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1. Predicting outcome in children with dilated cardiomyopathy: the use of repeated measurements of risk factors for outcome

Author

Marijke van derMeulen, Susanna denBoer, Gideon J. duMarchie Sarvaas, Nico Blom, Arend D.J. tenHarkel, Hans M.P.J. Breur, Lukas A.J. Rammeloo, Ronald Tanke, Ad J.J.C. Bogers, Willem A. Helbing, Eric Boersma, and Michiel Dalinghaus

Subjects
Dilated cardiomyopathy, Paediatric cardiology, Risk factors, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Abstract Aims We aimed to determine whether in children with dilated cardiomyopathy repeated measurement of known risk factors for death or heart transplantation (HTx) during disease progression can identify children at the highest risk for adverse outcome. Methods and results Of 137 children we included in a prospective cohort, 36 (26%) reached the study endpoint (SE: all‐cause death or HTx), 15 (11%) died at a median of 0.09 years [inter‐quartile range (IQR) 0.03–0.7] after diagnosis, and 21 (15%) underwent HTx at a median of 2.9 years [IQR 0.8–6.1] after diagnosis. Median follow‐up was 2.1 years [IQR 0.8–4.3]. Twenty‐three children recovered at a median of 0.6 years [IQR 0.5–1.4] after diagnosis, and 78 children had ongoing disease at the end of the study. Children who reached the SE could be distinguished from those who did not, based on the temporal evolution of four risk factors: stunting of length growth (−0.42 vs. −0.02 length Z‐score per year, P 6 years at presentation (all P

Published
2021
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2. Practical Workflow for Cardiovascular Assessment and Follow-Up in Kawasaki Disease Based on Expert Opinion

Author

Diana van Stijn, R. Nils Planken, Maarten Groenink, Nico Blom, Robbert J. de Winter, Taco Kuijpers, and Irene Kuipers

Subjects
Kawasaki disease, mucocutaneous lymph node syndrome, imaging, coronary artery aneurysms, cardiovascular assessment, Pediatrics, RJ1-570
Abstract

BackgroundApproximately 25% of the patients with a history of Kawasaki disease (KD) develop coronary artery pathology if left untreated, with coronary artery aneurysms (CAA) as an early hallmark. Depending on the severity of CAAs, these patients are at risk of myocardial ischemia, infarction and sudden death. In order to reduce cardiac complications it is crucial to accurately identify patients with coronary artery pathology by an integrated cardiovascular program, tailored to the severity of the existing coronary artery pathology.MethodsThe development of this practical workflow for the cardiovascular assessment of KD patients involve expert opinions of pediatric cardiologists, infectious disease specialists and radiology experts with clinical experience in a tertiary KD reference center of more than 1000 KD patients. Literature was analyzed and an overview of the currently most used guidelines is given.ConclusionsWe present a patient-specific step-by-step, integrated cardiovascular follow-up approach based on expert opinion of a multidisciplinary panel with expertise in KD.

Published
2022
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3. Executive Summary: HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

Author

Silvia G. Priori, MD, PhD, Arthur A. Wilde, MD, PhD, Minoru Horie, MD, PhD, Yongkeun Cho, MD, PhD, Elijah R. Behr, MA, MBBS, MD, FRCP, Charles Berul, MD, FHRS, CCDS, Nico Blom, MD, PhD, Josep Brugada, MD, PhD, Chern-En Chiang, MD, PhD, Heikki Huikuri, MD, Prince Kannankeril, MD, Andrew Krahn, MD, FHRS, Antoine Leenhardt, MD, Arthur Moss, MD, Peter J. Schwartz, MD, Wataru Shimizu, MD, PhD, Gordon Tomaselli, MD, FHRS, and Cynthia Tracy, MD

Subjects
Diseases of the circulatory (Cardiovascular) system, RC666-701
Published
2014
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4. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

Author

Silvia G. Priori, MD, PhD, Arthur A. Wilde, MD, PhD, Minoru Horie, MD, PhD, Yongkeun Cho, MD, PhD, Elijah R. Behr, MA, MBBS, MD, FRCP, Charles Berul, MD, FHRS, CCDS, Nico Blom, MD, PhD, Josep Brugada, MD, PhD, Chern-En Chiang, MD, PhD, Heikki Huikuri, MD, Prince Kannankeril, MD, Andrew Krahn, MD, FHRS, Antoine Leenhardt, MD, Arthur Moss, MD, Peter J. Schwartz, Wataru Shimizu, MD, PhD, Gordon Tomaselli, MD, FHRS, and Cynthia Tracy, MD

Subjects
Diseases of the circulatory (Cardiovascular) system, RC666-701
Published
2014
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5. Genetic Evaluation of A Nation-Wide Dutch Pediatric DCM Cohort: The Use of Genetic Testing in Risk Stratification

Author

Marijke H. van der Meulen, Johanna C. Herkert, Susanna L. den Boer, Gideon J. du Marchie Sarvaas, Nico Blom, Arend D.J. ten Harkel, Hans M.P.J. Breur, Lukas A.J. Rammeloo, Ronald Tanke, Carlo Marcelis, Ingrid M.B.H. van de Laar, Judith M.A. Verhagen, Ronald H. Lekanne dit Deprez, Daniela Q.C.M. Barge-Schaapveld, Annette Baas, Arjan Sammani, Imke Christiaans, J. Peter van Tintelen, Michiel Dalinghaus, Paediatric Cardiology, ACS - Heart failure & arrhythmias, Human Genetics, ACS - Pulmonary hypertension & thrombosis, ARD - Amsterdam Reproduction and Development, Pediatrics, and Clinical Genetics

