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1. Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO)

Author

Damien Bonnet, Ali Houeijeh, Gilles Bosser, Clément Karsenty, Pamela Moceri, Pauline Helms, Lisa Guirgis, Elise Barre, Quentin Hauet, Sébastien Hascoët, Khaled Hadeed, Virginie Lambert, Xavier Iriart, Nicolas Pangaud, Bérangère Urbina-Hiel, Meriem Mostefa-Kara, Charlotte Denis, Eric Hery, Zakaria Jalal, Nadir Benbrik, Pierre Mauran, Pascale Maragnes, Hugues Lucron, Pascal Amedro, Céline Gronier, Francisco investigators, Magalie Ladouceur, Stéphanie Douchin, François Godart, Bruno Lefort, Karine Warin Fresse, Jean Benoit Thambo, Maurice Guirgis, Diala Khraiche, Adeline Basquin, Daniela Laux, Ronan Bonefoy, Estibaliz Valdeolmillos, Ivan Bouzguenda, Caroline Ovaert, Antoine Legendre, Laurence Iserin, Samir Harchaoui, Laurence Cohen, Jean Marc Lupoglazoff, Bertrand Leobon, Anne-Sophie Leborgne, Carine Vastel, Aurélie Chalard, Nicolas Combes, Alban-Elouen Baruteau, Hélène Ansquer, Guy Vaksmann, Lucile Houyel, Claire Bertail, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)

Subjects
Heart Septal Defects, Ventricular, Cardiac Catheterization, medicine.medical_specialty, Septal Occluder Device, Heart Ventricles, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Volume overload, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Ventricular outflow tract, Prospective Studies, 030212 general & internal medicine, Child, Prospective cohort study, Stroke, Heart Failure, business.industry, General Medicine, medicine.disease, Haemolysis, 3. Good health, Observational Studies as Topic, Treatment Outcome, Child, Preschool, Heart failure, Pediatrics, Perinatology and Child Health, Cardiology, Cardiology and Cardiovascular Medicine, business, Watchful waiting, Cohort study
Abstract

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. Background The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. Methods The Filiale de Cardiologie Pediatrique et Congenitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018–2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. Conclusions The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

Published
2021
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2. Exposure to low-dose ionizing radiation from cardiac catheterization and risk of cancer: the COCCINELLE study

Author

Sophie Malekzadeh-Milani, Jean-Benoit Thambo, Marie-Odile Bernier, S. Dreuil, Clément Karsenty, François Godart, Estelle Rage, Caroline Ovaert, Sylvie Di Filippo, Kossi Abalo, Claire Dauphin, Jean-François Piéchaud, Klervi Leuraud, Patrice Guerin, Sébastien Hascoët, Stéphanie Douchin, Sarah S. Cohen, Tiphaine Feuillet, Damien Bonnet, Pauline Helms, Pierre Mauran, Bruno Lefort, PSE-SANTE/SESANE/LEPID, Institut de Radioprotection et de Sûreté Nucléaire (IRSN), 2.M3C-Necker, Hôpital universitaire Necker-Enfants malades, Université de Paris, Paris, France, 3.Cardiology department, Hôpital Marie Lannelongue, Le Plessis Robinson, France, PSE-SANTE/SER/UEM, Cardiology department, Hôpital Marie Lannelongue, Le Plessis Robinson, France, Service de Cardiologie Maladies Vasculaires [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Paediatric and Congential Cardiology Department, Hôpital Femme-Mère-Enfant, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France., Cardiopédiatrie, hôpital couple enfant, CHU Grenoble Alpes, 38043 Grenoble cedex 9, France, Service de Cardiologie Infantile et Congénitale, Institut Cœur Poumon, 59037 Lille Cedex France, CHU Nantes, INSERM, Nantes Université, Clinique Cardiologique et des Maladies Vasculaires, CIC 1413, Institut du Thorax, Nantes, France., Unit of Cardiopediatrics, University Hospital of Strasbourg, Strasbourg, France, Institut des Cardiopathies Congénitales, CHRU Tours, 49 boulevard Béranger, 37000 Tours, France, Unité de cardiologie pédiatrique et congénitale, American Memorial Hospital, CHU de Reims, 47 rue Cognacq-Jay, 51092 Reims Cedex, France, Cardiologie pédiatrique et congénitale, Timone enfants, AP-HM et INSERM 1251, Aix-Marseille Université, Marseille France, Institut Hospitalier Jacques-Cartier, 91300 Massy, France, Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital (CHU), 33600 Pessac, France, M3C-Necker, Hôpital universitaire Necker-Enfants malades, Université de Paris, Paris, France, Laboratoire d épidémiologie des rayonnements ionisants (IRSN/PSE-SANTE/SESANE/LEPID), Service de recherche sur les effets biologiques et Sanitaires des rayonnements ionisants (IRSN/PSE-SANTE/SESANE), Institut de Radioprotection et de Sûreté Nucléaire (IRSN)-Institut de Radioprotection et de Sûreté Nucléaire (IRSN), Unité d'expertise en radioprotection médicale (IRSN/PSE-SANTE/SER/UEM), Service d'études et d'expertise en radioprotection (IRSN/PSE-SANTE/SER), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Hôpital Femme Mère Enfant [CHU - HCL] (HFME), and Hospices Civils de Lyon (HCL)

