Published in N Engl J Med on September 30, 2004
Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest (2005) 4.74
Genomic analyses identify distinct patterns of selection in domesticated pigs and Tibetan wild boars. Nat Genet (2013) 3.41
Association of the metabolic syndrome with pulmonary venous hypertension. Chest (2009) 2.75
Modern age pathology of pulmonary arterial hypertension. Am J Respir Crit Care Med (2012) 2.68
A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J (2009) 2.63
Role of HIV and human herpesvirus-8 infection in pulmonary arterial hypertension. AIDS (2008) 2.12
Noninvasive assessment of murine pulmonary arterial pressure: validation and application to models of pulmonary hypertension. Circ Cardiovasc Imaging (2009) 2.05
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Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome. J Clin Invest (2008) 1.86
Plasma levels of high-density lipoprotein cholesterol and outcomes in pulmonary arterial hypertension. Am J Respir Crit Care Med (2010) 1.85
Anti-inflammatory and antiatherogenic role of BMP receptor II in endothelial cells. Arterioscler Thromb Vasc Biol (2013) 1.78
Bone morphogenetic protein 2 induces pulmonary angiogenesis via Wnt-beta-catenin and Wnt-RhoA-Rac1 pathways. J Cell Biol (2009) 1.77
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Targeting energetic metabolism: a new frontier in the pathogenesis and treatment of pulmonary hypertension. Am J Respir Crit Care Med (2011) 1.41
Pathobiology of pulmonary arterial hypertension and right ventricular failure. Eur Respir J (2012) 1.39
Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol (2008) 1.38
Reversible or irreversible remodeling in pulmonary arterial hypertension. Am J Respir Cell Mol Biol (2009) 1.33
Long term imatinib treatment in pulmonary arterial hypertension. Thorax (2006) 1.32
PDGF signaling in pulmonary arterial hypertension. J Clin Invest (2005) 1.32
Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax (2005) 1.29
Hypoxia-induced mitogenic factor (HIMF/FIZZ1/RELMalpha) induces the vascular and hemodynamic changes of pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol (2009) 1.28
Truncating and missense BMPR2 mutations differentially affect the severity of heritable pulmonary arterial hypertension. Respir Res (2009) 1.24
Hypermethylation of PTGER2 confers prostaglandin E2 resistance in fibrotic fibroblasts from humans and mice. Am J Pathol (2010) 1.23
Long-term effects of epoprostenol on the pulmonary vasculature in idiopathic pulmonary arterial hypertension. Chest (2010) 1.22
Two-pore potassium channels in the cardiovascular system. Eur Biophys J (2008) 1.21
Multidrug resistance-associated protein 4 regulates cAMP-dependent signaling pathways and controls human and rat SMC proliferation. J Clin Invest (2008) 1.19
Can we improve outcome of congenital diaphragmatic hernia? Pediatr Surg Int (2009) 1.19
Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res (2010) 1.19
Pulmonary arterial hypertension. Orphanet J Rare Dis (2013) 1.18
Pulmonary vascular and right ventricular dysfunction in adult critical care: current and emerging options for management: a systematic literature review. Crit Care (2010) 1.18
Lower socioeconomic status is associated with worse outcomes in pulmonary arterial hypertension. Am J Respir Crit Care Med (2012) 1.17
Rapamycin attenuates hypoxia-induced pulmonary vascular remodeling and right ventricular hypertrophy in mice. Respir Res (2007) 1.15
Late gadolinium enhancement cardiovascular magnetic resonance predicts clinical worsening in patients with pulmonary hypertension. J Cardiovasc Magn Reson (2012) 1.14
Carbon monoxide reverses established pulmonary hypertension. J Exp Med (2006) 1.14
Effect of sildenafil on ventilatory efficiency and exercise tolerance in pulmonary hypertension. Eur J Heart Fail (2007) 1.14
Inhibition of MRP4 prevents and reverses pulmonary hypertension in mice. J Clin Invest (2011) 1.13
Inhibition of endothelin receptors in the treatment of pulmonary arterial hypertension: does selectivity matter? Eur Heart J (2008) 1.10
Review of bosentan in the management of pulmonary arterial hypertension. Vasc Health Risk Manag (2007) 1.09
Sildenafil attenuates pulmonary inflammation and fibrin deposition, mortality and right ventricular hypertrophy in neonatal hyperoxic lung injury. Respir Res (2009) 1.09
Strategic plan for lung vascular research: An NHLBI-ORDR Workshop Report. Am J Respir Crit Care Med (2010) 1.09
Common variant near the endothelin receptor type A (EDNRA) gene is associated with intracranial aneurysm risk. Proc Natl Acad Sci U S A (2011) 1.08
