References
- AndrewsRPulmonary hypertension in pediatricsCurr Opin Ped200214603605
- GibbsJRecommendations on the management of pulmonary hypertension in clinical practiceHeart200186Suppl 111311410544
- TullohRCongenital heart disease in relation to pulmonary hypertension in paediatric practicePaediatr Respir Rev2005617418016153566
- FreedMDInvasive diagnostic and therapeutic techniques Part 1: cardiac catheterisationAdamsFHHeart Disease in Infants, Children and AdolescentsLondonWilliams and Wilkins2000130146
- AndrewsRAtrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease?Pediatr Cardiol20022352853012211202
- SimonneauGClinical classification of pulmonary hypertensionJ Am Coll Cardiol20044312 Suppl S5S12S15194173
- RabinovichMPathobiology of pulmonary hypertensionAnnu Rev Pathol2007236939918039104
- RabinovitchMVascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defectsCirculation1984696556676697454
- PietraGGHistopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension RegistryCirculation198980119812062805258
- JefferyTKMolecular and cellular basis of pulmonary vascular remodelling in pulmonary hypertensionProg Cardiovasc Dis20024517320212525995
- RichSPrimary pulmonary hypertension. A national prospective studyAnn Intern Med19871072162233605900
- WidlitzAPulmonary arterial hypertension in childrenEur Respir J20032115517612570125
- D’AlonzoGESurvival in patients with primary pulmonary hypertension. Results from a national prospective registryAnn Intern Med19911153433491863023
- RichSClinical insights into the pathogenesis of primary pulmonary hypertensionChest19981143 Suppl237S241S9741575
- National Pulmonary Hypertension Centres of the UK and IrelandConsensus statement on the management of pulmonary hypertension in clinical practice in the UK and IrelandThorax200863ii1ii4118308974
- ManesAPulmonary hypertension: classification and diagnostic algorithmItal Heart J2005683483916270476
- MaiyaSResponse to bosentan in children with pulmonary hypertensionHeart20069266467016216850
- AtzAMCombined effects of nitric oxide and oxygen during acute pulmonary vasodilator testingJ Am Coll Cardiol19993381381910080486
- MikhailGAn evaluation of nebulized prostacyclin in patients with primary and secondary pulmonary hypertensionEur Heart J1997189149915049458458
- MuthuranguVNovel method of quantifying pulmonary vascular resistance by use of simultaneous invasive pressure monitoring and phase-contrast magnetic resonance flowCirculation2004110782683415302793
- MuthuranguVMeasurement of total pulmonary arterial compliance using invasive pressure monitoring and MR flow quantification during MR-guided cardiac catheterizationAm J Physiol Heart Circ Physiol20052893H1301H130615879483
- HoeperMMGoal-oriented treatment and combination therapy for pulmonary arterial hypertensionEur Respir J20052685886316264047
- LanglebenDEndothelin-1 in acute lung injury and the adult respiratory distress syndromeAm Rev Respir Dis1993148(6 Pt 1):164616508256914
- DavieNET(A) and ET(B) receptors modulate the proliferation of human pulmonary artery smooth muscle cellsAm J Respir Crit Care Med2002165339840511818328
- HervéPIncreased plasma serotonin in primary pulmonary hypertensionAm J Med19959932492547653484
- ChristmanBWAn imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertensionN Engl J Med1992327270751603138
- PetkovVVasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertensionJ Clin Invest200311191339134612727925
- BandoKPulmonary hypertension after operations for congenital heart disease: analysis of risk factors and managementJ Thorac Cardiovasc Surg1996112616001607discussion 160716098975852
- LindbergLHow common is severe pulmonary hypertension after pediatric cardiac surgery?J Thorac Cardiovasc Surg200212361155116312063463
- OhsumiHEffects of fentanyl on carotid sinus baroreflex control of circulation in rabbitsAm J Physiol1989256(3 Pt 2):R625R6312493749
- AdriaenssensTAdvanced therapy may delay the need for transplantation in patients with the Eisenmenger syndromeEur Heart J200627121472147716707548
- BadeschDBMedical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelinesChest200713161917192817565025
- GalièNGuidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of CardiologyEur Heart J200425242243227815589643
- TaylorCJRisk of cardiac catheterization under anaesthesia in children with pulmonary hypertensionBr J Anaesth200798565766117401143
- SandovalJNocturnal oxygen therapy in patients with the Eisenmenger syndromeAm J Respir Crit Care Med200116491682168711719310
- SteudelWInhaled nitric oxide: basic biology and clinical applicationsAnesthesiology19999141090112110519513
- AdatiaIEffect of intracardiac repair on biosynthesis of thromboxane A2 and prostacyclin in children with a left to right shuntBr Heart J19947254524567818962
- BeghettiMContinuous low dose inhaled nitric oxide for treatment of severe pulmonary hypertension after cardiac surgery in paediatric patientsBr Heart J199573165687888265
- DavidsonDInhaled nitric oxide for the early treatment of persistent pulmonary hypertension of the term newborn: a randomized, double-masked, placebo-controlled, dose-response, multicenter study. The I-NO/PPHN Study GroupPediatrics1998101(3 Pt 1):3253349480993
- ThébaudBInhaled and exhaled nitric oxideCell Mol Life Sci1999558911031112
- LowsonSMAlternatives to nitric oxideBr Med Bull20047011913115531733
- OrtitzRMExtracorporeal membrane oxygenation in pediatric respiratory failurePediatr Clin North Am198734139463808772
- The Neonatal Inhaled Nitric Oxide Study GroupInhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failureNew Eng J Med19973365976049036320
- KinsellaJPInhaled nitric oxide therapy in childrenPaediatr Respir Rev20056319019816153568
- AbmanSHNeonatal pulmonary hypertension: a physiologic approach to treatmentPediatr Pulmonol Suppl20042612712815029626
- LevinDLPersistent pulmonary hypertension of the newborn infantJ Pediatr1976894626630784932
- HermonMIntravenous prostacyclin mitigates inhaled nitric oxide rebound effect: A case control studyArtif Organs1999231197597810564300
- GöthbergSResidual pulmonary hypertension in children after treatment with inhaled nitric oxide: a follow-up study regarding cardiopulmonary and neurological symptomsActa Paediatr200089121414141911195228
- BizzarroMInhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart diseaseCochrane Database Syst Rev20094CD005055
- MacraeDJInhaled nitric oxide therapy in neonates and children: reaching a European consensusIntensive Care Med200430337238014722629
- ChouPPulmonary changes following extracorporeal membrane oxygenation: autopsy study of 23 casesHum Pathol19932444054128491481
- ClarkRHLow-dose nitric oxide therapy for persistent pulmonary hypertension: 1 year follow-upJ Perinatol200323430030312774137
- FakiogluHHypoxic respiratory failure in term newborns: clinical indicators for inhaled nitric oxide and extracorporeal membrane oxygenation therapyJ Crit Care200520328829316253800
- LammersAEEpoprostenol treatment in children with severe pulmonary hypertensionHeart2007673974317065181
- MaiyaSResponse to bosentan in children with pulmonary hypertensionHeart200692566467016216850
- WimmerMExperience with long-term nifedipine therapy in paediatric cardiological patientsPadiatr Padol19902531811932116616
- HoudeCProfile of paediatric patients with pulmonary hypertension judged by responsiveness to vasodilatorsBr Heart J19937054614688260279
- MawatariEAmlodipine prevents monocrotaline-induced pulmonary arterial hypertension and prolongs survival in rats independent of blood pressure loweringClin Exp Pharmacol Physiol200734759460017581214
- AdatiaIInhaled nitric oxide and hemodynamic evaluation of patients with pulmonary hypertension before transplantationJ Am Coll Cardiol1995257165616647759720
- WeimannJSildenafil is a pulmonary vasodilator in awake lambs with acute pulmonary hypertensionAnesthesiology20009261702171210839922
- ReffelmannTTherapeutic potential of phosphodiesterase 5 inhibition for cardiovascular diseaseCirculation2003108223924412860892
- LeibovitchLTherapeutic applications of sildenafil citrate in the management of paediatric pulmonary hypertensionDrugs2007671577317209664
- HumplTBeneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot studyCirculation2005111243274328015956137