Subjects
medicine.medical_specialty, education.field_of_study, Childhood Cancer Registry, business.industry, medicine.medical_treatment, Incidence (epidemiology), [SDV]Life Sciences [q-bio], Population, Cancer, Immunosuppression, medicine.disease, Confidence interval, 030218 nuclear medicine & medical imaging, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, Internal medicine, Cohort, medicine, Cardiology and Cardiovascular Medicine, business, education, Cardiac catheterization
Abstract

International audience; Background The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of radiation associated cancer in patients who undergone cardiac catheterization (CC) procedures for diagnosis or treatment of congenital heart disease during childhood [1].Material and methods Children who undergone CC procedures from 01/01/2000 to 31/12/2013 before the age of 16 in one of the 15 pediatric cardiology departments which perform pediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure until the exit date, i.e. the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. The cohort was linked to the National Childhood Cancer Registry to identify patients diagnosed with cancer and with the French National Directory for the Identification of Natural Persons to retrieve the patients’ vital status. An external comparison was conducted using standardized incidence ratios (SIR). Breslow and Day’s approximation was used to estimate 95% confidence intervals (CI) for the SIRs [2]. Results A total of 17,104 children were included in the cohort and followed for 110,335 person-years, with 22,227 CC procedures collected. Among the patients, 81.6 % received only one procedure. Fifty-nine cancer cases were observed in the cohort. SIRs were increased for all-cancer (SIR = 3.8, 95% CI 2.9, 4.9), leukemia (SIR = 3.3, 95% CI 2.0, 5.4), lymphoma (SIR = 14.9, 95% CI 9.9, 22.5) and solid cancers excluding central nervous system tumors (SIR = 3.3, 95% CI 2.0, 5.5) compared with the general population. Conclusion Increased risks of cancer were observed. Potential explanations include shared genetic or environmental factors, immunosuppression drugs, and exposure to medical ionizing radiation procedures. The dose-response analysis between ionizing radiation doses received during CCs and cancer occurrence will allow to address this question.Keywords: Ionizing radiation, cardiac catheterization, congenital heart disease, cancerCompeting interestsThe authors declare that they have no competing of interest.References1.Baysson H, Nkoumazok B, Barnaoui S, Réhel J, Girodon B, Milani G, Boudjemline Y, Bonnet D, Laurier D, Bernier M (2015) Follow-up of children exposed to ionising radiation from cardiac catheterisation: the Coccinelle study. Radiation protection dosimetry 165:13–162.Breslow NE, Day NE (1987) Statistical Methods in Cancer Research Volume II: The Design and Analysis of Cohort Studies, IARC Scientific Publication. IARC Scientific Publication No. 82, Lyon, France: International Agency for Research on Cancer.NotesCOCCINELLE: French acronym for COhorte sur le risque de Cancer après Cardiologie INterventionnELLECC: Cardiac CatheterizationSIR: Standardized Incidence Ratios CI: Confidence IntervalsCNS: Central Nervous System