The adult patient with eisenmenger syndrome: a medical update after dana point part I: epidemiology, clinical aspects and diagnostic options. Curr Cardiol Rev (2010) 1.06
Novel computed tomographic chest metrics to detect pulmonary hypertension. BMC Med Imaging (2011) 1.05
Immune and inflammatory mechanisms in pulmonary arterial hypertension. Prog Cardiovasc Dis (2012) 1.03
Protective role of the antidiabetic drug metformin against chronic experimental pulmonary hypertension. Br J Pharmacol (2009) 1.01
Inhibition of SOC/Ca2+/NFAT pathway is involved in the anti-proliferative effect of sildenafil on pulmonary artery smooth muscle cells. Respir Res (2009) 1.00
Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br J Clin Pharmacol (2009) 0.98
The crossroads of iron with hypoxia and cellular metabolism. Implications in the pathobiology of pulmonary hypertension. Am J Respir Cell Mol Biol (2014) 0.98
Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry. J Cardiovasc Magn Reson (2012) 0.98
Inhibition of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) reverses experimental pulmonary hypertension. J Exp Med (2012) 0.97
The 'sweet' and 'bitter' involvement of glycosaminoglycans in lung diseases: pharmacotherapeutic relevance. Br J Pharmacol (2009) 0.96
RhoA/Rho-kinase signaling: a therapeutic target in pulmonary hypertension. Vasc Health Risk Manag (2009) 0.96
Effect of chronic hypoxia on voltage-independent calcium influx activated by 5-HT in rat intrapulmonary arteries. Pflugers Arch (2006) 0.96
Enhanced store-operated Ca²+ entry and TRPC channel expression in pulmonary arteries of monocrotaline-induced pulmonary hypertensive rats. Am J Physiol Cell Physiol (2011) 0.95
Rapid transition from inhaled iloprost to inhaled treprostinil in patients with pulmonary arterial hypertension. Cardiovasc Ther (2013) 0.94
Design and implementation of the START (STem cells for ARDS Treatment) trial, a phase 1/2 trial of human mesenchymal stem/stromal cells for the treatment of moderate-severe acute respiratory distress syndrome. Ann Intensive Care (2014) 0.93
Phosphodiesterase 4 inhibition attenuates persistent heart and lung injury by neonatal hyperoxia in rats. Am J Physiol Lung Cell Mol Physiol (2011) 0.93
Loss of microRNA-17∼92 in smooth muscle cells attenuates experimental pulmonary hypertension via induction of PDZ and LIM domain 5. Am J Respir Crit Care Med (2015) 0.93
Sildenafil for the treatment of pulmonary hypertension in pediatric patients. Pediatr Cardiol (2009) 0.92
Pathophysiology of pulmonary hypertension in acute lung injury. Am J Physiol Lung Cell Mol Physiol (2012) 0.92
Cyclooxygenase-2 inhibition and hypoxia-induced pulmonary hypertension: effects on pulmonary vascular remodeling and contractility. Trends Cardiovasc Med (2009) 0.92
Multi tyrosine kinase inhibitor dasatinib as novel cause of severe pre-capillary pulmonary hypertension? BMC Pulm Med (2011) 0.92
Pulmonary hypertension--a new manifestation of mitochondrial disease. J Inherit Metab Dis (2005) 0.91
Type 5 phosphodiesterase expression is a critical determinant of the endothelial cell angiogenic phenotype. Am J Physiol Lung Cell Mol Physiol (2008) 0.91
Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol (2007) 0.91
Cardiac manifestations in systemic sclerosis. World J Cardiol (2014) 0.91
Increasing cGMP-dependent protein kinase I activity attenuates cisplatin-induced kidney injury through protection of mitochondria function. Am J Physiol Renal Physiol (2013) 0.91
Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int (2013) 0.90
Lung involvement in systemic sclerosis. Presse Med (2010) 0.90
Assessing response to therapy in idiopathic pulmonary arterial hypertension: a consensus survey of Canadian pulmonary hypertension physicians. Can Respir J (2011) 0.90
Nicorandil attenuates monocrotaline-induced vascular endothelial damage and pulmonary arterial hypertension. PLoS One (2012) 0.90
A review of pulmonary arterial hypertension: role of ambrisentan. Vasc Health Risk Manag (2007) 0.90
MicroRNAs in pulmonary arterial hypertension. Am J Respir Cell Mol Biol (2015) 0.90
The molecular targets of approved treatments for pulmonary arterial hypertension. Thorax (2015) 0.90
Low-Dose FK506 (Tacrolimus) in End-Stage Pulmonary Arterial Hypertension. Am J Respir Crit Care Med (2015) 0.89
A refined radio-telemetry technique to monitor right ventricle or pulmonary artery pressures in rats: a useful tool in pulmonary hypertension research. Pflugers Arch (2007) 0.89