- RossiRSildenafil improves endothelial function in patients with pulmonary hypertensionPulm Pharmacol Ther200821117217717428713
- DillerGPCirculating endothelial progenitor cells in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertensionCirculation2008117233020303018519847
- BarnettCFSildenafil in the treatment of pulmonary hypertensionVasc Health Risk Manag20062441142217323595
- SpringRMSildenafil for pulmonary hypertension: dose-dependent improvement in exercise performancePulm Pharmacol Ther200821351652118249145
- GalièNSildenafil citrate therapy for pulmonary arterial hypertensionN Engl J Med20053532148215716291984
- BharaniAThe efficacy and tolerability of sildenafil in patients with moderate-to-severe pulmonary hypertensionIndian Heart J200355555912760589
- AtzAMSildenafil augments the effect of inhaled nitric oxide for postoperative pulmonary hypertensive crisesJ Thorac Cardiovasc Surg2002124362862912202882
- KothariSSChronic oral sildenafil therapy in severe pulmonary artery hypertensionIndian Heart J200254440440912462669
- LeeSHCurrent treatment strategies for pulmonary arterial hypertensionJ Intern Med2005258319921516115293
- Della TorreFSildenafil in pulmonary hypertensionSarcoidosis Vasc Diffuse Lung Dis2005221787915881285
- TeixeiraCEDifferential effects of the phosphodiesterase type 5 inhibitors sildenafil, vardenafil, and tadalafil in rat aortaJ Pharmacol Exp Ther2006316265466116204472
- ToqueHAVardenafil, but not sildenafil or tadalafil, has calcium-channel blocking activity in rabbit isolated pulmonary artery and human washed plateletsBr J Pharmacol2008154478779618536732
- LangIMPaediatric pulmonary vascular diseasePaediatr Respir Rev20045323824815276136
- GiaidAExpression of endothelin-1 in the lungs of patients with pulmonary hypertensionN Engl J Med199332824173217398497283
- CacoubPEndothelin-1 in pulmonary hypertensionN Engl J Med199332926196719688247067
- RosenzweigEBEffects of long-term bosentan in children with pulmonary arterial hypertensionJ Am Coll Cardiol200546469770416098438
- GalièNBosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled studyCirculation20061141485416801459
- GiannakoulasGBosentan in mild pulmonary hypertensionLancet2008372965117301731author reply 1731.
- BeghettiMSafety experience with bosentan in 146 children 2–11years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance programPediatr Res200864220020418414142
- Van LoonRLELong-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?Am Heart J200715477678217893008
- ApostolopoulouSCSitaxsentan in pulmonary arterial hypertensionChest200312351772; author reply 1772–177312740304
- BarstRJClinical efficacy of sitaxsentan, an endothelin-A receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot studyChest200212161860186812065350
- BenzaRLSitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survivalChest2008134477578218625676
- MehtaPKEndothelin receptor antagonists improve exercise tolerance and oxygen saturations in patients with Eisenmenger syndrome and congenital heart defectsTex Heart Inst J200835325626118941642
- GalièNAmbrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2Circulation2008117233010301918506008
- TudorAObservations on peripheral microcirculation in young hypertensive patientsRom J Intern Med200543127378
- BarstRJVasodilator therapy for primary pulmonary hypertension in childrenCirculation19999991197120810069788
- RosenzweigEBLong-term prostacyclin for pulmonary hypertension with associated congenital heart defectsCirculation199999141858186510199883
- RosenzweigEBPulmonary arterial hypertension in childrenPediatr Pulmonol200438122215170869
- TakaokaSCurrent therapies for pulmonary arterial hypertensionSemin Cardiothorac Vasc Anesth20071113714817536117
- BarstRJDiagnosis and treatment of pulmonary artery hypertensionCurr Opin Pediatr1996855125198946133
- BadeschDBProstanoid therapy for pulmonary arterial hypertensionJ Am Coll Cardiol20044312 Suppl S56S61S15194179
- SimonneauGContinuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trialAm J Respir Crit Care Med2002165680080411897647
