Keywords: ERA; endothelin receptor antagonist; IPAH; idiopathic pulmonary arterial hypertension; PAH; pulmonary arterial hypertension; PDE; phosphodiesterase; SC; subcutaneous; 6MWT; 6-minute walk test; Subcutaneous prostanoid; Pediatric treatment; Pulmonary hypert
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Keywords: Iron deficiency; Pulmonary hypertension; Connective tissue disease; High sensitive C reactive protein; PH; Pulmonary hypertension; PVR; pulmonary vascular resistance; PAH; pulmonary arterial hypertension; CTEPH; chronic thromboembolic pulmonary hypertensi
Keywords: biomarkers; catheterizations; pulmonary arterial hypertension; 6MWD; 6-min walk distance; BNP; brain natriuretic peptide; CHD; congenital heart disease; CTD; connective tissue disease; ESC/ERS; European Society of Cardiology/European Respiratory Society;
Keywords: AF; atrial fibrillation; BWH; Brigham and Women's Hospital; DOACs; direct oral anticoagulants; HULP; Hospital Universitario La Paz; INR; international normalized ratio; IPAH; idiopathic pulmonary arterial hypertension; IQR; interquartilic range; mPAP; mea
Keywords: congenital heart disease; developmental lung disease; pulmonary vascular disease; right heart failure; risk factors; treatment algorithm; CHD; congenital heart disease; ES; Eisenmenger syndrome; HPAH; heritable pulmonary arterial hypertension; IPAH; idiop
Keywords: connective tissue disease; idiopathic; pulmonary hypertension; systemic sclerosis; C; compliance; CMR; cardiac magnetic resonance; Dlco; diffusing capacity for carbon monoxide; IPAH; idiopathic pulmonary arterial hypertension; IPAH:exLHD; excluded from pr
Keywords: Intravascular ultrasound; Pulmonary arterial hypertension; Pulmonary vascular properties; Connective tissue diseases; Inflammation factors; CHD; Congenital heart disease; CO; Cardiac output; CTD; Connective tissue disease; DPAP; Diastolic pulmonary artery
Keywords: heart failure with preserved ejection fraction; idiopathic pulmonary arterial hypertension; ERA; endothelin receptor antagonist; HFpEF; heart failure with preserved ejection fraction; IPAH; idiopathic pulmonary arterial hypertension; PAPm; mean pulmonary
Keywords: heart failure; hemodynamics; pulmonary circulation; pulmonary hypertension; 6-MWD; 6-min walk distance; AUC; area under the curve; CO; cardiac output; CPA; pulmonary arterial compliance; DPG; diastolic pulmonary vascular pressure gradient; iPAH; idiopathi
Keywords: Ang1/2; angiopoietin 1 or 2; BMPR2; Bone morphogenetic protein receptor type 2; CAV-1/2; caveolin-1/2; CHD; congenital heart disease; EC; endothelial cell; ECFC; endothelial colony forming cells; EGR-1; early growth response protein 1; EndoMT; endothelial
Keywords: asthma; COPD; pulmonary fibrosis; pulmonary hypertension; ADDS; antibody-dependent degranulatory synapse; ASM; airway smooth muscle; β2-AR; β2-adrenergic receptor; Ca2+; calcium ion; CADM1; cell adhesion molecule 1; CD; cluster of differentiation; CPA3;
Keywords: ARDS; acute respiratory distress syndrome; AHR; airway hyperresponsiveness; ASM; airway smooth muscle; BAL; bronchoalveolar lavage; CPA; carboxypeptidase A; CADM1; cell adhesion molecule 1; COPD; chronic obstructive pulmonary disease; NADPH; dihydronicoti
Keywords: IPAH; Survival; 6MWD; Depression
Keywords: CTD; connective tissue disease; FC; functional class; IPAH; idiopathic pulmonary arterial hypertension; mPAP; mean pulmonary arterial pressure; PAH; pulmonary arterial hypertension; PVR; pulmonary vascular resistance; REVEAL Registry; Registry to Evaluate
Keywords: PH; pulmonary hypertension; PAH; pulmonary arterial hypertension; IPAH; Idiopathic pulmonary arterial Hypertension; CHD; congenital heart disease; PVR; pulmonary vascular remodeling; ANP; atrial natriuretic peptide; BNP; brain natriuretic peptide; RDW; re
