A5033001671- Pulmonary Hypertension Research and Treatments
- Congenital Heart Disease Studies
- Cardiovascular Issues in Pregnancy
- Vascular Anomalies and Treatments
- Heart Failure Treatment and Management
- Cardiovascular Function and Risk Factors
- Liver Disease and Transplantation
- Transplantation: Methods and Outcomes
- Cardiac Structural Anomalies and Repair
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Congenital Diaphragmatic Hernia Studies
- Ultrasound in Clinical Applications
- Coronary Artery Anomalies
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Inflammasome and immune disorders
- Gout, Hyperuricemia, Uric Acid
- Blood Pressure and Hypertension Studies
- Pediatric health and respiratory diseases
- Pleural and Pulmonary Diseases
- Asthma and respiratory diseases
- Cardiovascular and exercise physiology
- Cardiac Valve Diseases and Treatments
- Trauma Management and Diagnosis
University of Groningen
2014-2025
University Medical Center Groningen
2015-2025
Beatrix Kinderziekenhuis
2011-2021
Boston Children's Hospital
2014
Medisch Spectrum Twente
2009
Danbury Hospital
1985
Childhood-onset pulmonary arterial hypertension (PAH) is rare and differs from adult-onset disease in clinical presentation, with often unexplained mental retardation dysmorphic features (MR/DF). Mutations the major PAH gene, BMPR2, were reported to cause only 10-16% of childhood-onset patients. We aimed identify more genes associated PAH.We studied 20 consecutive cases idiopathic or heritable PAH. In patients accompanying MR/DF (n=6) array-comparative genomic hybridisation analysis was...
To be able to design goal-oriented treatment strategies in paediatric pulmonary arterial hypertension (PAH), we aimed identify goals by investigating the prognostic value of treatment-induced changes noninvasive predictors transplant-free survival. 66 consecutive, treatment-naïve PAH patients Dutch National Network for Paediatric Pulmonary Hypertension who started taking PAH-targeted drugs between January 2000 and April 2013 underwent prospective, standardised follow-up. Clinical,...
AimsTo assess the occurrence and prognostic value of acute vasodilator response (AVR) in paediatric vs. adult pulmonary arterial hypertension, idiopathic/hereditary hypertension (iPAH/HPAH) associated with congenital heart disease (PAH-CHD) using three different criteria.
Background— The value of echocardiography in assessing disease severity and predicting outcome pediatric pulmonary arterial hypertension (PAH) is insufficiently defined. aim this study was to describe correlations between PAH. Methods Results— Forty-three consecutive children (median age, 8.0 years; range, 0.4–21.5) with idiopathic/hereditary PAH (n=25) or associated congenital heart (n=18) were enrolled a prospective single-center observational study. Anatomic right ventricular-functional...
<h3>Objective</h3> In paediatric pulmonary arterial hypertension (PAH), the effectiveness of add-on combination PAH-therapy has not yet been systematically studied. The purpose this study was to determine effect sildenafil therapy in PAH patients treated with bosentan. <h3>Methods</h3> observational within a national patient cohort, follow-up data 24 consecutive initially bosentan monotherapy and prospectively followed at Dutch referral centre for 2007–2013, were reviewed. Patients received...
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...
Pulmonary arterial hypertension (PAH) is a fatal disease characterized by increased pulmonary pressure, inflammation, and neointimal remodeling of arterioles. Serum levels interleukin (IL)-1β IL-18 are elevated in PAH patients may enhance proinflammatory remodeling. NLRP3 inflammasome activation induces cleavage the cytokines IL-1β IL-18, required for their secretion. Pirfenidone (PFD), an antifibrotic anti-inflammatory drug, has been suggested to inhibit activation. We hypothesized that PFD...
Pediatric pulmonary hypertension (PH) is a severe incurable disease with poor prognosis. In pediatric PH, trial design hampered by the absence of age-appropriate endpoints. This study evaluated physical activity (PA) measured hip-anchored accelerometry as potential endpoint in PH. Is PA-accelerometry associated severity, and based on this association, what minimal important differences (MIDs) correspond to meaningful changes severity PH? Accelerometer outputs from 54 children hemodynamically...
Background and study aim Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease with remodelling leading to an increased resistance (PVRi) decreased compliance (PACi). It debated whether PACi provides prognostic information additional PVRi the relationship between PACi, expressed as their product resistance-compliance-time (RC-time), constant. The of this investigate determine value in addition newly diagnosed children idiopathic or heritable (IPAH/HPAH). Methods...
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in PAH associated CHD (PAH‐CHD), assess the applicability Nice classification, explore whether this classification accurately reflects patient/disease characteristics survival. All children from contemporary cohort consecutive patients followed three major referral centers (Denver, New York, Netherlands) were...
Prognosis in children with pulmonary vascular disease (PVD) is closely linked to right ventricular (RV) failure due increased RV-afterload. Pulmonary arterial (PA) stiffening known occur early the course of PVD and constitutes a main component This study aimed evaluate clinical value PA-stiffness by determining its association advanced hypertension (PAH) mortality at long-term follow-up.Forty-one various stages PVD, defined as mean PA-pressure ≥20 mmHg and/or pulmonary-to-systemic flow-ratio...
Rationale: There are currently no data supporting specific dosing and weaning strategies for parenteral prostanoid therapy in children with pulmonary arterial hypertension (PAH). Objectives: To describe the clinical practice of intravenous (IV) or subcutaneous (SC) pediatric PAH identify associated favorable outcome. Methods: From an international multicenter cohort 275 PAH, 98 patients who received IV/SC were retrospectively analyzed. Results: prostanoids given as monotherapy (20%) combined...
Adverse ventricle-ventricle interaction and resultant left ventricular (LV) dysfunction are a recognized pathophysiological component of disease progression in pulmonary arterial hypertension (PAH) can be associated with electrical mechanical dyssynchrony. The purpose this study was to investigate the clinical mechanistic implications LV electromechanical dyssynchrony children PAH by using novel systolic stretch diastolic relaxation discoordination indexes derived noninvasively from cardiac...