4.0 Article

Recommendations from the Association for European Paediatric and Congenital Cardiology for training in pulmonary hypertension

Journal

CARDIOLOGY IN THE YOUNG
Volume 29, Issue 11, Pages 1323-1327

Publisher

CAMBRIDGE UNIV PRESS
DOI: 10.1017/S104795111900235X

Keywords

Pulmonary hypertension; children; young adults; heart disease; Association for European Paediatric and Congenital Cardiology

Funding

  1. German Research Foundation (DFG) [KFO 311, HA4348/6-1]

Ask authors/readers for more resources

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient's age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group pulmonary hypertension, heart failure and transplantation has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.0
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

Correction Cardiac & Cardiovascular Systems

Managing Uncertainty in Decision-Making of Common Congenital Cardiac Defects (vol32, pg.no: 1705, 2022)

Colin J. McMahon, Skaiste Sendzikaite, Anusha Jegatheeswaran, Yiu-Fai Cheung, David S. Majdalany, Vibeke Hjortdal, Andrew N. Redington, Jeffrey P. Jacobs, Maryam Asoodar, Matthew Sibbald, Tal Geva, Jeroen J. G. van Merrienboer, Justin T. Tretter

CARDIOLOGY IN THE YOUNG (2023)

Article Pediatrics

Central pontine myelinolysis and locked-IN syndrome associated with tacrolimus after pediatric heart transplantation

Dimpna C. C. Albert-Brotons, Waleed Alharkan, Reem Beheri, Mohammed AlHaddad, Yasser Alali, Yasser Alheraish, Aziza Chedrawi, Zohair AlHalees

Summary: This article presents a case of a 10-year-old boy who developed severe locked-in syndrome after heart transplantation. The patient had a spontaneous recovery after supportive treatment and substitution of calcineurin inhibitor with an mTOR inhibitor (sirolimus). This is the first reported case of locked-in syndrome post-heart transplant in pediatrics cases in the literature.

PEDIATRIC TRANSPLANTATION (2023)

Article Virology

Only Subclinical Alterations in the Haemostatic System of People with Diabetes after COVID-19 Vaccination

Margret Paar, Faisal Aziz, Caren Sourij, Norbert J. Tripolt, Harald Kojzar, Alexander Mueller, Peter Pferschy, Anna Obermayer, Tamara Banfic, Bruno Di Geronimo Quintero, Nandu Goswami, Axel Schlagenhauf, Martin Koestenberger, Thomas Baernthaler, Thomas Wagner, Andelko Hrzenjak, Willibald Wonisch, Gilbert Reibnegger, Reinhard B. Raggam, Harald Sourij, Gerhard Cvirn

Summary: The coagulation responses of people with diabetes to COVID-19 vaccination were only subclinical and comparable to those observed in healthy individuals.

VIRUSES-BASEL (2023)

Article Medicine, General & Internal

Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation

Katharina Meinel, Felicitas Korak, Martin Dusleag, Tanja Strini, Daniela Baumgartner, Ante Burmas, Hannes Sallmon, Barbara Zieger, Axel Schlagenhauf, Martin Koestenberger

Summary: This study explores the relationship between hemodynamic alterations in Fontan patients and hemostatic dysbalance and Fontan-associated liver disease. The accumulation of bile acids is found to be related to certain hemostatic factors, and aspirin may reduce complications. Screening for AVWS is recommended for Fontan patients.

JOURNAL OF CLINICAL MEDICINE (2023)

Article Cardiac & Cardiovascular Systems

Simultaneous Aortic and Pulmonary Valve Replacement in Repaired Congenital Heart Disease

Dmitry Bobylev, Klea Hysko, Murat Avsar, Tomislav Cvitkovic, Elena Petena, Samir Sarikouch, Mechthild Westhoff Bleck, Georg Hansmann, Axel Haverich, Alexander Horke

Summary: Patients with congenital heart disease often require surgical or percutaneous valve replacement after initial repair. Simultaneous replacement of both semilunar valves is challenging and associated with increased complexity, morbidity, and mortality. This study analyzes the outcomes of simultaneous aortic and pulmonary valve replacements following multiple surgical interventions for congenital heart disease.