Keywords: Pulmonary hypertension; Tumor necrosis factor-related apoptosis-inducing ligand; Mouse model; 6MWD; 6-min walk distance; CAD; coronary artery disease; CHD; congenital heart disease; CTD; connective tissue disease; CTEPH; chronic thromboembolic pulmonary
Keywords: 6MWD; 6-min walk distance; BSI; blood stream infection; CRI; chronic renal insufficiency; CTD; connective tissue disease; IPAH; idiopathic pulmonary arterial hypertension; NYHA FC; New York Heart Association functional class; PAH; pulmonary arterial hyper
Keywords: 6MWD; 6-min walk distance; APAH-CHD; pulmonary arterial hypertension associated with congenital heart disease; APAH-non-CHD; pulmonary arterial hypertension associated with conditions other than congenital heart disease; CW; clinical worsening; HPAH; heri
Keywords: Autonomic dysfunction; Heart rate variability; Idiopathic pulmonary arterial hypertension; Pulmonary arterial pressure; Ventricular arrhythmia6MWT, 6-minute walking test; ANS, autonomic nervous system; HF, high frequency; HRV, heart rate variability; IPAH
Keywords: 6MWD; 6-min walk distance; CMR; cardiac MRI; CO; cardiac output; GFR; glomerular filtration rate; IPAH; idiopathic pulmonary arterial hypertension; IQR; interquartile range; NT-proBNP; N-terminal pro-brain natriuretic peptide; PAH; pulmonary arterial hype
Keywords: Echocardiography; Idiopathic pulmonary hypertension; Strain echocardiography; Right ventricular performance; BSA; Body surface area; iPAH; Idiopathic pulmonary arterial hypertension; PAH; Pulmonary arterial hypertension; PVR; Pulmonary vascular resistance
Keywords: BMPR2; bone morphogenetic protein receptor type II; CFH; cell-free hemoglobin; CTD-PAH; connective tissue disease-associated pulmonary arterial hypertension; HPAH; heritable pulmonary arterial hypertension; IPAH; idiopathic pulmonary arterial hypertension
Keywords: HFpEF; heart failure with preserved ejection fraction; IPAH; idiopathic pulmonary arterial hypertension; mPAP; mean pulmonary artery pressure; PAH; pulmonary arterial hypertension; PAOP; pulmonary artery occlusion pressure; PH; pulmonary hypertension; WHO
Keywords: pediatrics; pulmonary hypertension; survival; APAH; associated pulmonary arterial hypertension; BNP; brain natriuretic peptide; CCB; calcium channel blocker; CHD; congenital heart disease; HPAH; hereditary pulmonary arterial hypertension; IPAH; idiopathic
Keywords: 6MWD; 6-min walk distance; AHRQ; Agency for Healthcare Research and Quality; ARIES; Ambrisentan in Pulmonary Arterial Hypertension, Randomized Double-Blind, Placebo-Controlled, Multicenter, Efficacy Study; BNP; brain natriuretic peptide; CB; consensus-bas
5-HTT, BMPR2, EDN1, ENG, KCNA5 gene polymorphisms and susceptibility to pulmonary arterial hypertension: A meta-analysis
Keywords: ORs; odds ratios; CI; confidence interval; PAH; pulmonary arterial hypertension; IPAH; idiopathic pulmonary arterial hypertension; HPAH; Hypoxic Pulmonary Artery Hypertension; COPD; chronic obstructive pulmonary disease; BMPR2; bone morphogenetic protein
Functional analysis of a novel ENG variant in a patient with hereditary hemorrhagic telangiectasia (HHT) identifies a new Sp1 binding-site
Keywords: Ab; antibody; ACTB; β-actin; ACVRL1; Activin A Receptor type II-Like Kinase 1; AVM; ArterioVenous Malformation; BMP; Bone Morphogenetic Protein; BMPR2; Bone Morphogenetic Protein Receptor Type 2; CAVM; Cerebral ArterioVenous Malformation; CTRL; control;
Long noncoding RNA LISPR1 is required for S1P signaling and endothelial cell function
Keywords: COPD; Chronic obstructive pulmonary disease; CTEPH; Chronic thromboembolic pulmonary hypertension; CRISPR/Cas9; Clustered Regularly Interspaced Short Palindromic Repeats/CRISPR associated protein 9; HUVEC; Human umbilical vein endothelial cells; IPAH; Idi
