A5046530892- Pulmonary Hypertension Research and Treatments
- Congenital Heart Disease Studies
- Cardiovascular Issues in Pregnancy
- Neonatal Respiratory Health Research
- Nitric Oxide and Endothelin Effects
- Vascular Anomalies and Treatments
- Liver Disease and Transplantation
- Transplantation: Methods and Outcomes
- Cardiac Arrhythmias and Treatments
- Congenital Diaphragmatic Hernia Studies
- Cardiovascular Function and Risk Factors
- Neuroscience of respiration and sleep
- Tracheal and airway disorders
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Respiratory Support and Mechanisms
- Heart Failure Treatment and Management
- Coronary Artery Anomalies
- Mitochondrial Function and Pathology
- Congenital heart defects research
- Cardiac Structural Anomalies and Repair
- ATP Synthase and ATPases Research
- Renal and Vascular Pathologies
- Cardiovascular Conditions and Treatments
- Blood Pressure and Hypertension Studies
Assistance Publique – Hôpitaux de Paris
2015-2025
Hôpital Necker-Enfants Malades
2016-2025
Université Paris Cité
2015-2024
Institut Necker Enfants Malades
2023-2024
Hôpital Pellegrin
2023
Hôpital Robert-Debré
2022
Délégation Paris 5
2000-2020
Sorbonne Paris Cité
2012-2020
Inserm
2009-2018
Hôpital Européen Georges-Pompidou
2002-2009
Background— Congenital heart disease can be complicated by pulmonary arterial hypertension (PAH), the reversibility of which is often difficult to predict. We recently reported a lung biopsy study showing impaired apoptotic regulation endothelial cells in irreversible PAH. The objective present was identify noninvasive biomarkers turnover that could used congenital patients at risk Methods and Results— Circulating (CECs) isolated with CD146-coated beads circulating CD34 + CD133 progenitor...
Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH).A retrospective multicentre study was performed to assess short- and long-term outcomes after shunt.From 2003 2014, 24 underwent a [19 surgical, median age: 7.7 years (1.5-17 years), weight: 19.5 kg (10.2-47 kg) 5 transcatheter, 8.1 (2.3-9.7 22 (12.5-31 kg)] for drug-refractory PAH. For the first time humans, we an unidirectional valved anastomosis child infrasystemic PAH on...
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. objective this study was to determine the mutation frequency PH genes a cohort and describe clinical characteristics carriers.The involved 66 index cases with PH: 35 children idiopathic arterial (IPAH); five familial PAH (FPAH); three veno-occlusive disease (PVOD); 23 associated congenital heart (APAH-CHD).No were found APAH-CHD. In 40 IPAH or FPAH, 12 found: on BMPR2; four ACVRL1; TBX4. PVOD...
Heritable forms of pulmonary arterial hypertension (PAH) and veno-occlusive disease/pulmonary capillary haemangiomatosis (PVOD/PCH) diverge by lung histopathological lesions, clinical para-clinical presentation, their responsible genes, mode transmission. Since the identification BMPR2 gene in families affected PAH, mutations several other genes have been discovered for both forms. The mutation landscape these new is not yet well known.We set up a next-generation sequencing-based targeted...
Introduction TBX4 mutation causes small patella syndrome (SPS) and/or pulmonary arterial hypertension (PAH). The characteristics and outcomes of PAH associated with mutations are largely unknown. Methods We report the clinical, functional, radiologic, histologic haemodynamic heritable patients carrying a from French (PH) network. Results 20 were identified in 17 families. They characterised by median age at diagnosis 29 years (0–76 years) female to male ratio three. Most (70%) New York Heart...
Background Bone morphogenetic proteins 9 and 10 (BMP9 BMP10), encoded by GDF2 BMP10 , respectively, play a pivotal role in pulmonary vascular regulation. variants have been reported arterial hypertension (PAH) hereditary haemorrhagic telangiectasia (HHT). However, the phenotype of carriers remains largely unexplored. Methods We report characteristics outcomes PAH patients from French Dutch registries. A literature review explored phenotypic spectrum these patients. Results 26 were...
Pulmonary vasodilators in general and prostacyclin therapy particular, have markedly improved the outcome of patients with pulmonary arterial hypertension (PAH). As endothelial dysfunction is a key feature PAH, as progenitor cells (EPC) may contribute to vascular repair we suspected that might enhance EPC numbers functions. In present study, objectives were determine whether vasodilator treatment efficacy PAH.We quantified CD34+ cells, CFU-Hill ECFC (endothelial colony forming cells)...
PurposeAlthough some caregivers are using epigallocatechin gallate (EGCG) off label in hopes of improving cognition young adults with Down syndrome (DS), nothing is known about its safety, tolerability, and efficacy the DS pediatric population. We aimed to evaluate safety tolerability a dietary supplement containing EGCG if improves cognitive functional performance.MethodsA total 73 children (aged 6-12 years) were randomized. Participants received 0.5% (10 mg/kg daily dose) or placebo for 6...
Background Pulmonary vasodilators in general and prostacyclin analogues particular have improved the outcome of patients with pulmonary arterial hypertension (PAH). Endothelial dysfunction is a key feature PAH we previously described that circulating endothelial cell (CEC) level could be used as biomarker PAH. We now hypothesized an efficient PAH-specific vasodilator therapy might decrease CEC level. Methods/Results CECs were prospectively quantified by immunomagnetic separation mAb...
Abstract Objectives The aim of this study was to determine the probability intervention at birth after prenatal diagnosis CHD. Methods A 10-year retrospective including all foetuses with a prenatally diagnosed CHD and those delivered in tertiary-care cardiac centre between January, 2002 December, 2011 carried out. Patients were classified into eight groups according anticipated risk neonatal intervention. Results need for urgent and/or PGE1 infusion within first 48 hours life 47%...
Treatment strategies in paediatric pulmonary arterial hypertension (PAH) have evolved over the last years, but survival is still poor. Recently, adults with severe PAH, upfront triple combination therapy (uTCT) from diagnosis has been reported to show significant clinical improvement and excellent long-term outcome. This retrospective, observational study aimed assess efficacy of uTCT PAH.Children diagnosed PAH between 2010 2019 started were included. World Health Organization Functional...
Background Right heart catheterization (RHC) is a high-risk procedure in children with pulmonary arterial hypertension without clear guidelines for the indications and targets of invasive reassessment. Our objectives are to define aims repeated RHC evaluate correlation between noninvasive criteria hemodynamic parameters. Methods Results Clinical characteristics from 71 incident treatment-naïve (median age 6.2 years) who had baseline reevaluation were analyzed. Correlations predictors...
ABSTRACT Aim Pulmonary hypertension is frequent in neonates with a congenital diaphragmatic hernia, but long‐term data have been scarce. Our aim was to examine its prevalence, evolution and management identify factors associated persistence. Methods This French retrospective cohort study compared the characteristics of who had persistent pulmonary hypertension, or died it, those normalised pressures at 1 month year age. Results Most (92%) 88 we studied underwent surgery. Two‐thirds (67%)...