THORACIC AND CARDIOVASCULAR SURGEON (2023)

Article Cardiac & Cardiovascular Systems

Prognostic Value of Transthoracic Echocardiography in Children With Pulmonary Arterial Hypertension

Astrid E. Lammers, Jan Marek, Gerhard-Paul Diller, Sheila G. Haworth, Shahin Moledina

Summary: This study aimed to develop a comprehensive and simple echocardiographic risk stratification for children with pulmonary arterial hypertension (PAH). Through echocardiographic assessment of 63 children with PAH, it was found that right atrial area and left ventricular diastolic eccentricity index were robust predictors of transplant-free survival. Additionally, a combination of right atrial area, left ventricular diastolic eccentricity index, and tricuspid annular plane systolic excursion were identified as independent predictors of mortality. These findings suggest the potential use of simple risk scores for prognosis determination in children with PAH.

JOURNAL OF THE AMERICAN HEART ASSOCIATION (2023)

Article Cardiac & Cardiovascular Systems

Characterization of Aortic Flow Patterns by High-Frame-Rate Blood Speckle Tracking Echocardiography in Children

Massimiliano Cantinotti, Pietro Marchese, Marco Scalese, Raffaele Giordano, Eliana Franchi, Nadia Assanta, Martin Koestenberger, Benjamin T. Barnes, Simona Celi, Vivek Jani, Inga Voges, Shelby Kutty

Summary: This study demonstrates the feasibility and reproducibility of blood speckle tracking echocardiography in the aortas of healthy children, which allows for direct quantification of interventricular and aortic flow profiles. These findings provide a baseline for evaluating aortic flow patterns in children with congenital and acquired heart disease.

JOURNAL OF THE AMERICAN HEART ASSOCIATION (2023)

Review Pediatrics

Cardiac Arrhythmias Requiring Electric Countershock during the Neonatal Period-A Systematic Review

Nathalie Oeffl, Marlies Krainer, Stefan Kurath-Koller, Martin Koestenberger, Bernhard Schwaberger, Berndt Urlesberger, Lukas P. Mileder

Summary: This article provides a comprehensive review of electric countershock therapy in neonatal arrhythmias, showing that it is an effective treatment option, especially in atrial flutter. Among the 113 reported cases, atrial flutter was the most common arrhythmia.

CHILDREN-BASEL (2023)

Review Pediatrics

Antiarrhythmic Drug Dosing in Children-Review of the Literature

Nathalie Oeffl, Lukas Schober, Patrick Faudon, Sabrina Schweintzger, Martin Manninger, Martin Koestenberger, Hannes Sallmon, Daniel Scherr, Stefan Kurath-Koller

Summary: Antiarrhythmic drugs are important for treating pediatric arrhythmias, but there is a lack of official guidelines on this topic. Some medications have specific dosage recommendations, while others have broad recommendations. To avoid uncertainties and errors in dosing, we summarized published dosage recommendations for antiarrhythmic drugs in children. It is recommended for centers to develop their own protocols for pediatric antiarrhythmic drug therapy due to variations in availability, regulatory approval, and experience.

CHILDREN-BASEL (2023)

Article Cardiac & Cardiovascular Systems

Assessment for learning of paediatric cardiology trainees in 41 centres from 19 European countries

Colin J. Mcmahon, Ornella Milanesi, Olli Pitkaenen-Argillander, Dimpna C. Albert-Brotons, Ina Michel-Behnke, Inga Voges, Skaiste Sendzikaite, Ruth Heying

Summary: There is significant variation in instructional design and assessment among European paediatric cardiac centres. A wide range of assessment tools are used, with verbal summative feedback being the most common.

