monomorphic PTLD, with DLBCL accounting for 15 cases. Polymorphic disease occurred in nine, and non-destructive lesions in six cases. One patient had a non-classifiable PTLD. Thirteen/34 patients are surviving event-free in first remission (non-destructive, n = 4/6; polymorphic, n = 4/9; monomorphic, n = 6/18). Fourteen/34 patients lacked complete response to first-line therapy, of whom seven died. Four/34 patients relapsed, of whom two died. In 3/34 patients, death occurred as a first event. The 5-year overall and event-free survival rates were 64% ± 9% and 35% ± 9% for the whole cohort. Among all parameters analyzed, only malignant disease as the indication for transplantation had a significantly poor influence on survival.

Conclusions: This study shows PTLD still to be a major cause of mortality following SOT or HSCT in children. A continued understanding of the molecular biology of the disease shall allow to decrease treatment intensity for lower risk patients and to identify patients who may benefit from newer therapy approaches to improve outcome and decrease morbidity.
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http://dx.doi.org/10.1002/cnr2.1375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8551996PMC
October 2021

Negative view slow-flow color Doppler: A potential improvement for first trimester cardiac screening.

Eur J Obstet Gynecol Reprod Biol 2021 May 8;260:234-235. Epub 2021 Mar 8.

Practice for Prenatal Medicine, Vienna, Austria.

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http://dx.doi.org/10.1016/j.ejogrb.2021.02.030DOI Listing
May 2021

Macitentan in infants and children with pulmonary hypertensive vascular disease. Feasibility, tolerability and practical issues - a single-centre experience.

Pulm Circ 2021 Jan-Mar;11(1):2045894020979503. Epub 2021 Jan 20.

Department of Paediatrics and Adolescent Medicine, Paediatric Heart Centre Vienna, Medical University of Vienna, Vienna, Austria.

Macitentan is a safe and effective substance for treatment of adults with pulmonary arterial hypertension. Data on its use in paediatric patients are limited. In this single-centre prospective study, we report on our experience with macitentan in children focusing on applicability and practical aspects. Between December 2014 and July 2018, macitentan was introduced to paediatric patients according to a dosing protocol adjusted to body weight. Blood pressure, heart rate, saturation and clinical symptoms were recorded daily during introduction. Liver function parameters and haemoglobin levels were measured at baseline, four weeks and three months after initiation and after one year of treatment. Twenty-four patients (14 male, 10 female) were enrolled for treatment with macitentan. The mean age was 10.7 ± 7.6 years (range: 0.1 year-23 years). Fifteen out of 24 patients were World Health Organization functional class (FC) II, 7 patients in FC III and 2 patients in FC IV. Twenty out of 24 patients (83%) received additional advanced therapy with sildenafil and/or prostacyclines. We had two early discontinuations because of clinical relevant oedema. In the remaining 22 patients, macitentan was well tolerated. Liver function parameters and blood count levels remained stable during the observational time. The introduction of macitentan was feasible and mostly well tolerated in paediatric patients. Special attention should be paid to oedema during introduction of the drug. To the best of our knowledge, this is the first study to report on its applicability in infants and children. However, larger prospective trials are warranted to verify these preliminary findings.
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http://dx.doi.org/10.1177/2045894020979503DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7829536PMC
January 2021

European position paper on the management of patients with patent foramen ovale. Part II - Decompression sickness, migraine, arterial deoxygenation syndromes and select high-risk clinical conditions.

Eur Heart J 2021 04;42(16):1545-1553

Ospedale San Giovanni Bosco, Turin, Italy.

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.
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http://dx.doi.org/10.1093/eurheartj/ehaa1070DOI Listing
April 2021

COVID-19 FAQs in paediatric and congenital cardiology: AEPC position paper.

Cardiol Young 2021 Mar 7;31(3):344-351. Epub 2021 Jan 7.

Department of Paediatric Cardiology, University Hospital of Children and Adolescent Medicine, Department of Paediatric Cardiology/Paediatric Heart Center, Medical University of Vienna, Vienna, Austria.

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.
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http://dx.doi.org/10.1017/S1047951120005028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7900664PMC
March 2021

Copeptin: Prognostic Relevance as a Perioperative Marker in Pediatric Cardiac Surgery.

Ann Thorac Surg 2022 01 4;113(1):174-180. Epub 2020 Dec 4.