Abnormal pulmonary artery stiffness in pulmonary arterial hypertension: in vivo study with intravascular ultrasound. PLoS One (2012) 0.88
Hypoxia-induced mitogenic factor (HIMF/FIZZ1/RELMα) in chronic hypoxia- and antigen-mediated pulmonary vascular remodeling. Respir Res (2013) 0.87
The PPARbeta/delta agonist GW0742 relaxes pulmonary vessels and limits right heart hypertrophy in rats with hypoxia-induced pulmonary hypertension. PLoS One (2010) 0.87
Lysophosphatidic acid signaling protects pulmonary vasculature from hypoxia-induced remodeling. Arterioscler Thromb Vasc Biol (2011) 0.87
Stability and microbiological properties of a new formulation of epoprostenol sodium when reconstituted and diluted. Drug Des Devel Ther (2012) 0.87
Platelet distribution width and mean platelet volume in children with pulmonary arterial hypertension secondary to congenital heart disease with left-to-right shunt: new indices of severity? Pediatr Cardiol (2012) 0.87
Pulmonary hypertension in cardiac surgery. Curr Cardiol Rev (2010) 0.87
Sildenafil treatment attenuates lung and kidney injury due to overproduction of oxidant activity in a rat model of sepsis: a biochemical and histopathological study. Clin Exp Immunol (2011) 0.87
Adenosine monophosphate-activated protein kinase is required for pulmonary artery smooth muscle cell survival and the development of hypoxic pulmonary hypertension. Am J Respir Cell Mol Biol (2013) 0.87
HMGB1 promotes the development of pulmonary arterial hypertension in rats. PLoS One (2014) 0.86
Update on the clinical utility of sildenafil in the treatment of pulmonary arterial hypertension. Drug Des Devel Ther (2010) 0.86
Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin (2012) 0.86
Serotonin transporter interacts with the PDGFβ receptor in PDGF-BB-induced signaling and mitogenesis in pulmonary artery smooth muscle cells. Am J Physiol Lung Cell Mol Physiol (2010) 0.86
VIP and endothelin receptor antagonist: an effective combination against experimental pulmonary arterial hypertension. Respir Res (2011) 0.85
Review of inhaled iloprost for the control of pulmonary artery hypertension in children. Vasc Health Risk Manag (2009) 0.85
Skeletal muscle abnormalities in pulmonary arterial hypertension. PLoS One (2014) 0.85
Targeted treatment in COPD: a multi-system approach for a multi-system disease. Int J Chron Obstruct Pulmon Dis (2009) 0.85
Interleukin-6 as a potential therapeutic target for pulmonary arterial hypertension. Int J Rheumatol (2010) 0.85
Translating Research into Improved Patient Care in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med (2016) 0.84
Treprostinil for pulmonary hypertension. Vasc Health Risk Manag (2008) 0.84
Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med (2005) 9.27
Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med (2006) 6.06
Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation (2010) 5.87
Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med (2013) 4.98
Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol (2002) 4.79
Inhaled iloprost for severe pulmonary hypertension. N Engl J Med (2002) 3.90
Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol (2004) 3.63
Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med (2008) 3.13
Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation (2005) 2.98
Pulmonary arterial hypertension in patients treated by dasatinib. Circulation (2012) 2.87
Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation (2013) 2.64
Chronic thromboembolic pulmonary hypertension. Circulation (2006) 2.58
Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol (2009) 2.57
Dysregulated renin-angiotensin-aldosterone system contributes to pulmonary arterial hypertension. Am J Respir Crit Care Med (2012) 2.52
Platelet-derived growth factor expression and function in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med (2008) 2.48
New formula for predicting mean pulmonary artery pressure using systolic pulmonary artery pressure. Chest (2004) 2.47
Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest (2009) 2.40
Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J (2006) 2.34
Vascular and right ventricular remodelling in chronic thromboembolic pulmonary hypertension. Eur Respir J (2012) 2.32
Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat (2006) 2.30
Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med (2015) 2.23
CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am J Respir Crit Care Med (2002) 2.15
C-kit-positive cells accumulate in remodeled vessels of idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med (2011) 2.12
Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med (2008) 2.09
Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med (2007) 2.03
Proinflammatory cytokine levels are linked to death in pulmonary arterial hypertension. Eur Respir J (2013) 1.92
Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation (2011) 1.92
Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology (2005) 1.88
Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol (2005) 1.82
Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol (2002) 1.77
Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest (2004) 1.72
Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med (2008) 1.71
Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest (2003) 1.71
Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol (2008) 1.69
Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies. Chest (2012) 1.68
End points and clinical trial design in pulmonary arterial hypertension. J Am Coll Cardiol (2009) 1.63
Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med (2015) 1.62
2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Rev Esp Cardiol (Engl Ed) (2016) 1.61
Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant (2007) 1.58
Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg (2012) 1.54
IgG from patients with pulmonary arterial hypertension and/or systemic sclerosis binds to vascular smooth muscle cells and induces cell contraction. Ann Rheum Dis (2011) 1.53
Role of endothelium-derived CC chemokine ligand 2 in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med (2007) 1.52
Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation. Am J Respir Crit Care Med (2010) 1.50
Independent association of urinary F2-isoprostanes with survival in pulmonary arterial hypertension. Chest (2012) 1.48
Characterization of pulmonary arterial hypertension patients walking more than 450 m in 6 min at diagnosis. Chest (2010) 1.48
Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med (2003) 1.46
Pre-implantation genetic diagnosis in pulmonary arterial hypertension due to BMPR2 mutation. Eur Respir J (2012) 1.45
Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore) (2008) 1.43
Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol (2007) 1.42
Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol (2004) 1.41
Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum (2008) 1.40
Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med (2004) 1.40
Left ventricular ejection time in acute heart failure complicating precapillary pulmonary hypertension. Chest (2013) 1.39
Chemokine RANTES in severe pulmonary arterial hypertension. Am J Respir Crit Care Med (2002) 1.38
Sildenafil for pulmonary arterial hypertension: still waiting for evidence. Am J Respir Crit Care Med (2004) 1.38
Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum (2011) 1.35
Diagnostic strategy for patients with suspected pulmonary embolism: a prospective multicentre outcome study. Lancet (2002) 1.32
Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest (2006) 1.29
HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS (2010) 1.27
Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol (2004) 1.22
Pulmonary arterial hypertension: thin-section CT predictors of epoprostenol therapy failure. Radiology (2002) 1.20
Endothelin receptor antagonists in pulmonary arterial hypertension. J Am Coll Cardiol (2004) 1.19
Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res (2010) 1.19
Pulmonary arterial hypertension. Orphanet J Rare Dis (2013) 1.18
Evaluation of various empirical formulas for estimating mean pulmonary artery pressure by using systolic pulmonary artery pressure in adults. Chest (2008) 1.16
Phosphodiesterase type 5 inhibitors in pulmonary arterial hypertension. Adv Ther (2009) 1.10
Autocrine fibroblast growth factor-2 signaling contributes to altered endothelial phenotype in pulmonary hypertension. Am J Respir Cell Mol Biol (2010) 1.08
Future perspectives for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol (2009) 1.08
Treatment of pulmonary arterial hypertension with targeted therapies. Nat Rev Cardiol (2011) 1.08
Automatic quantification of right ventricular function with gated blood pool SPECT. J Nucl Cardiol (2004) 1.08
Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med (2010) 1.06
Current and future management of chronic thromboembolic pulmonary hypertension: from diagnosis to treatment responses. Proc Am Thorac Soc (2006) 1.05
Out-of-proportion pulmonary hypertension and heart failure with preserved ejection fraction. Respiration (2012) 1.03