CARDIOLOGY IN THE YOUNG (2023)

Review Medicine, General & Internal

Echocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis

Massimiliano Cantinotti, Colin Joseph McMahon, Pietro Marchese, Martin Koestenberger, Marco Scalese, Eliana Franchi, Giuseppe Santoro, Nadia Assanta, Xander Jacquemyn, Shelby Kutty, Raffaele Giordano

Summary: The aim of this review is to assess the strengths and limitations of echocardiographic parameters and scores used to predict favorable outcome in complex congenital heart diseases (CHDs) with a borderline right ventricle. A systematic search was conducted in the National Library of Medicine and 22 studies were analyzed. The studies showed variability in the echocardiographic parameters and cutoff values used, making it difficult to determine the recommended measures. Despite these limitations, certain scores and measurements, such as TV/MV ratios and RV end-diastolic area, were found to be accurate predictors of successful repair in larger studies.

JOURNAL OF CLINICAL MEDICINE (2023)

Article Pharmacology & Pharmacy

Metformin Impedes Oxidation of LDL In Vitro

Christine Rossmann, Cornelia Ranz, Gerd Kager, Gerhard Ledinski, Martin Koestenberger, Willibald Wonisch, Thomas Wagner, Sebastian P. Schwaminger, Bruno Di Geronimo, Andelko Hrzenjak, Seth Hallstoem, Gilbert Reibnegger, Gerhard Cvirn, Margret Paar

Summary: This study investigated whether metformin can inhibit the oxidation of LDL, a crucial step in the development of endothelial dysfunction and atherosclerosis. The results showed that metformin effectively reduced lipid hydroperoxide formation and decreased the oxidation level of LDL. This study suggests that metformin, in addition to its glucose-lowering effect, may be a suitable agent for inhibiting the development of atherosclerosis.

PHARMACEUTICS (2023)

Article Cardiac & Cardiovascular Systems

Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up

Thomas Jack, Julia Carlens, Franziska Diekmann, Hosan Hasan, Philippe Chouvarine, Nicolaus Schwerk, Carsten Mueller, Ivonne Wieland, Igor Tudorache, Gregor Warnecke, Murat Avsar, Alexander Horke, Fabio Ius, Dmitry Bobylev, Georg Hansmann

Summary: A prospective observational study at Hannover Medical School showed that lung transplantation (LuTx) in children with end-stage pulmonary arterial hypertension (PAH) resulted in excellent midterm outcomes with 100% survival at 2 years post-LuTx. Postoperative VA-ECMO support facilitated early extubation and contributed to sustained biventricular reverse-remodeling and improved systolic function.

FRONTIERS IN CARDIOVASCULAR MEDICINE (2023)

Article Pediatrics

Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey

Tanguy Dutilleux, Nesrine Farhat, Ruth Heying, Marie-Christine Seghaye, Maurice Beghetti

Summary: This case report presents a 7-year-old boy with idiopathic pulmonary arterial hypertension (IPAH), who initially presented with cardiac murmur and exercise intolerance. Pulmonary hypertension was confirmed by echocardiography and cardiac catheterization. Despite treatment with sildenafil and bosentan, the patient's pulmonary artery pressure remained stable but not decreased. Subsequent follow-up showed an increase in estimated pulmonary pressure and worsening of the child's condition.

PEDIATRIC REPORTS (2023)

Article Cardiac & Cardiovascular Systems

Second International Pulmonary Hypertension/Heart Failure Symposium-Structural heart disease, right ventricular dysfunction, and stem cell therapy: The European Pediatric Pulmonary Vascular Disease Network

Philippe Chouvarine, Klea Hysko, Stephen Y. Chan, Paulo Oliveira, Bradley A. Maron, Stella Kourembanas, Georg Hansmann

PULMONARY CIRCULATION (2023)

No Data Available