T-type Ca2 + channels elicit pro-proliferative and anti-apoptotic responses through impaired PP2A/Akt1 signaling in PASMCs from patients with pulmonary arterial hypertension
Keywords: CDK4/6; cyclin-dependent kinase 4 and 6; CTL; control; CycD1; cyclin D1; FoxO3A; forkhead transcription factor; ICaT; T-type Ca2Â + current; OA; okadaïc acid; PAH; pulmonary arterial hypertension; iPAH; idiopathic PAH; PASMC; pulmonary arterial smooth ce
EIF2AK4 Mutations in Patients Diagnosed With Pulmonary Arterial Hypertension
Keywords: genetics; pulmonary capillary hemangiomatosis; pulmonary hypertension; pulmonary venoocclusive disease; BMPR2; bone morphogenetic protein receptor type II; EIF2AK4; eukaryotic translation initiation factor 2 alpha kinase 4; HPAH; heritable pulmonary arte
CTA-derived left to right atrial size ratio distinguishes between pulmonary hypertension due to heart failure and idiopathic pulmonary arterial hypertension
Keywords: CMR; cardiac magnetic resonance imaging; CT-(A); computed tomography - (angiography); HFpEF; heart failure with preserved ejection fraction; IPAH; idiopathic pulmonary arterial hypertension; LA; left atrium; (m)PAP; mean pulmonary artery pressure; MPR; mu
Five-Year Outcomes of Patients Enrolled in the REVEAL Registry
Keywords: 6MWD; 6-min walk distance; APAH-CHD; pulmonary arterial hypertension associated with congenital heart disease including both repaired and unrepaired; FC; functional class; FPAH; familial pulmonary arterial hypertension; IPAH; idiopathic pulmonary arterial
Plasma 15-F2t-isoprostane in idiopathic pulmonary arterial hypertension
Keywords: BMI; body mass index; BNP; brain natriuretic peptide; CI; cardiac index; 95% CI; 95% confidence interval; IPAH; idiopathic pulmonary arterial hypertension; 15-F2t-IsoP; 15-F2t-isoprostane; 6MWD; 6-minute walk distance; PAP; mean pulmonary arterial pressur
Pharmacoperones and the calcium sensing receptor: Exogenous and endogenous regulators
Keywords: ADH; autosomal dominant hypocalcemia; CaSR; calcium sensing receptor; xCT; cystine-glutamate transporter; DHPs; dihydropyridines; EAAT; excitatory amino acid transporter; EGFR; endothelial growth factor receptor; ER; endoplasmic reticulum; ERK1/2; extrace
Pulmonary Artery Denervation to Treat Pulmonary Arterial Hypertension: The Single-Center, Prospective, First-in-Man PADN-1 Study (First-in-Man Pulmonary Artery Denervation for Treatment of Pulmonary Artery Hypertension)
Keywords: 6-min walk test; denervation; pulmonary artery hypertension; pulmonary artery pressure; 6MWT; 6-min walk test; CO; cardiac output; IPAH; idiopathic pulmonary arterial hypertension; MPA; main pulmonary artery; NT-BNP; N-terminal brain natriuretic peptide;
Real-time PCR using SYBR Green for the detection of Shigella spp. in food and stool samples
Keywords: Shigella detection; Stool; Food; ipaH; SYBR Green; Real-time PCR
Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: A 14-year single-center experience
Keywords: 12; CHD; congenital heart disease; CT; computed tomography; CTD; connective tissue disease; CTEPH; chronic thromboembolic pulmonary hypertension; ECLS; extracorporeal life support; GERD; gastroesophageal reflux disease; iPAH; idiopathic pulmonary arterial
Comparison of Body Habitus in Patients With Pulmonary Arterial Hypertension Enrolled in the Registry to Evaluate Early and Long-term PAH Disease Management With Normative Values From the National Health and Nutrition Examination Survey
Keywords: APAH; associated PAH; BMI; body mass index; CHD-APAH; congenital heart disease-APAH; CTD-APAH; connective tissue disease-APAH; FPAH; familial PAH; HIV-APAH; human immunodeficiency virus infection-APAH; IPAH; idiopathic PAH; LVEDP; left ventricular e
Key role of the RhoA/Rho kinase system in pulmonary hypertension
