Shahin Moledina
A5022681668- Pulmonary Hypertension Research and Treatments
- Congenital Heart Disease Studies
- Cardiovascular Issues in Pregnancy
- Cardiovascular Function and Risk Factors
- Heart Failure Treatment and Management
- Medical Imaging and Pathology Studies
- Liver Disease Diagnosis and Treatment
- Liver Disease and Transplantation
- Genomics and Rare Diseases
- Transplantation: Methods and Outcomes
- Renin-Angiotensin System Studies
- Cardiac Arrhythmias and Treatments
- Neonatal Respiratory Health Research
- Congenital Diaphragmatic Hernia Studies
- Vascular Anomalies and Treatments
- PI3K/AKT/mTOR signaling in cancer
- Cardiovascular and Diving-Related Complications
- Telomeres, Telomerase, and Senescence
- Genetics and Neurodevelopmental Disorders
- Cystic Fibrosis Research Advances
- Retinal Development and Disorders
- RNA modifications and cancer
- Genetic factors in colorectal cancer
- Immunodeficiency and Autoimmune Disorders
- Phosphodiesterase function and regulation
Great Ormond Street Hospital
2015-2024
University College London
2015-2024
Great Ormond Street Hospital for Children NHS Foundation Trust
2013-2023
National Health Service
2022
Imperial College London
2021
Medical Research Council
2019
National Institute for Health Research
2019
British Heart Foundation
2016-2019
University of Cambridge
2019
Level (Czechia)
2019
Background— Chronic thromboembolic pulmonary hypertension results from incomplete resolution of emboli. Pulmonary endarterectomy (PEA) is potentially curative, but residual following surgery common and its impact on long-term outcome poorly understood. We wanted to identify factors correlated with poor after specifically define clinically relevant post-PEA. Methods Results— Eight hundred eighty consecutive patients (mean age, 57 years) underwent PEA for chronic hypertension. Patients...
Pulmonary arterial hypertension (PAH) is a rare disorder with poor prognosis. Deleterious variation within components of the transforming growth factor-β pathway, particularly bone morphogenetic protein type 2 receptor (BMPR2), underlies most heritable forms PAH. To identify missing heritability we perform whole-genome sequencing in 1038 PAH index cases and 6385 PAH-negative control subjects. Case-control analyses reveal significant overrepresentation variants ATP13A3, AQP1 SOX17, provide...
Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart the most common cardiovascular condition this group, present up to 50% people syndrome contributing poor outcomes. Additional factors outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, metabolic diseases; risk for atherosclerotic disease. Moreover, disparities care compared general population, which vary across different geographies health systems,...
<h3>Objective</h3> To clarify the clinical characteristics and epidemiology of idiopathic pulmonary arterial hypertension (IPAH) in childhood, a rare condition with bad prognosis, poorly documented children. Also, to describe long-term outcome. <h3>Design</h3> A retrospective study 7 years9 experience. <h3>Setting</h3> UK Service for Pulmonary Hypertension Children based at tertiary referral centre. <h3>Patients</h3> 64 <h3>Interventions</h3> Patients were initially treated prostanoids...
Background: Pulmonary arterial hypertension (PAH) is a rare disease with an emerging genetic basis. Heterozygous mutations in the gene encoding bone morphogenetic protein receptor type 2 ( BMPR2 ) are commonest cause of PAH, whereas biallelic eukaryotic translation initiation factor alpha kinase 4 EIF2AK4 described pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis. Here, we determine frequency these and define genotype-phenotype characteristics large cohort patients...
Pulmonary hypertension (PH) is highly heterogeneous and despite treatment advances it remains a life‐shortening condition. There have been significant in imaging technologies, but evidence of their potential clinical utility, practice variable, dependent part on availability expertise. This statement summarizes current emerging modalities role the diagnosis assessment suspected PH. It also includes review commonly encountered radiological scenarios, modeling‐based biomarkers. An expert panel...
There are very few validated prognostic markers in pediatric pulmonary hypertension. Cardiac MRI is a useful, noninvasive method for determining prognosis adults. The present study the first to assess its value children. A total of 100 children with hypertension (median, 10.4 years; range, 0.5-17.6 years) were evaluated (idiopathic, n=60; repaired congenital heart disease, n=22; miscellaneous, n=18). In all patients, ventricular volumes and great vessel flow measured. Volumetric data...
Rationale: Recently, rare heterozygous mutations in GDF2 were identified patients with pulmonary arterial hypertension (PAH). encodes the circulating BMP (bone morphogenetic protein) type 9, which is a ligand for BMP2 receptor.Objectives: Here we determined functional impact of and characterized plasma BMP9 BMP10 levels idiopathic PAH.Methods: Missense mutant proteins expressed vitro on protein processing secretion, endothelial signaling, activity was assessed. Plasma assayed PAH variants...
<h3></h3> The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific such as arterial hypertension (PAH)-congenital heart (PH) associated with bronchopulmonary dysplasia, persistent PH the newborn, related cardiac dysfunction. <h3>Methods</h3> writing group members...
