Published in N Engl J Med on August 02, 2001
Pulmonary Hypertension--Mechanisms and Family Registry | NCT00005357
Cellular and molecular basis of pulmonary arterial hypertension. J Am Coll Cardiol (2009) 3.69
Hereditary haemorrhagic telangiectasia: a clinical and scientific review. Eur J Hum Genet (2009) 2.83
Hereditary haemorrhagic telangiectasia: current views on genetics and mechanisms of disease. J Med Genet (2005) 2.72
Bone morphogenetic protein-9 is a circulating vascular quiescence factor. Circ Res (2008) 2.43
Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol (2009) 2.31
A functional single-nucleotide polymorphism in the TRPC6 gene promoter associated with idiopathic pulmonary arterial hypertension. Circulation (2009) 2.17
Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension. Nat Med (2015) 2.04
Relevant issues in the pathology and pathobiology of pulmonary hypertension. J Am Coll Cardiol (2013) 1.96
Pathogenic mechanisms of pulmonary arterial hypertension. J Mol Cell Cardiol (2007) 1.85
Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J (2003) 1.77
Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia. J Med Genet (2003) 1.76
Bone morphogenetic protein (BMP) and activin type II receptors balance BMP9 signals mediated by activin receptor-like kinase-1 in human pulmonary artery endothelial cells. J Biol Chem (2009) 1.68
Altered immune phenotype in peripheral blood cells of patients with scleroderma-associated pulmonary hypertension. Clin Transl Sci (2010) 1.56
NOX4 mediates hypoxia-induced proliferation of human pulmonary artery smooth muscle cells: the role of autocrine production of transforming growth factor-{beta}1 and insulin-like growth factor binding protein-3. Am J Physiol Lung Cell Mol Physiol (2008) 1.52
Pathobiology of pulmonary arterial hypertension and right ventricular failure. Eur Respir J (2012) 1.39
Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects. Hum Mutat (2015) 1.38
Hereditary hemorrhagic telangiectasia: an update on clinical manifestations and diagnostic measures. Wien Klin Wochenschr (2006) 1.37
PDGF signaling in pulmonary arterial hypertension. J Clin Invest (2005) 1.32
Bone morphogenetic protein receptor type II C-terminus interacts with c-Src: implication for a role in pulmonary arterial hypertension. Am J Respir Cell Mol Biol (2005) 1.28
Identification of genetic polymorphisms associated with risk for pulmonary hypertension in sickle cell disease. Blood (2008) 1.28
Altered MicroRNA processing in heritable pulmonary arterial hypertension: an important role for Smad-8. Am J Respir Crit Care Med (2011) 1.27
Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res (2010) 1.19
Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era. Front Genet (2015) 1.18
Pulmonary arterial hypertension. Orphanet J Rare Dis (2013) 1.18
Pulmonary hypertension: diagnosis and management. Mayo Clin Proc (2009) 1.18
Hereditary hemorrhagic telangiectasia, a vascular dysplasia affecting the TGF-beta signaling pathway. Clin Med Res (2006) 1.17
The genetic basis of pulmonary arterial hypertension. Hum Genet (2014) 1.14
Role of microRNAs in lung development and pulmonary diseases. Pulm Circ (2013) 1.14
Hereditary hemorrhagic telangiectasia: ENG and ALK-1 mutations in Dutch patients. Hum Genet (2004) 1.11
Pulmonary hypertension: therapeutic targets within the serotonin system. Br J Pharmacol (2008) 1.11
Strategic plan for lung vascular research: An NHLBI-ORDR Workshop Report. Am J Respir Crit Care Med (2010) 1.09
Genetics of pulmonary arterial hypertension. Semin Respir Crit Care Med (2009) 1.07
Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med (2007) 1.06
Molecular mechanisms of pulmonary arterial remodeling. Mol Med (2014) 1.05
One hundred years of research in the pathogenesis of pulmonary hypertension. Am J Respir Cell Mol Biol (2005) 1.05
Evidence for cell fusion is absent in vascular lesions associated with pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol (2008) 1.03
Immune and inflammatory mechanisms in pulmonary arterial hypertension. Prog Cardiovasc Dis (2012) 1.03
Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. Thorax (2005) 1.03
Polymorphism in the angiotensin II type 1 receptor (AGTR1) is associated with age at diagnosis in pulmonary arterial hypertension. J Heart Lung Transplant (2009) 1.01
Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations. Respir Res (2011) 1.01
Visceral manifestations in hereditary haemorrhagic telangiectasia type 2. J Med Genet (2003) 1.01