Subjects
Cardiomyopathy, Dilated, Myocarditis, All institutes and research themes of the Radboud University Medical Center, Neurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7], Other Research Radboud Institute for Health Sciences [Radboudumc 0], Humans, pediatric cardiology, General Medicine, Genetic Testing, cardiomyopathy, dilated, Risk Assessment, Genetic Association Studies
Abstract

Background: This study aimed to describe the current practice and results of genetic evaluation in Dutch children with dilated cardiomyopathy and to evaluate genotype-phenotype correlations that may guide prognosis. Methods: We performed a multicenter observational study in children diagnosed with dilated cardiomyopathy, from 2010 to 2017. Results: One hundred forty-four children were included. Initial diagnostic categories were idiopathic dilated cardiomyopathy in 67 children (47%), myocarditis in 23 (16%), neuromuscular in 7 (5%), familial in 18 (13%), inborn error of metabolism in 4 (3%), malformation syndrome in 2 (1%), and “other” in 23 (16%). Median follow-up time was 2.1 years [IQR 1.0–4.3]. Hundred-seven patients (74%) underwent genetic testing. We found a likely pathogenic or pathogenic variant in 38 children (36%), most often in MYH7 (n = 8). In 1 patient initially diagnosed with myocarditis, a pathogenic LMNA variant was found. During the study, 39 patients (27%) reached study endpoint (SE: all-cause death or heart transplantation). Patients with a likely pathogenic or pathogenic variant were more likely to reach SE compared with those without (hazard ratio 2.8; 95% CI 1.3–5.8, P = 0.007), while transplant-free survival was significantly lower ( P = 0.006). Clinical characteristics at diagnosis did not differ between the 2 groups. Conclusions: Genetic testing is a valuable tool for predicting prognosis in children with dilated cardiomyopathy, with carriers of a likely pathogenic or pathogenic variant having a worse prognosis overall. Genetic testing should be incorporated in clinical work-up of all children with dilated cardiomyopathy regardless of presumed disease pathogenesis.

Published
2022
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7. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac Death. The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology

Author

Silvia G, Priori, Carina, Blomström-Lundqvist, Andrea, Mazzanti, Nico, Blom, Martin, Borggrefe, John, Camm, Perry Mark, Elliott, Donna, Fitzsimons, Robert, Hatala, Gerhard, Hindricks, Paulus, Kirchhof, Keld, Kjeldsen, Karl-Heinz, Kuck, Antonio, Hernandez-Madrid, Nikolaos, Nikolaou, Tone M, Norekvål, Christian, Spaulding, and Dirk J, Van Veldhuisen

Subjects
Heart Failure, Death, Sudden, Cardiac, Heart Diseases, Heart Ventricles, Decision Trees, Humans, Arrhythmias, Cardiac, Algorithms
Published
2016

8. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013

Author

Silvia G, Priori, Arthur A, Wilde, Minoru, Horie, Yongkeun, Cho, Elijah R, Behr, Charles, Berul, Nico, Blom, Josep, Brugada, Chern-En, Chiang, Heikki, Huikuri, Prince, Kannankeril, Andrew, Krahn, Antoine, Leenhardt, Arthur, Moss, Peter J, Schwartz, Wataru, Shimizu, Gordon, Tomaselli, and Cynthia, Tracy

Subjects
Consensus, Practice Guidelines as Topic, Disease Management, Humans, Arrhythmias, Cardiac, Genetic Predisposition to Disease, Syndrome, Congresses as Topic, Societies, Medical, Retrospective Studies
Published
2013

9. Impact of prenatal diagnosis of ductal origin of the left pulmonary artery

Author

Christine, van Velzen, Bauke, Adriaanse, Lukas, Rammeloo, Nico, Blom, and Jaroslav, Hruda

Subjects
Adult, Male, Postnatal Care, Brachial Artery, Infant, Newborn, Infant, Pulmonary Artery, Ultrasonography, Prenatal, Consanguinity, Pregnancy, Humans, Female, Ductus Arteriosus, Patent, Vascular Surgical Procedures
Published
2013

10. 2015 ESC GUIDELINES FOR THE MANAGEMENT OF PATIENTS WITH VENTRICULAR ARRHYTHMIAS AND THE PREVENTION OF SUDDEN CARDIAC DEATH

Author

Priori G. Silvia, Blomström-Lundqvist Carina, Andrea Mazzanti, Nico Bloma, Martin Borggrefe, John Camm, Perry Mark Elliott, Donna Fitzsimons, Robert Hatala, Gerhard Hindricks, Paulus Kirchhof, Keld Kjeldsen, Karl-Heinz Kuck, Antonio Hernandez, Nikolaos Nikolaou, Tone M. Norekvål, Christian Spaulding, and Dirk J. Van Veldhuisen

Subjects
acute coronary syndrome, cardiac resynchronization therapy, cardiomyopathy, congenital heart disease, defibrillator, guidelines, heart failure, implantable cardioverter defibrillator, myocardial infarction, resuscitation, stable coronary artery disease, sudden cardiac death, tachycardia, valvular heart, disease, ventricular arrhythmia, Diseases of the circulatory (Cardiovascular) system, RC666-701
Published
2016
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