Published
2021
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3. Transcatheter closure of a perimembranous ventricular septal defect with Nit-Occlud Lê VSD Coil: A French multicentre study

Author

Xavier Iriart, Philippe Acar, Zakaria Jalal, Alban-Elouen Baruteau, Khaled Hadeed, Jean-Benoit Baudelet, Alain Fraisse, Clément Karsenty, Philippe Aldebert, François Heitz, Pierre Mauran, Lisa Guirguis, François Godart, Jean Benoit Thambo, Ender Odemis, Caroline Ovaert, Sébastien Hascoët, Ali Houeijeh, Pathologies Pulmonaires et Plasticité Cellulaire - UMR-S 1250 (P3CELL), and Université de Reims Champagne-Ardenne (URCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Aortic valve, medicine.medical_specialty, business.industry, medicine.medical_treatment, [SDV]Life Sciences [q-bio], General Medicine, 030204 cardiovascular system & hematology, Haemolysis, medicine.disease, Pulmonary hypertension, 3. Good health, Surgery, Aortic valvuloplasty, 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, Ventricle, Interquartile range, medicine, 030212 general & internal medicine, Cardiology and Cardiovascular Medicine, Complication, business, Atrioventricular block, ComputingMilieux_MISCELLANEOUS
Abstract

Summary Background Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Le VSD coil was recently introduced in France as an alternative to occluder devices. Aims To study the safety and feasibility of pmVSD closure with the Nit-Occlud Le VSD coil. Methods All consecutives cases of pmVSD closure with the Nit-Occlud Le VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. Results Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7–31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7–10) mm on the left ventricle side and 5 (4–6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7–95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9–26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4–20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P = 0.001), residual shunt (P = 0.007) and multi-exit VSD (P = 0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. Conclusion pmVSD closure with the Nit-Occlud Le VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Le VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Published
2020
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4. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study

Author

Pierre Mauran, Ali Houeijeh, Elise Barre, Clément Karsenty, Hélène Bouvaist, Pamela Moceri, Xavier Iriart, Elie Fadel, Adélaïde Richard, Nathalie Souletie, Emmanuelle Fournier, Lauriane Le Gloan, Magalie Ladouceur, Xavier Jaïs, Yvette Bernard, François Godart, Jelena Radojevic, Jérôme Petit, Laurence Iserin, Damien Bonnet, Gilles Bosser, Sébastien Hascoët, Pascal Amedro, Claire Dauphin, Adeline Basquin, Olivier Sitbon, Marc Humbert, Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Centre de Référence des cardiopathies congénitales (M3C), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris-Saclay, Hôpital Bicêtre, Centre hospitalier universitaire de Nantes (CHU Nantes), Service de médecine interne, hôpital Gabriel-Montpied, CHU Clermont-Ferrand, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Bordeaux [Bordeaux], Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Cardiologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Toulouse [Toulouse], Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Hôpital Pasteur [Nice] (CHU), CHU Grenoble, Centre Hospitalier Universitaire de Reims (CHU Reims), CHU Rouen, Normandie Université (NU), Service de cardiologie et maladies vasculaires, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], CHU Pontchaillou [Rennes], CHU Necker - Enfants Malades [AP-HP], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre chirurgical Marie Lannelongue, Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre chirurgical Marie Lannelongue, Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Service de Médecine Interne [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], and Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Male, Pediatrics, Time Factors, Kaplan-Meier Estimate, 030204 cardiovascular system & hematology, Pulmonary arterial hypertension, Gastroenterology, Congenital heart diseases, 0302 clinical medicine, Risk Factors, Cause of Death, 030212 general & internal medicine, Longitudinal Studies, Child, Cause of death, Outcome, Cardiopathies congénitales, Age Factors, Hypertension artérielle pulmonaire, General Medicine, Middle Aged, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, Syndrome d’Eisenmenger, 3. Good health, Treatment Outcome, Disease Progression, Female, France, Drug therapy, Cardiology and Cardiovascular Medicine, Cohort study, Médicament, Adult, medicine.medical_specialty, Adolescent, Hypertension, Pulmonary, Pulmonary Artery, Lower risk, Disease-Free Survival, 03 medical and health sciences, Young Adult, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Internal medicine, medicine, Pronostic, Humans, Arterial Pressure, Antihypertensive Agents, Proportional Hazards Models, Retrospective Studies, Chi-Square Distribution, business.industry, Eisenmenger syndrome, Retrospective cohort study, Eisenmenger Complex, medicine.disease, Pulmonary hypertension, Confidence interval, Transplantation, Multivariate Analysis, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business
Abstract