Pulmonary arterial hypertension: basis of sex differences in incidence and treatment response. Br J Pharmacol (2014) 0.97
Paigen diet-fed apolipoprotein E knockout mice develop severe pulmonary hypertension in an interleukin-1-dependent manner. Am J Pathol (2011) 0.97
Transforming growth factor-beta1 causes pulmonary microvascular endothelial cell apoptosis via ALK5. Am J Physiol Lung Cell Mol Physiol (2009) 0.96
The genetics of pulmonary arterial hypertension. Circ Res (2014) 0.95
Signal transduction in the development of pulmonary arterial hypertension. Pulm Circ (2013) 0.95
Pulmonary arterial hypertension associated with systemic sclerosis. Expert Rev Respir Med (2011) 0.95
Increased oxidative stress and severe arterial remodeling induced by permanent high-flow challenge in experimental pulmonary hypertension. Respir Res (2011) 0.95
The lysosomal inhibitor, chloroquine, increases cell surface BMPR-II levels and restores BMP9 signalling in endothelial cells harbouring BMPR-II mutations. Hum Mol Genet (2013) 0.94
Transforming growth factor-β and smooth muscle differentiation. World J Biol Chem (2012) 0.94
Clinical and analytical sensitivities in hereditary hemorrhagic telangiectasia testing and a report of de novo mutations. J Mol Diagn (2007) 0.93
Serum endostatin is a genetically determined predictor of survival in pulmonary arterial hypertension. Am J Respir Crit Care Med (2015) 0.91
Pulmonary hypertension. Am J Respir Crit Care Med (2005) 0.90
Anticipated classes of new medications and molecular targets for pulmonary arterial hypertension. Pulm Circ (2013) 0.90
Mutation study of Spanish patients with hereditary hemorrhagic telangiectasia. BMC Med Genet (2008) 0.89
Cell-specific dual role of caveolin-1 in pulmonary hypertension. Pulm Med (2011) 0.87
Transforming growth factor-β inhibition attenuates pulmonary arterial hypertension in rats. Int J Clin Exp Med (2010) 0.87
Translating MicroRNA Biology in Pulmonary Hypertension: It Will Take More Than "miR" Words. Am J Respir Crit Care Med (2016) 0.87
Thoracic aortic disease in two patients with juvenile polyposis syndrome and SMAD4 mutations. Am J Med Genet A (2012) 0.86
Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin (2012) 0.86
Eisenmenger syndrome and atrial septal defect: nature or nurture? Can J Cardiol (2006) 0.86
Linking a serotonin transporter polymorphism to vascular smooth muscle proliferation in patients with primary pulmonary hypertension. J Clin Invest (2001) 0.85
Transforming growth factor-beta1 protects against pulmonary artery endothelial cell apoptosis via ALK5. Am J Physiol Lung Cell Mol Physiol (2008) 0.85
Hemodynamic and genetic analysis in children with idiopathic, heritable, and congenital heart disease associated pulmonary arterial hypertension. Respir Res (2013) 0.85
Pulmonary arterial hypertension: an imaging review comparing MR pulmonary angiography and perfusion with multidetector CT angiography. Br J Radiol (2012) 0.85
Pulmonary arterial hypertension: insights from genetic studies. Proc Am Thorac Soc (2011) 0.84
Genetic clues to the cause of primary pulmonary hypertension. N Engl J Med (2001) 0.84
Mutations of NOTCH3 in childhood pulmonary arterial hypertension. Mol Genet Genomic Med (2014) 0.84
Connective tissue disorders and cardiovascular complications: the indomitable role of transforming growth factor-beta signaling. Adv Exp Med Biol (2014) 0.83
The management of pulmonary hypertension in children. Arch Dis Child (2008) 0.83
Novel promoter and exon mutations of the BMPR2 gene in Chinese patients with pulmonary arterial hypertension. Eur J Hum Genet (2009) 0.82
Nitric oxide and pulmonary hypertension. Korean J Anesthesiol (2010) 0.82
New insights into the pathogenesis and treatment of primary pulmonary hypertension. Thorax (2001) 0.82
Recent trends in pulmonary arterial hypertension. Lung India (2011) 0.82
Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia. Heart Vessels (2010) 0.82
Targeting BMP signalling in cardiovascular disease and anaemia. Nat Rev Cardiol (2015) 0.82
Emerging roles of BMP9 and BMP10 in hereditary hemorrhagic telangiectasia. Front Genet (2015) 0.81
Heritable forms of pulmonary arterial hypertension. Semin Respir Crit Care Med (2013) 0.81
Endoglin: a critical mediator of cardiovascular health. Vasc Health Risk Manag (2013) 0.81
Regulation of bone morphogenetic protein 9 (BMP9) by redox-dependent proteolysis. J Biol Chem (2014) 0.81
Future breakthroughs in the genetics of pulmonary arterial hypertension. Pulm Circ (2013) 0.80