Division of Cardiac Surgery, Pediatric Heart Center, Department of Surgery, Medical University of Vienna, Vienna, Austria. Electronic address:

Background: Copeptin is a cleavage product of vasopressin. This study aimed to figure out if copeptin would be a suitable biomarker in patients with congenital heart disease in the postoperative course.

Methods: The primary outcome endpoint of this study was the change in copeptin concentration perioperatively in patients with congenital heart disease after surgery, with the use of a cardiopulmonary bypass. Three blood samples were taken from 81 patients up to 6 years of age in order to evaluate changes in copeptin concentration.

Results: Significant increase of copeptin concentration was shown between the first and second blood draws as well as between the first and third blood draws (Ps < .001). Additionally, positive and significant correlations (r ≥ .27) between the cardiopulmonary bypass times, The Society of Thoracic Surgeons and European Association for Cardio-Thoracic Surgery mortality category, the inotropic score, the duration of mechanical ventilation, the length of stay at the intensive care unit (ICU), the length of stay at the hospital, and the preoperative as well as the ICU copeptin levels were found.

Conclusions: Copeptin showed a tendency to predict the clinical outcome of patients after congenital heart surgery. Patients with higher copeptin levels underwent more complex procedures, had longer cardiopulmonary bypass times, required more catecholamine support, needed longer time of invasive ventilation, and had longer overall stay and ICU stay.
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http://dx.doi.org/10.1016/j.athoracsur.2020.11.017DOI Listing
January 2022

Double atrioventricular valve replacement using Melody™ transcatheter valves in an infant with unbalanced atrioventricular septal defect: a case report.

Eur Heart J Case Rep 2020 Aug 5;4(4):1-6. Epub 2020 Aug 5.

Division of Cardiac Surgery, Pediatric Heart Center Vienna, Department of Surgery, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria.

Background: To the best of our knowledge, this is the first report of a successful one-stage double atrioventricular valve (AVV) replacement using two Melody™ transcatheter valves in an infant.

Case Summary: We report a successful case of double AVV replacement with Melody™ transcatheter valves in a 9-month-old infant with a right ventricular dominant atrioventricular septal defect (AVSD). The boy initially presented with borderline sized left-sided heart structures, congenital left AVV stenosis, ventricular displacement of the right AVV with high-grade insufficiency and moderate valvar pulmonary stenosis. Double AVV replacement was performed 2 months after an unsuccessful attempt to repair the defect with persisting left AVV stenosis, underfilling of the small left ventricle and high-grade right AVV, and pulmonary valve regurgitation, resulting in low cardiac output. During double Melody™ AVV replacement, the right ventricular outflow tract was replaced with a pulmonary homograft. The boy was discharged on post-operative Day 28 and presented with competent valves, no ventricular outflow tract obstruction and no paravalvular leak at 11 months of follow-up.

Discussion: The presented innovative approach allowed for biventricular correction of an unbalanced AVSD with unfavourable anatomy for standard techniques. The heart team should decide individualized, after careful assessment of cardiac anatomy and function, if the benefits of replacement of AVVs with Melody™ transcatheter valves may outweigh the benefits of univentricular palliation in case of unbalanced AVSD.
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http://dx.doi.org/10.1093/ehjcr/ytaa174DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7501891PMC
August 2020

Direct postoperative protein S100B and NIRS monitoring in infants after pediatric cardiac surgery enrich early mortality assessment at the PICU.

Heart Lung 2020 Nov - Dec;49(6):731-736. Epub 2020 Sep 4.

Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Medical University of Vienna, Austria. Electronic address:

Background: Neuromonitoring using plasmatic biomarkers such as S100B and near-infrared spectroscopy (NIRS) represents a standard procedure for detecting cerebral damage after cardiac surgery. Their use in pediatric clinical assessment, however, is negligible.

Objectives: The goal of this study was to evaluate the predictive role of S100B levels and cerebral oxygenation in postoperative pediatric cardiac patients for survival and potential cerebral injuries.

Methods: A retrospective cohort study of infants after cardiac surgery. Primary outcome was survival until discharge. Intra/postoperative vital signs and laboratory data were measured and statistically analyzed.

Results: Seven out of 226 infants were non-survivors. Non-survivors had significantly lower cerebral saturation than survivors, as well as elevated S100B values at admission, associated with lower arterial pressure and higher serum lactate levels.