International audience; Background: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial.Aims: To investigate outcomes in patients with ES, and their relationship with PAH-SDT.Methods: Retrospective, observational, nationwide, multicentre cohort study.Results: We included 340 patients with ES: genetic syndrome (n = 119; 35.3%); pretricuspid defect (n = 75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA + PDE5I) 40.9%; triple therapy (ERA + PDE5I + prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0–9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4–47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8–21.6%] and 46.4% [95% confidence interval 38.2–55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P = 0.0001 and P = 0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P < 0.001 and P < 0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P = 0.002, P = 0.01 and P = 0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P = 0.009).Conclusions: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.; Contexte: L’intérêt du traitement médical spécifique (TMS) de l’hypertension artérielle pulmonaire (HTAP) dans le syndrome d’Eisenmenger (SE) est controversé.Objectifs: Étudier le pronostic à long terme des patients ayant un SE et la relation avec le TMS.Méthodes: Une cohorte observationnelle longitudinale multicentrique rétrospective historique française de 340 SE a été constituée.Résultats: Le shunt était prétricuspide dans 75 cas (22,1 %). Au total, 276 (81,2 %) patients étaient sous TMS (monothérapie 46,7 % ; bi-thérapie 40,9 % ; tri-thérapie 9,1 %). La durée médiane de TMS était de 5,5 ans [3,0–9,1]. Un événement clinique majeur (ECM : décès, transplantation cardiopulmonaire ou bipulmonaire) a été observé dans 95 (27,9 %) cas à un âge médian de 40,5 [29,4–47,6] ans. La survenue cumulée d’un ECM était de 16,7 % [IC 95 % 12,8–21,6 %] et 46,4 % [IC 95 % 38,2–55,4 %] à l’âge de 40 et 60 ans. Avec l’âge ou le délai depuis le premier examen comme échelle temporelle, la survenue cumulée des ECM était moindre chez les patients sous un ou deux TMS (p = 0,0001 et p = 0,004), en particulier chez les patients avec un shunt post-tricuspide (p < 0,001 et p < 0,02) comparée aux patients sans TMS. Une analyse multivariée de Cox avec le délai depuis le diagnostic de l’HTAP comme échelle temporelle a montré qu’une classe fonctionnelle III ou IV de la NYHA/WHO, une saturation périphérique en oxygène basse (en variable continue), un shunt pré-tricuspide et l’absence de TMS étaient associés à un risque augmenté d’ECM (p = 0,002 ; p = 0,01 ; p = 0,04 and p = 0,009, respectivement).Conclusions: Le TMS dans le SE semble associé à un meilleur pronostic. Néanmoins, même avec un traitement médical palliatif, le pronostic du SE reste altéré. Les patients avec un shunt prétricuspide ont un profil clinique et un pronostic plus sombre malgré une survenue plus tardive de l’HTAP.