Genetic epidemiology: systemic sclerosis. Arthritis Res (2002) 0.80
TBX4 mutations (small patella syndrome) are associated with childhood-onset pulmonary arterial hypertension. J Med Genet (2013) 0.79
Mutations of activin-receptor-like kinase 1 (ALK-1) are not found in patients with pulmonary hypertension and underlying connective tissue disease. Clin Rheumatol (2006) 0.79
Oxidative stress contributes to endothelial dysfunction in mouse models of hereditary hemorrhagic telangiectasia. Oxid Med Cell Longev (2012) 0.79
Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr Transplant (2010) 0.78
High Frequency of Pulmonary Hypertension-Causing Gene Mutation in Chinese Patients with Chronic Thromboembolic Pulmonary Hypertension. PLoS One (2016) 0.77
Cardiovascular changes in cirrhosis: pathogenesis and clinical implications. Saudi J Gastroenterol (2010) 0.76
From zebrafish heart jogging genes to mouse and human orthologs: using Gene Ontology to investigate mammalian heart development. F1000Res (2013) 0.76
Genomics of pulmonary arterial hypertension: implications for therapy. Heart Fail Clin (2010) 0.76
The role of genetics in pulmonary arterial hypertension. J Pathol (2016) 0.75
Pulmonary vascular disease related to hemodynamic stress in the pulmonary circulation. Compr Physiol (2011) 0.75
Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis. Lung India (2016) 0.75
Regression of pulmonary artery hypertension due to development of a pulmonary arteriovenous malformation. Indian Heart J (2014) 0.75
Integrating molecular genetics and systems approaches to pulmonary vascular diseases. Pulm Circ (2013) 0.75
Pulmonary hypertension in hereditary haemorrhagic telangiectasia. World J Cardiol (2015) 0.75
A rare case of pulmonary arterio-venous malformation with recurrent anemia: Hereditary hemorrhagic telangiectasia. Lung India (2015) 0.75
Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with connective tissue diseases. Ann Thorac Med (2014) 0.75
Pharmacologic treatments for pulmonary hypertension: exploring pharmacogenomics. Future Cardiol (2013) 0.75
Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Genetics of pulmonary hypertension. Ann Thorac Med (2014) 0.75
Allergic Rhinitis and its Impact on Asthma (ARIA) 2008 update (in collaboration with the World Health Organization, GA(2)LEN and AllerGen). Allergy (2008) 12.35
Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med (1996) 11.76
Effect of first-grade classroom environment on shy behavior, aggressive behavior, and concentration problems. Am J Community Psychol (1991) 7.50
Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. Nat Genet (2000) 7.20
A clinical trial of vena caval filters in the prevention of pulmonary embolism in patients with proximal deep-vein thrombosis. Prévention du Risque d'Embolie Pulmonaire par Interruption Cave Study Group. N Engl J Med (1998) 7.08
Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease. N Engl J Med (1995) 6.97
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature (2001) 6.95
Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet (2001) 6.88
Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J (2009) 6.64
A comparison of two antimicrobial-impregnated central venous catheters. Catheter Study Group. N Engl J Med (1999) 6.08
Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet (2002) 5.06
Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet (2008) 5.01
An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med (1992) 4.74
Treatment of venous thrombosis with intravenous unfractionated heparin administered in the hospital as compared with subcutaneous low-molecular-weight heparin administered at home. The Tasman Study Group. N Engl J Med (1996) 4.10
Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med (2000) 4.07
Inflammation in pulmonary arterial hypertension. Eur Respir J (2003) 3.18
Murine model of cutaneous infection with gram-positive cocci. Infect Immun (1992) 2.92
Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J (2010) 2.90
Long-term followup of breast cancer patients: the 30-year report. Cancer (1974) 2.86
Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J (2005) 2.83
Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet (2000) 2.82
Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med (1995) 2.76
Outcomes of the Kaiser Permanente Tele-Home Health Research Project. Arch Fam Med (2000) 2.69
Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest (2000) 2.64
Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest (2001) 2.59
BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension. Am J Hum Genet (2000) 2.51
Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred. N Engl J Med (2001) 2.45
Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J (2009) 2.41
Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. Eur Respir J (2004) 2.40
Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J (2006) 2.39
Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis (2006) 2.28
Systematic lung scans reveal a high frequency of silent pulmonary embolism in patients with proximal deep venous thrombosis. Arch Intern Med (2000) 2.25
Pulmonary vein stenosis after catheter ablation of atrial fibrillation. Circulation (1998) 2.25
Fast track open aortic surgery: reduced post operative stay with a goal directed pathway. Eur J Vasc Endovasc Surg (2007) 2.22
Allergic Rhinitis and its Impact on Asthma (ARIA): achievements in 10 years and future needs. J Allergy Clin Immunol (2012) 2.17
Sildenafil inhibits hypoxia-induced pulmonary hypertension. Circulation (2001) 2.15
Inhaled nitric oxide as a screening agent for safely identifying responders to oral calcium-channel blockers in primary pulmonary hypertension. Eur Respir J (1998) 2.14
Constitutive activation of hypoxia-inducible genes related to overexpression of hypoxia-inducible factor-1alpha in clear cell renal carcinomas. Cancer Res (2001) 2.12
Dermatitis/nephropathy syndrome of pigs. Vet Rec (1993) 2.11
Mutations in CDMP1 cause autosomal dominant brachydactyly type C. Nat Genet (1997) 2.06
Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax (2005) 2.03
Primary pulmonary hypertension and fenfluramine use. Br Heart J (1993) 2.02
Gene expression patterns in the lungs of patients with primary pulmonary hypertension: a gene microarray analysis. Circ Res (2001) 2.00
Smooth muscle cell matrix metalloproteinases in idiopathic pulmonary arterial hypertension. Eur Respir J (2005) 1.98
Hereditary haemorrhagic telangiectasia, an Australian cohort: clinical and investigative features. Intern Med J (2014) 1.98
Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax (2004) 1.96
Spotted fever in Brazil: a seroepidemiological study and description of clinical cases in an endemic area in the state of São Paulo. Am J Trop Med Hyg (2001) 1.95
Histoplasmosis in normal hosts. Medicine (Baltimore) (1981) 1.95
Incidence of miltiple primary cancers. IV. Cancers of the female breast and genital organs. J Natl Cancer Inst (1971) 1.92
Predicting and evaluating response to omalizumab in patients with severe allergic asthma. Respir Med (2007) 1.86
Increased plasma serotonin in primary pulmonary hypertension. Am J Med (1995) 1.86
Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J (2009) 1.85
Permeability properties of the intact mammalian bladder epithelium. Am J Physiol (1996) 1.84
Spontaneous functional correction of homozygous fanconi anaemia alleles reveals novel mechanistic basis for reverse mosaicism. Nat Genet (1999) 1.81
Pulmonary veno-occlusive disease: a case series and new observations. Chest (2000) 1.80
Synpolydactyly phenotypes correlate with size of expansions in HOXD13 polyalanine tract. Proc Natl Acad Sci U S A (1997) 1.78
Dendritic cell recruitment in lesions of human and experimental pulmonary hypertension. Eur Respir J (2006) 1.78
Modifying the request behaviour of clinicians. J Clin Pathol (1992) 1.78
Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia. J Med Genet (2003) 1.76
Further clinical and molecular delineation of the 9q subtelomeric deletion syndrome supports a major contribution of EHMT1 haploinsufficiency to the core phenotype. J Med Genet (2009) 1.74
[Guidelines for the diagnosis and treatment of pulmonary hypertension]. Rev Mal Respir (2010) 1.74
Chronic thromboembolic pulmonary hypertension. Eur Respir J (2004) 1.74
Molecular and cellular basis of pulmonary vascular remodeling in pulmonary hypertension. Prog Cardiovasc Dis (2003) 1.73
Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J (2004) 1.73
Changing trends in the management of iatrogenic ureteral injuries. J Urol (1995) 1.72
Randomized clinical trial of diethylstilbestrol versus tamoxifen in postmenopausal women with advanced breast cancer. N Engl J Med (1981) 1.70
Heterozygosity for a mutation in the parkin gene leads to later onset Parkinson disease. Neurology (2003) 1.70
High prevalence of detectable deep venous thrombosis in patients with acute pulmonary embolism. Chest (1999) 1.70
Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis (1984) 1.68