Conclusion: Although significant differences of S100B and crO values between survivors and non-survivors were found, no critical thresholds could be established from the data. Nevertheless, changes from the norm in these parameters should raise awareness for critical clinical development.
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http://dx.doi.org/10.1016/j.hrtlng.2020.08.014DOI Listing
March 2021

A Cyanotic Newborn with a Pink Right Upper Extremity.

Case Rep Pediatr 2020 29;2020:8873156. Epub 2020 Jun 29.

Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management.
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http://dx.doi.org/10.1155/2020/8873156DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7341379PMC
June 2020

Surgical stented transcatheter valve-in-valve implantation in atrioventricular position in children.

Multimed Man Cardiothorac Surg 2020 Jun 8;2020. Epub 2020 Jun 8.

Department of Cardiac Surgery Medical University of Vienna Spitalgasse 23, A1090 Vienna, Austria.

The Melody valve (Medtronic, Minneapolis, MN, USA) is a stented bovine jugular vein graft that was primarily approved for transcatheter implantation in a pulmonary valve position. The prosthetic valve can also be implanted in an atrioventricular position in infants and young children, and in these cases it must be modified appropriately.  In this tutorial we demonstrate the surgical preparation of a stented transcatheter Melody valve for implantation in the atrioventricular position. Additionally, we present a safe and effective method for surgical valve-in-valve implantation in a 3-year-old patient with hypoplastic left heart syndrome.
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http://dx.doi.org/10.1510/mmcts.2020.026DOI Listing
June 2020

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

J Heart Lung Transplant 2019 09 21;38(9):879-901. Epub 2019 Jun 21.

Department of Paediatric Cardiology, German Pediatric Heart Centre, Sankt Augustin, Germany.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.
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http://dx.doi.org/10.1016/j.healun.2019.06.022DOI Listing
September 2019

PD-L1 and PD1 expression in post-transplantation lymphoproliferative disease (PTLD) of childhood and adolescence: An inter- and intra-individual descriptive study covering the whole spectrum of PTLD categories.

Cancer Med 2019 08 3;8(10):4656-4668. Epub 2019 Jul 3.

Department of Pediatric Hematology and Oncology, St. Anna Children's Hospital, Vienna, Austria.

Therapy of children with post-transplantation lymphoproliferative disorder (PTLD) after hematopoietic stem cell (HSCT) and solid organ transplantation (SOT) can be challenging. In this retrospective study, we investigated PD-L1 and PD1 expression in all PTLD categories of childhood and adolescence to see whether checkpoint inhibition with PD-L1/PD1 inhibitors may serve as a therapy option. We included 21 patients aged 19 years or younger (at date of transplant) with PTLD following SOT or HSCT having adequate tumor samples available (n = 29). Using immunohistochemistry, we evaluated PD-L1/PD1 expression on both tumor cells and cells of the microenvironment in all samples. Availability of consecutively matched tumor samples during 6 of 21 patients' disease courses also allowed an intra-individual assessment of PD-L1/PD1 expression. We observed lower PD-L1 and higher PD1 expression in non-destructive lesions, and higher PD-L1 and lower PD1 expression in polymorphic and, in particular, in monomorphic PTLD, mostly diffuse large B-cell lymphomas (DLBCL, n = 10/21). The amount of PD-L1- and PD1-positive cells changed in the opposite way in sequential biopsies of the same individual correlating well with the PTLD category. This is the first comprehensive pediatric study assessing PD-L1 and PD1 expression on tumor cells and in the microenvironment of PTLD including not only monomorphic, but also non-destructive early lesions. PD-L1 expression of the tumor cells inversely correlated with PD1 expression in surrounding tissues, with the highest expression in DLBCL. Since PTLD can be therapeutically challenging, our results indicate a potential efficacy of checkpoint inhibitors if standard immune- and/or chemotherapy fail or are impossible. We therefore recommend routine staining of PD-L1 and PD1 in all PTLD categories.
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http://dx.doi.org/10.1002/cam4.2394DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6712474PMC
August 2019

Regenerative therapies in young hearts with structural or congenital heart disease.

Transl Pediatr 2019 Apr;8(2):140-150

Pediatric Heart Center, Justus-Liebig University, Giessen, Germany.