Published
2017
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5. Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block

Author

Hervé Le Marec, Caroline Bonnet, Jean-René Lusson, Raphaël P. Martins, Jean-Jacques Schott, Francis Rouault, Alban-Elouen Baruteau, Jean-Benoit Thambo, Christian Dina, Elisabeth Villain, François Godart, Philippe Mabo, Vincent Probst, Alain Fraisse, François Marçon, Albin Behaghel, Véronique Gournay, Sophie Guillaumont, Yves Dulac, Jean-Marc Schleich, Stéphanie Chatel, Swanny Fouchard, Alain Chantepie, Béatrice Delasalle, Jean-Claude Daubert, unité de recherche de l'institut du thorax UMR1087 UMR6291 ( ITX ), Université de Nantes ( UN ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Department de Chirurgie cardiaque des cardiopathies congénitales, Centre chirurgical Marie Lannelongue, Service de cardiologie et maladies vasculaires, Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], CIC-IT Rennes, Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Laboratoire Traitement du Signal et de l'Image ( LTSI ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Laboratoire de génie des procédés - environnement - agroalimentaire ( GEPEA ), Mines Nantes ( Mines Nantes ) -Université de Nantes ( UN ) -Ecole Nationale Vétérinaire, Agroalimentaire et de l'alimentation Nantes-Atlantique ( ONIRIS ) -Centre National de la Recherche Scientifique ( CNRS ), Service de cardiologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service cardiologie pédiatrique [Bordeaux], CHU Bordeaux [Bordeaux], Service de Cardiologie Infantile [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Service de cardiologie pédiatrique [Nantes], Centre hospitalier universitaire de Nantes ( CHU Nantes ), Cabinet de Cardiologie pédiatrique, Service de cardiologie pédiatrique [Tours], CHU Tours, Service de cardiologie Pédiatrique [Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Service de cardiologie Pédiatrique [Lille], Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de cardiologie pédiatrique et congénitale adulte [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Service de cardiologie Pédiatrique [Marseille], Hôpital de la Timone [CHU - APHM] ( TIMONE ), Service de cardiologie [Clermont-Ferrand], CHU Clermont-Ferrand, Service pédiatrie-cardiologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de génie des procédés - environnement - agroalimentaire (GEPEA), Mines Nantes (Mines Nantes)-Université de Nantes - UFR des Sciences et des Techniques (UN UFR ST), Université de Nantes (UN)-Université de Nantes (UN)-Ecole Nationale Vétérinaire, Agroalimentaire et de l'alimentation Nantes-Atlantique (ONIRIS)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Cardiologie Maladies Vasculaires [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, CHU Toulouse [Toulouse], Unité de recherche de l'institut du thorax (ITX-lab), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Centre Chirurgical Marie Lannelongue (CCML), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Nantes (UN)-Université de Nantes (UN)-École nationale vétérinaire, agroalimentaire et de l'alimentation Nantes-Atlantique (ONIRIS)-Centre National de la Recherche Scientifique (CNRS), Service Cardiologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Le Corre, Morgane, and Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS)