Keywords: Pulmonary hypertension; Pulmonary arterial hypertension; Rho kinase; Fasudil; Monocrotaline; Chronic hypoxia; BMPR-II; bone morphogenetic receptor type II; CH; chronic hypoxia; EC; endothelial cell; hPAH; heritable pulmonary arterial hypertension; HPV; hy
Surveillance of shigellosis by real-time PCR suggests underestimation of shigellosis prevalence by culture-based methods in a population of rural China
Keywords: Disease burden; Shigella; Real-time PCR; ipaH; Culture
Elevated platelet angiostatin and circulating endothelial microfragments in idiopathic pulmonary arterial hypertension: A preliminary study
Keywords: EMF; endothelial microfragment; IPAH; idiopathic pulmonary arterial hypertension; NO; nitric oxide; PGI2; prostacyclin; TXA2; thromboxane; VEGF; vascular endothelial growth factor; Platelets; Angiostatin; Endothelial microfragments; Idiopathic pulmonary a
Current Diagnostic and Prognostic Assessment of Pulmonary Hypertension
Keywords: Pulmonary arterial hypertension; Epidemiology; Prognosis; Referral centre; Hipertensión arterial pulmonar; EpidemiologÃa; Pronóstico; Unidad de referencia; CTEPH; chronic thromboembolic pulmonary hypertension; HIV; human immunodeficiency virus; IPAH; i
Pulmonary Hypertension: Diagnosis and Management
Keywords: 5-HTT; 5-hydroxytryptamine transporter; BMPR2; bone morphogenetic protein receptor, type II; FDA; Food and Drug Administration; IPAH; idiopathic pulmonary arterial hypertension; mPAP; mean pulmonary arterial pressure; PAH; pulmonary arterial hypertension;
Sitaxsentan for the Treatment of Pulmonary Arterial Hypertension
Keywords: bosentan; endothelin antagonists; pulmonary arterial hypertension; sitaxsentan; ALT; alanine aminotransferase; AST; aspartate aminotransferase; CI; confidence interval; ET; endothelin; ETRA; endothelin-receptor antagonist; IPAH; idiopathic pulmonary arter
Design of the REVEAL Registry for US Patients With Pulmonary Arterial Hypertension
Keywords: IPAH; idiopathic pulmonary arterial hypertension; NHLBI; National Heart, Lung, and Blood Institute; PAH; pulmonary arterial hypertension; PCWP; pulmonary capillary wedge pressure; PH; pulmonary hypertension; REVEAL; Registry to Evaluate Early and Long-ter
The Current Treatment of Pulmonary Arterial Hypertension
Keywords: bosentan; clinical trials; prostacyclin; sildenafil; IPAH; idiopathic pulmonary arterial hypertension; PAH; pulmonary arterial hypertension;
Perfusion lung scan as a prognostic indicator of response to beraprost sodium in idiopathic pulmonary arterial hypertension
Keywords: Idiopathic pulmonary arterial hypertension; Perfusion lung scan; Beraprost sodium; IPAH; idiopathic pulmonary arterial hypertension; NYHA; New York Heart Association; Vmax; peak velocity through tricuspid valve; MPAP; mean pulmonary arterial pressure; PVR
Enhanced Hypoxic Pulmonary Vasoconstriction in Families of Adults or Children With Idiopathic Pulmonary Arterial Hypertension
Keywords: BMPR2; bone morphogenetic protein receptor II; IPAH; idiopathic pulmonary arterial hypertension; PASP; pulmonary artery systolic pressure; RH; risk haplotype;
Ambrisentan Therapy for Pulmonary Arterial Hypertension
Keywords: 6MWD; 6-min walk distance; ERA; endothelin receptor antagonist; ET; endothelin; IPAH; idiopathic pulmonary arterial hypertension; mPAP; mean pulmonary arterial pressure; PAH; pulmonary arterial hypertension; PCWP; pulmonary capillary wedge pressure; PVR;
Effects of Long-Term Bosentan in Children With Pulmonary Arterial Hypertension
Keywords: b.i.d.; twice daily; CHD; congenital heart disease; CI; cardiac index; IPAH; idiopathic pulmonary arterial hypertension; PAH; pulmonary arterial hypertension; PAPm; mean pulmonary artery pressure; PVRI; pulmonary vascular resistance index; RAPm; mean righ