Pediatric heart failure (HF) is rare. The prognosis is generally poor. HF is most frequently related to cardiomyopathy or congenital heart disease (CHD). Associated phenotypes are HF with preserved (HFpEF) or reduced ejection fraction (HFrEF); both in children with biventricular or univentricular circulation. Cardiac growth, differentiation, proliferation and consecutively regenerative and repair mechanisms are inversely related to the patient's age; edaphic and circulating cardiac progenitor cells as well; in sum, there are enormous endogenous potentials repairing a diseased heart in particular in young children. Efforts supporting pediatric cardiac regeneration are clearly justified; cell-based therapies have been addressed in small series of children with end-stage HF of either the left or right ventricle, more recently in randomized clinical trials. Different cell populations like autologous bone marrow mononuclear cells, progenitor cells or cardiac derived cells have been injected into coronaries or directly into the myocardium. Beneficial at least transient improvement of cardiac function was observed in patients with dilative cardiomyopathy and CHD, mainly hypoplastic left heart syndrome (HLHS). Cellular repopulation and possibly more crucial, paracrine effects contributed in slowing down progression of pediatric end-stage HF. Our review summarizes the current knowledge in different scenarios of HF by cell-based cardiac therapies in critically ill children. Based on the actual clinical experience future work to distinguish responders from non-responders among other refinements will lead to individualized precision treatment of HF in children, what means a lot to a child on a long list waiting for heart transplantation (HTX).
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http://dx.doi.org/10.21037/tp.2019.03.01DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6514281PMC
April 2019

Surgical Atrioventricular Valve Replacement With Melody Valve in Infants and Children.

Circ Cardiovasc Interv 2018 11;11(11):e007145

Department of Cardiology and Cardiac Surgery, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Donato, Milan, Italy (V.M., S.M.E.).

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.
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http://dx.doi.org/10.1161/CIRCINTERVENTIONS.118.007145DOI Listing
November 2018

Custom tailoring of medical implants for pulmonary artery bifurcation stenosis.

Int J Cardiol 2019 06 29;284:82-83. Epub 2018 Nov 29.

University Hospital of Children and Adolescent Medicine, Department of Pediatric Cardiology/Pediatric Heart Center, Medical University of Vienna, Austria. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2018.11.120DOI Listing
June 2019

First-in-man pulmonary artery stenting in children using the Bentley® BeGrow™ stent system for newborns and infants.

Int J Cardiol 2019 Feb 19;276:107-109. Epub 2018 Nov 19.

Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children's Hospital Zurich, Switzerland.

Background: Stent implantation into growing vessels is a common treatment option in infants and children with congenital heart disease (CHD) and corresponding vessel lesions. After stent implantation in small children, repetitive stent redilations are frequently necessary to accommodate for somatic growth. Until now, all available stents have limited final expansion diameters.

Material And Results: The new Bentley BeGrow™ stent system for newborns and infants is a L605 cobalt‑chromium, pre-mounted, balloon expandable stent, which is compatible with a 4 French sheath and 0.014 inch guide wire thus allowing implantation in small vessels (4-6 mm). It offers a new, unique stent design that allows post-dilation steps up to Ø11.5 mm. While re-dilating up to Ø11.5 mm this new stent maintains radial force and shows uniform expansion with only minimal foreshortening. Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5 mm. Residual radial force maintains even after stent opening due to spiral arrangement of the predetermined breaking points. The 2 first-in-man pulmonary artery stent implantations in a newborn with univentricular circulation and a toddler with biventricular circulation are reported as part of the currently performed licencing trial (ClinicalTrials.govNCT03287024).

Conclusion: The low-profile BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. In our first experience, it can be effectively implanted. Longer follow-up will evaluate multiple, stepwise redilations and controlled stent strut breakage, which have the potential to accommodate for somatic vessel growth and/or subsequent implantation of larger stents.
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http://dx.doi.org/10.1016/j.ijcard.2018.11.029DOI Listing
February 2019

Feasibility, safety and diagnostic impact of endomyocardial biopsies for the diagnosis of myocardial disease in children and adolescents.

EuroIntervention 2018 Nov;14(10):1089-1095

Department of Paediatric Cardiology, University Heart Centre Hamburg, Hamburg, Germany.

Aims: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value.