Subjects
Male, Parents, MESH : Retrospective Studies, Heart malformation, MESH : NAV1.5 Voltage-Gated Sodium Channel, MESH : Prevalence, MESH : Aged, Electrocardiography, 0302 clinical medicine, MESH: Pregnancy, MESH : Child, Pregnancy, Prenatal Diagnosis, MESH: Child, Medicine, Sinus rhythm, genetics, Child, 0303 health sciences, MESH: Middle Aged, MESH: Genetic Testing, MESH : Infant, MESH: Infant, 3. Good health, MESH : Phenotype, MESH: Young Adult, MESH : Electrocardiography, Child, Preschool, Cardiology and Cardiovascular Medicine, medicine.medical_specialty, conduction, MESH : Young Adult, Prenatal diagnosis, MESH: Phenotype, Sudden death, 03 medical and health sciences, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Physiology (medical), MESH : Adolescent, MESH : Genetic Testing, Humans, MESH : Middle Aged, Genetic Testing, MESH: Prenatal Diagnosis, MESH : Prenatal Diagnosis, MESH: Prevalence, Aged, Retrospective Studies, [SDV.IB] Life Sciences [q-bio]/Bioengineering, MESH: Adolescent, MESH: Parents, MESH: Humans, MESH: Child, Preschool, MESH : Humans, Infant, MESH: Adult, MESH: Retrospective Studies, medicine.disease, MESH : Pregnancy, MESH : Genetic Predisposition to Disease, Atrioventricular block, MESH: Female, Pediatrics, ECG screening, 030204 cardiovascular system & hematology, MESH : Child, Preschool, NAV1.5 Voltage-Gated Sodium Channel, atrioventricular block, Prevalence, Mass Screening, MESH : Female, [ SDV.IB ] Life Sciences [q-bio]/Bioengineering, Family history, MESH : Parents, MESH: Aged, MESH: Infant, Newborn, MESH: Genetic Predisposition to Disease, Middle Aged, MESH : Adult, [ SDV.MHEP.CSC ] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, MESH : Atrioventricular Block, Phenotype, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, medicine.symptom, Adult, Adolescent, pediatrics, MESH : Male, MESH : Infant, Newborn, Asymptomatic, Young Adult, MESH: Atrioventricular Block, Genetic Predisposition to Disease, MESH: Mass Screening, PR interval, 030304 developmental biology, MESH : Mass Screening, business.industry, Infant, Newborn, MESH: NAV1.5 Voltage-Gated Sodium Channel, MESH: Male, MESH: Electrocardiography, business
Abstract

Background— The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. Methods and Results— A multicenter retrospective study (13 French referral centers, from 1980–2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0±6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively ( P P P P SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. Conclusions— ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Published
2012
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6. Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study

Author

François Marçon, Caroline Bonnet, Sophie Guillaumont, Elisabeth Villain, Véronique Gournay, François Godart, Albin Behaghel, Yves Dulac, Jean-Benoit Thambo, Swanny Fouchard, Vincent Probst, Jean-Jacques Schott, Jean-Marc Schleich, Alain Chantepie, Francis Rouault, Christophe Leclercq, Alban-Elouen Baruteau, Alain Fraisse, Hervé Le Marec, Jean-Claude Daubert, Jean-René Lusson, Philippe Mabo, Service de cardiologie et maladies vasculaires, Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], CIC-IT Rennes, Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Institut du thorax, Université de Nantes ( UN ) -IFR26-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Laboratoire Traitement du Signal et de l'Image ( LTSI ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), unité de recherche de l'institut du thorax UMR1087 UMR6291 ( ITX ), Université de Nantes ( UN ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Service de cardiologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service cardiologie pédiatrique [Bordeaux], CHU Bordeaux [Bordeaux], Service de Cardiologie Infantile [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Service de cardiologie pédiatrique [Nantes], Centre hospitalier universitaire de Nantes ( CHU Nantes ), Cabinet de Cardiologie pédiatrique, Service de cardiologie pédiatrique [Tours], CHU Tours, Service de cardiologie Pédiatrique [Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Service de cardiologie Pédiatrique [Lille], Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de cardiologie pédiatrique et congénitale adulte [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Service de cardiologie Pédiatrique [Marseille], Hôpital de la Timone [CHU - APHM] ( TIMONE ), Service de cardiologie [Clermont-Ferrand], CHU Clermont-Ferrand, Service pédiatrie-cardiologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, 'Protocole Hospitalier de Recherche Clinique' 2001 R20/03 and 2004 R20/07 from the University Medical Center of Nantes, France, the 2009 Philippe Coumel Research Grant from the French Society of Cardiology, grant no 05 CVD 01 (Preventing Sudden Death) from the Foundation Leducq Trans-Atlantic Network of Excellence, 'Agence Nationale de la Recherche' grant 05-MRAR-028., Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Nantes (UN)-IFR26-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Cardiologie Maladies Vasculaires [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, CHU Toulouse [Toulouse], Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de recherche de l'institut du thorax (ITX-lab), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service Cardiologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS)