Abstract Background Pulmonary arterial hypertension (PAH) is a lethal vasculopathy characterized by pathogenic remodeling of pulmonary arterioles leading to increased pressures, right ventricular hypertrophy, and heart failure. PAH can be associated with other diseases (APAH: connective tissue diseases, congenital disease, others) but often the etiology idiopathic (IPAH). Mutations in bone morphogenetic protein receptor 2 ( BMPR2 ) are cause most heritable cases vast majority genetically...
Pulmonary wave reflections are a potential hemodynamic biomarker for pulmonary hypertension (PH) and can be analyzed using intensity analysis (WIA). In this study we used vessel area flow obtained cardiac magnetic resonance (CMR) to implement WIA noninvasively. We hypothesized that method could detect differences in PH patients compared with healthy controls also differentiate certain subtypes. Twenty (35% CTEPH 75% female) 10 (60% were recruited. Right left artery (LPA RPA) curves acquired...
Rationale: Pulmonary arterial hypertension (PAH) is characterized by structural remodeling of pulmonary arteries and arterioles. Underlying biological processes are likely reflected in a perturbation circulating proteins. Objectives: To quantify analyze the plasma proteome patients with PAH using inherited genetic variation to inform on underlying molecular drivers. Methods: An aptamer-based assay was used measure proteins 357 idiopathic or heritable PAH, 103 healthy volunteers, 23 relatives...
The aim of the present study was to evaluate a 5-yr experience bosentan in children with pulmonary arterial hypertension (PAH). A retrospective, observational made UK Pulmonary Hypertension Service for Children (Great Ormond Street Hospital Children, London, UK) who were given as monotherapy or combination, from February 2002 May 2008 and followed up ≥ 6 months. Detailed studies 101 idiopathic PAH (IPAH) (n = 42) associated congenital heart disease 59). Before treatment, World Health...
<h3>Objectives</h3> To develop a non-invasive method of assessing disease severity in pulmonary hypertension by quantifying the overall degree vascular pruning using fractal geometry. <h3>Design</h3> A retrospective analysis ECG-gated CT angiograms. <h3>Setting</h3> single national referral centre for investigation and treatment children with hypertension. <h3>Patients</h3> Consecutive angiograms young adults (mean age 10.3 years, range 0.7–19.1) arterial assessed between January 2007 April...
Standardization of the diagnostic routine for children with congenital heart disease associated pulmonary arterial hypertension (PAH‐CHD) is crucial, in particular since inappropriate assignment to repair cardiac lesions (e.g., surgical patients elevated vascular resistance) may be detrimental and poor outcomes. Thus, members Congenital Heart Disease Pediatric Task Forces Pulmonary Vascular Research Institute decided conduct a survey aimed at collecting expert opinion from different...
Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, efficacy. Data obtained at cardiac continue to play an role determining surgical options for children with congenital heart clinical evidence increased resistance. The Pediatric Congenital Heart Disease Task Forces Pulmonary Vascular Research...
Interstitial lung disease (ILD) in infants represents a rare and heterogenous group of disorders, distinct from those occurring adults. In recent years new entity within this category is being recognized, namely filamin A (FLNA) mutation related disease. Our aims are to describe the clinical radiological course patients with aid clinicians prognostic counseling management similar they may encounter.A retrospective case note review was conducted all treated at our institution (a specialist...
Childhood pulmonary hypertension (PH) is a heterogenous disease associated with considerable morbidity and mortality. Invasive assessment of haemodynamics crucial for accurate diagnosis guidance medical therapy. However, adequate imaging increasingly important in children PH to evaluate the right heart vasculature. Cardiac MR (CMR) computed tomography (CT) represent non-invasive modalities that may enable comprehensive ventricular (RV) function haemodynamics. Here, we present graded...
While traffic and air pollution exposure is associated with increased mortality in numerous diseases, its association disease severity outcomes pulmonary arterial hypertension (PAH) remains unknown. Exposure to particulate matter a 50% cut-off aerodynamic diameter ≤2.5 μm (PM 2.5 ), nitrogen dioxide (NO 2 ) indirect measures of traffic-related (distance main road length roads within buffer zones surrounding residential addresses) were estimated for 301 patients idiopathic/heritable PAH...
Abstract Idiopathic pulmonary arterial hypertension (IPAH) is a rare but fatal disease diagnosed by right heart catheterisation and the exclusion of other forms hypertension, producing heterogeneous population with varied treatment response. Here we show unsupervised machine learning identification three major patient subgroups that account for 92% cohort, each unique whole blood transcriptomic clinical feature signatures. These are associated poor, moderate, good prognosis. The poor...
Background— This study assesses the relationship between septal curvature and mean pulmonary artery pressure indexed vascular resistance in children with hypertension. We hypothesized that could be used to estimate right ventricular afterload track acute changes hemodynamics. Methods Results— Fifty patients a median age of 6.7 years (range, 0.45–16.5 years) underwent combined cardiac catheterization cardiovascular magnetic resonance. The majority had idiopathic arterial hypertension (n=30);...