Methods And Results: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95±6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%; p<0.001). The overall complication rate was 9.7%, whereas major complications occurred in only 0.97% of cases. Risk factors associated with a higher complication rate were biopsy during the first year of life (20.5%) and from the left ventricle (31.1%) (p<0.05). There was no procedure-related mortality. Treatment was changed in 18.0% of cases based on biopsy results.

Conclusions: Today, endomyocardial biopsies in older children with suspected myocardial disease can be performed safely with a low risk of major complications and mortality, whereas the risk of complications if the biopsy is carried out in the first year of life or taken from the left ventricle remains high.
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http://dx.doi.org/10.4244/EIJ-D-18-00128DOI Listing
November 2018

Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology.

Heart Vessels 2018 Nov 18;33(11):1350-1357. Epub 2018 May 18.

Pediatric Heart Center Vienna/Division of Pediatric Cardiology, University Children's Hospital Vienna, Medical University Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.

Turner syndrome (TS) is a common genetic disorder in females with high incidence of ascending aortic dilatation and even dissection occurring as early as in the second decade. Known risk factors (RF) are bicuspid aortic valves (BAV), coarctation of the aorta (CoA), and arterial hypertension. Since 10% of dissections occur in patients without RF, an intrinsic aortic wall abnormality has been postulated. This study aimed to investigate the elasticity of the ascending aorta as a surrogate marker of aortic wall texture. Forty-six pediatric patients with genetically proven TS were prospectively examined for the morphology of their aortic valve, and size and elasticity indices of the adjacent aorta. Cohorts of 46 female subjects with tricuspid aortic valves (TAV) and ten non-syndromic females with BAV were investigated as separate control groups. Comparison of healthy controls with TS patients revealed significantly deteriorated elasticity indices in those with TS. Furthermore, normalized aortic dimensions were greater in TS patients, but dilatations of the ascending aorta with z-score levels above two were restricted to those with BAV (14/46). Deteriorated elasticity indices were measured in TS patients, independent of aortic dilatation, BAV, and CoA, and were comparable to those of patients with isolated, non-syndromic BAVs. By measuring elasticity levels as a surrogate for aortic wall texture, we were able to gather evidence that TS presents with an intrinsic abnormality of the ascending aorta even in patients without concomitant BAV, CoA or dilatations as early as in childhood.
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http://dx.doi.org/10.1007/s00380-018-1187-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6208677PMC
November 2018

Clinical cardiac regenerative studies in children.

World J Cardiol 2017 Feb;9(2):147-153

Imre J Pavo, Ina Michel-Behnke, Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, A-1090 Vienna, Austria.

Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients. We review here the available literature data, searching the MEDLINE, Google Scholar and EMBASE database for completed, and www.clinicaltrials.gov homepage for ongoing studies involving pediatric cardiac regeneration reports. Because of difficulties conducting randomized blinded clinical trials in pediatric patients, mostly case reports or cohort studies with a limited number of individuals have been published in the field of pediatric regenerative cardiology. The majority of pediatric autologous cell transplantations into the cardiac tissue have been performed in critically ill children with severe or terminal heart failure. Congenital heart disease, myocarditis, and idiopathic hypertrophic or dilated cardiomyopathy leading to congestive heart failure are some possible areas of interest for pediatric cardiac regeneration therapy. Autologous bone marrow mononuclear cells, progenitor cells, or cardiospheres have been applied either intracoronary or percutaneously intramyocardially in severely ill children, leading to a reported clinical benefit of cell-based cardiac therapies. In conclusion, compassionate use of autologous stem cell administration has led to at least short-term improvement in heart function and clinical stability in the majority of the critically ill pediatric patients.
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http://dx.doi.org/10.4330/wjc.v9.i2.147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5329741PMC
February 2017

Diagnostic performance and reference values of novel biomarkers of paediatric heart failure.

Heart 2016 10 24;102(20):1633-9. Epub 2016 May 24.

Department of Paediatrics and Adolescent Medicine, Division of Paediatric Cardiology, Medical University of Vienna, Vienna, Austria.

Objective: Biomarkers play a pivotal role in heart failure (HF) management. Reference values and insights from studies in adults cannot be extrapolated to the paediatric population due to important differences in pathophysiology and compensatory reserve. We assessed the diagnostic utility of four novel biomarkers in paediatric HF.