Subjects
Male, Pacemaker, Artificial, Heart disease, MESH : Retrospective Studies, law.invention, Electrocardiography, 0302 clinical medicine, MESH: Pregnancy, MESH : Child, Pregnancy, Prenatal Diagnosis, MESH: Child, Age of Onset, Child, MESH: Treatment Outcome, MESH: Bundle-Branch Block, Clinical outcome, Cardiac Pacing, Artificial, MESH : Infant, MESH: Infant, 3. Good health, MESH : Age of Onset, Pacemaker, MESH: Young Adult, MESH : Electrocardiography, Child, Preschool, Disease Progression, MESH: Pacemaker, Artificial, MESH: Disease Progression, Cardiology and Cardiovascular Medicine, medicine.medical_specialty, MESH : Bundle-Branch Block, MESH: Age of Onset, MESH : Young Adult, MESH: Cardiac Pacing, Artificial, Disease-Free Survival, 03 medical and health sciences, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, MESH : Adolescent, Humans, MESH: Prenatal Diagnosis, MESH : Prenatal Diagnosis, Retrospective Studies, MESH: Adolescent, MESH: Humans, MESH: Child, Preschool, MESH : Humans, Infant, MESH: Adult, MESH: Retrospective Studies, MESH : Disease Progression, medicine.disease, MESH : Pregnancy, MESH: Disease-Free Survival, Atrioventricular block, MESH: Female, Pediatrics, Paediatric electrocardiology, 030204 cardiovascular system & hematology, MESH : Child, Preschool, Risk Factors, law, MESH: Risk Factors, MESH : Female, [ SDV.IB ] Life Sciences [q-bio]/Bioengineering, medicine.diagnostic_test, Dilated cardiomyopathy, MESH : Adult, [ SDV.MHEP.CSC ] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, MESH : Risk Factors, MESH : Atrioventricular Block, Treatment Outcome, MESH : Disease-Free Survival, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, medicine.symptom, Adult, Adolescent, MESH : Male, Bundle-Branch Block, MESH : Treatment Outcome, Asymptomatic, Young Adult, MESH: Atrioventricular Block, 030225 pediatrics, medicine, business.industry, MESH : Pacemaker, Artificial, Retrospective cohort study, MESH: Male, Surgery, MESH: Electrocardiography, MESH : Cardiac Pacing, Artificial, Artificial cardiac pacemaker, Age of onset, business
Abstract

International audience; AIMS: The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS: We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). CONCLUSION: In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.

Published
2012
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7. Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France

Author

Pierre Clerson, Damien Bonnet, Virginie Gressin, Xavier Jaïs, Maurice Beghetti, M. Voisin, Jean-Marc Schleich, Alain Fraisse, Sylvie Di Filippo, François Godart, Pascale Maragnès, Claire Dauphin, Service de cardiologie Pédiatrique [Marseille], Hôpital de la Timone [CHU - APHM] (TIMONE), Service de cardiologie et maladies vasculaires, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Service de Cardiologie Pédiatrique, Hospices Civils de Lyon (HCL)-Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Pediatric Cardiology Unit, Hôpital Universitaire de Genève, Actelion Pharmaceuticals, Actelion Pharmaceuticals France, Orgamétrie biostatistiques, Service de cardiologie Pédiatrique [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de Référence M3C Malformations Cardiaques Congénitales Complexes, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP], Hôpital de la Timone [CHU - APHM] ( TIMONE ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Hospices Civils de Lyon ( HCL ) -Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon ( HCL ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), and Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Descartes - Paris 5 ( UPD5 ) -PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP]