Methods: Midregional (MR) pro-atrial natriuretic peptide (proANP), soluble ST2 (sST2), growth differentiation factor-15 (GDF-15), MR-pro-adrenomedullin (proADM) and N-terminal pro-B natriuretic peptide (NT-proBNP) were measured in 114 patients and 89 controls. HF was defined as the presence of HF symptoms and/or abnormal systolic ventricular function. Receiver-operating characteristics were plotted, and the area under the curve (AUC) was measured. This was repeated for subgroups with cardiomyopathy and congenital heart disease (CHD). Ventricular systolic function was measured by magnetic resonance or echocardiography. Reference values were calculated according to the current guidelines.

Results: The AUC for diagnosing HF was 0.76 for MR-proANP (CI 0.70 to 0.84) and 0.82 for NT-proBNP (CI 0.75 to 0.88). These parameters performed similarly in the subgroups with CHD and cardiomyopathy. By contrast, MR-proADM, GDF-15 and sST2 performed poorly. When used in conjunction with NT-proBNP, no parameter added significantly to its diagnostic accuracy. NT-proBNP, MR-proANP, GDF-15 and sST2 could accurately discriminate between patients with preserved and patients with poor functional status. In a subset of patients with dilated cardiomyopathy, NT-proBNP, MR-proANP, MR-proADM and GDF-15 were associated with poor LV function.

Conclusions: MR-proANP could accurately detect HF in children and adolescents. Its diagnostic performance was comparable with that of NT-proBNP, regardless of the underlying condition. Reference values are presented.
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http://dx.doi.org/10.1136/heartjnl-2016-309460DOI Listing
October 2016

Transthoracic echocardiography in the evaluation of pediatric pulmonary hypertension and ventricular dysfunction.

Pulm Circ 2016 Mar;6(1):15-29

Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.

Transthoracic echocardiography (TTE) is the most accessible noninvasive diagnostic procedure for the initial assessment of pediatric pulmonary hypertension (PH). This review focuses on principles and use of TTE to determine morphologic and functional parameters that are also useful for follow-up investigations in pediatric PH patients. A basic echocardiographic study of a patient with PH commonly includes the hemodynamic calculation of the systolic pulmonary artery pressure (PAP), the mean and diastolic PAP, the pulmonary artery acceleration time, and the presence of a pericardial effusion. A more detailed TTE investigation of the right ventricle (RV) includes assessment of its size and function. RV function can be evaluated by RV longitudinal systolic performance (e.g., tricuspid annular plane systolic excursion), the tricuspid regurgitation velocity/right ventricular outflow tract velocity time integral ratio, the fractional area change, tissue Doppler imaging-derived parameters, strain measurements, the systolic-to-diastolic duration ratio, the myocardial performance (Tei) index, the RV/left ventricle (LV) diameter ratio, the LV eccentricity index, determination of an enlarged right atrium and RV size, and RV volume determination by 3-dimensional echocardiography. Here, we discuss the potential use and limitations of TTE techniques in children with PH and/or ventricular dysfunction. We suggest a protocol for TTE assessment of PH and myocardial function that helps to identify PH patients and their response to pharmacotherapy. The outlined protocol focuses on the detailed assessment of the hypertensive RV; RV-LV crosstalk must be analyzed separately in the evaluation of different pathologies that account for pediatric PH.
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http://dx.doi.org/10.1086/685051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4860554PMC
March 2016

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Heart 2016 May;102 Suppl 2:ii86-100

Department of Paediatric Cardiology, German Paediatric Heart Centre, Sankt Augustin, Germany.

Unlabelled: : The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction.

Methods: The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms 'pulmonary hypertensioń' and 5-10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children.

Results: A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH.

Conclusions: The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.
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http://dx.doi.org/10.1136/heartjnl-2015-309132DOI Listing
May 2016

Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Heart 2016 May;102 Suppl 2:ii42-8

Division of Paediatric Cardiology, Pediatric Heart Center, University Hospital for Children and Adolescents, Medical University Vienna, Vienna, Austria.