Subjects
Male, MESH : Hypertension, Pulmonary, Heart disease, MESH : Prevalence, Epidemiology, MESH : Prospective Studies, Pulmonary arterial hypertension, MESH : Antihypertensive Agents, 0302 clinical medicine, MESH : Child, Surveys and Questionnaires, MESH: Child, Prospective Studies, Child, MESH: Treatment Outcome, MESH : Hemodynamics, Congenital heart defect, MESH : Infant, General Medicine, MESH: Follow-Up Studies, MESH: Infant, 3. Good health, Child, Preschool, épidémiologie, Cardiology and Cardiovascular Medicine, Artery, Heart Defects, Congenital, medicine.medical_specialty, MESH: Hemodynamics, Hypertension, Pulmonary, Disease-Free Survival, 03 medical and health sciences, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, MESH : Adolescent, Humans, MESH : Heart Defects, Congenital, MESH: Kaplan-Meier Estimate, MESH: Prevalence, MESH: Adolescent, MESH: Humans, MESH: Hypertension, Pulmonary, MESH: Questionnaires, MESH : Humans, Qualité de vie, MESH: Child, Preschool, Infant, MESH : Follow-Up Studies, MESH: Heart Defects, Congenital, medicine.disease, 030228 respiratory system, MESH: Disease-Free Survival, MESH : Genetic Predisposition to Disease, MESH: Educational Status, MESH: Exercise Test, MESH: Female, MESH : Educational Status, [ SDV.MHEP.PSR ] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Pediatrics, Time Factors, [ SDV.MHEP.PED ] Life Sciences [q-bio]/Human health and pathology/Pediatrics, Hemodynamics, Kaplan-Meier Estimate, MESH : Child, Preschool, 030204 cardiovascular system & hematology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Risk Factors, MESH: Risk Factors, Prevalence, MESH : Female, [ SDV.IB ] Life Sciences [q-bio]/Bioengineering, Prospective cohort study, Respiratory disease, MESH: Genetic Predisposition to Disease, MESH : Questionnaires, Hypertension artérielle pulmonaire, [ SDV.MHEP.CSC ] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, MESH : Risk Factors, Treatment Outcome, medicine.anatomical_structure, Paediatric, Cardiology, MESH : Disease-Free Survival, Educational Status, Portal hypertension, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, France, MESH : Time Factors, Quality of life, Adolescent, MESH : Male, Pédiatrie, MESH : Treatment Outcome, MESH : Kaplan-Meier Estimate, Internal medicine, medicine, Genetic Predisposition to Disease, MESH : France, Cardiopathie congénitale, Antihypertensive Agents, MESH: Antihypertensive Agents, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, business.industry, MESH: Time Factors, MESH: Quality of Life, MESH : Quality of Life, Pulmonary hypertension, MESH: Male, MESH: Prospective Studies, MESH: France, MESH : Exercise Test, Exercise Test, business, Follow-Up Studies
Abstract

International audience; BACKGROUND: Limited data are available describing paediatric pulmonary arterial hypertension. AIMS: To characterize the epidemiology, management and impact on quality of life and outcome of paediatric pulmonary arterial hypertension, excluding persistent pulmonary hypertension of the newborn and pulmonary arterial hypertension caused by congenital heart disease. METHODS: In this multicentre study, children with pulmonary arterial hypertension were included and followed prospectively for two years at 21 referral centres in France. WHO functional class, 6-minute walk distance and quality of life (CHQ-PF50 questionnaire) were evaluated. RESULTS: Fifty children were included with a mean age of 8.9 +/- 5.4 years from May 2005 until June 2006. The estimated prevalence for pulmonary arterial hypertension was 3.7 cases/million. Patients had idiopathic pulmonary arterial hypertension (60%), familial pulmonary arterial hypertension (10%), pulmonary arterial hypertension associated with, but not caused by, congenital heart disease (24%), pulmonary arterial hypertension associated with connective tissue disease (4%) or portal hypertension (2%). During follow-up, the combination of pulmonary arterial hypertension-specific therapies was increasingly prescribed (44% patients versus 22% at inclusion). Patients remained stable regarding clinical status, 6-minute walk distance and quality of life. Survival estimates after one and two years were 86% (95% CI 76, 96) and 82% (95% CI 71, 93), respectively. CONCLUSIONS: In children, idiopathic/familial pulmonary arterial hypertension accounts for the majority of cases. A specific pulmonary arterial hypertension group in conjunction with congenital heart disease can be identified that resembles patients with idiopathic pulmonary arterial hypertension. Combined pulmonary arterial hypertension-specific therapies may have contributed to disease stability and favourable survival.

Published
2010
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