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance. Evaluation and treatment of PAH-CHD/PPHVD-CHD can be divided into in different topics. First, defining criteria for operability and initiation of advanced therapies preoperatively and postoperatively is an unresolved issue. Second, management of Eisenmenger syndrome is still an important question, with recent evidence on the severity of the disease and a more rapidly progressive course than previously described. Third, the Fontan circulation with no subpulmonary ventricle requires a distinct discussion, definition and classification since even a mild rise in pulmonary vascular resistance may lead to the so-called failing Fontan situation. Patients with CHD and single-ventricle physiology (Fontan/total cavopulmonary anastomosis) require a particularly stepwise and individualised approach. This consensus statement is on the current evidence for the most accurate evaluation and treatment of increased pulmonary artery pressure and resistance, as well as ventricular dysfunction, in children with congenital heart defects, and provides according practical recommendations. To optimise preoperative and postoperative management in patients with PAH-CHD, diagnostic and treatment algorithms are provided.
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http://dx.doi.org/10.1136/heartjnl-2015-308378DOI Listing
May 2016

Monitoring unfractionated heparin in children: a parallel-cohort randomized controlled trial comparing 2 dose protocols.

Blood 2015 Oct 3;126(18):2091-7. Epub 2015 Aug 3.

Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Austria;

Monitoring unfractionated heparin (UFH) is crucial to prevent over- or under-anticoagulation. However, the optimal parameters for monitoring UFH in children are not well established. The study objectives were to investigate (1) the relationship between UFH dose and its anticoagulant effect as assessed by anti-Xa, activated partial thromboplastin time (aPTT) and activated clotting time (ACT); (2) other factors influencing UFH effect; (3) the agreement between the assays; and (4) the association between UFH effect and clinical outcome. HEARTCAT was a parallel-cohort randomized controlled trial comparing high-dose (100 U/kg bolus followed by age-based continuous infusion in randomized children) vs low-dose UFH (50 U/kg bolus) during cardiac catheterization in children. Blood samples were drawn before and after UFH administration at 30, 60, and 90 minutes. Four-hundred and two samples of 149 patients were evaluable. Anti-Xa, aPTT, and ACT all showed good discrimination between UFH doses. Regression models demonstrated the following determinants of UFH effect: UFH dose, age, baseline antithrombin (for anti-Xa), and baseline levels of aPTT and ACT, respectively. UFH effects were lower in infants compared with older children, which was more pronounced at low-dose than at high-dose UFH. Agreement between the 3 assays was poor. Most aPTT values were above therapeutic range or beyond measuring limit and thus of limited value for UFH monitoring. No association of UFH dose or effect with clinical outcome could be observed. In conclusion, all assays reflected a significant UFH dose-effect relationship, however, with poor agreement between the respective tests. The age-dependency of UFH effect was confirmed. Notably, the influence of age on UFH effect was dose-dependent.
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http://dx.doi.org/10.1182/blood-2015-06-651661DOI Listing
October 2015

Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection.

Catheter Cardiovasc Interv 2015 Dec 29;86(7):1189-94. Epub 2015 Jun 29.

Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.

Objectives: We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients.

Background: In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure.

Methods And Results: From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.2 years, interquartile range (IQR) 2.7-3.7 years)) with failing Fontan circulation and exceeding systemic venous pressure. In 16 patients, the procedure was performed for acute postoperative failure 1-24 days after surgery. After perforation of the conduit and atrial wall by a Brockenbrough needle and gradual balloon dilation, premounted stents were expanded to create a diabolo configuration with flaring stent edges, leaving a slight but definitive central waist. The procedure resulted in regression of pleural effusions and a significant decrease in systemic venous pressure. Clinical improvement was observed in 16 of the 19 treated patients. Follow-up demonstrated sustained fenestration in 85% of treated patients for at least 24 months.

Conclusion: Transcatheter creation of a Fontan fenestration is a safe approach despite the anatomic gap between the extracardiac conduit cavity and the atrial wall. Stent implantation allows defining the diameter of the fenestration, reduces spontaneous occlusion, and ensures sustained clinical improvement.
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http://dx.doi.org/10.1002/ccd.26042DOI Listing
December 2015

Fifteen-year single center experience with the "Giessen Hybrid" approach for hypoplastic left heart and variants: current strategies and outcomes.

Pediatr Cardiol 2015 Feb 2;36(2):365-73. Epub 2014 Sep 2.

Pediatric Heart Center, Justus-Liebig University, Feulgenstr. 12, 30385, Giessen, Germany,

Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a "Giessen Hybrid" stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2%), and the interstage I mortality was 6.7%, and stage II mortality 9%, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84% and following BVR 89%, respectively. The Fifteen-year survival rate for HLHS and variants was 77%, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.
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http://dx.doi.org/10.1007/s00246-014-1015-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4303711PMC
February 2015
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