- IvyDDTransition of stable pediatric patients with pulmonary arterial hypertension from intravenous epoprostenol to intravenous treprostinilAm J Cardiol200799569669817317374
- VizzaCDLong term treatment of pulmonary arterial hypertension with beraprost, an oral prostacyclin analogueHeart200186666166511711462
- BarstRJBeraprost therapy for pulmonary arterial hypertensionJ Am Coll Cardiol200341122119212512821234
- BakerSEInhaled iloprost in pulmonary arterial hypertensionAnn Pharmacother2005397–81265127415976392
- OlschewskiHInhaled iloprost for severe pulmonary hypertensionN Engl J Med2002347532232912151469
- GesslerTInhaled prostanoids in the therapy of pulmonary hypertensionJ Aerosol Med Pulm Drug Deliv200821111218518827
- IshikuraKBeneficial acute effects of rho-kinase inhibitor in patients with pulmonary arterial hypertensionCirc J200670217417816434811
- AlapatiVRMechanisms of U46619- and 5-HT-induced contraction of bovine pulmonary arteries: role of chloride ionsBr J Pharmacol200715181224123417592513
- SomlyoAPSignal transduction by G-proteins, rho-kinase and protein phosphatase to smooth muscle and non-muscle myosin IIJ Physiol2000522Pt 2:17718510639096
- ShimokawaHRho-kinase is an important therapeutic target in cardiovascular medicineArterioscler Thromb Vasc Biol20052591767177516002741
- AbeKLong-term treatment with a Rho-kinase inhibitor improves monocrotaline-induced fatal pulmonary hypertension in ratsCirc Res200494338539314670839
- FukumotoYRecent progress in the treatment of pulmonary arterial hypertension: expectation for rho-kinase inhibitorsTohoku J Exp Med2007211430932017409670
- FukumotoYAcute vasodilator effects of a Rho-kinase inhibitor, fasudil, in patients with severe pulmonary hypertensionHeart200591339139215710736
- JanosiTDifferential roles of endothelin-1 ETA and ETB receptors and vasoactive intestinal polypeptide in regulation of the airways and the pulmonary vasculature in isolated rat lungExp Physiol200893111210121918567602
- TofovicSP2-Ethoxyestradiol is antimitogenic and attenuates monocrotaline-induced pulmonary hypertension and vascular remodelingVascul Pharmacol20084846174183
- GurbanovEThe key role of apoptosis in the pathogenesis and treatment of pulmonary hypertensionEur J Cardiothorac Surg200630349950716870458
- MichelakisEDEmerging concepts and translational priorities in pulmonary arterial hypertensionCirculation20081181486149518824655
- AltieriDCSurvivin and apoptosis controlAdv Cancer Res200388315212665052
- GuerardPThe HMG-CoA reductase inhibitor, pravastatin, prevents the development of monocrotaline-induced pulmonary hypertension in the rat through reduction of endothelial cell apoptosis and overexpression of eNOSNaunyn Schmiedebergs Arch Pharmacol2006373640141416896805
- RakotoniainaZCelecoxib but not the combination of celecoxib +atorvastatin prevents the development of monocrotaline-induced pulmonary hypertension in the ratNaunyn Schmiedebergs Arch Pharmacol2008378324125118542928
- McMurtryMSStatin therapy, alone or with rapamycin, does not reverse monocrotaline pulmonary arterial hypertension: the rapamcyin-atorvastatin-simvastatin studyAm J Physiol Lung Cell Mol Physiol20072934L933L94017675370
- HervéPIncreased plasma serotonin in primary pulmonary hypertensionAm J Med19959932492547653484
- MacLeanMROverexpression of the 5-hydroxytryptamine transporter gene: effect on pulmonary hemodynamics and hypoxia-induced pulmonary hypertensionCirculation2004109172150215515078799
- MarcosESerotonin transporter inhibitors protect against hypoxic pulmonary hypertensionAm J Respir Crit Care Med2003168448749312773327
- GuignabertCSerotonin transporter inhibition prevents and reverses monocrotaline-induced pulmonary hypertension in ratsCirculation2005111212812281915927991
- KawutSMSelective serotonin reuptake inhibitor use and outcomes in pulmonary arterial hypertensionPulm Pharmacol Ther200619537037416483811
- ArimaMClinical features of acute pulmonary thromboembolism in younger patientsCirc J200367433033312655164
- CastilloLWhole body nitric oxide synthesis in healthy men determined from [15N] arginine-to-[15N] citrulline labelingProc Natl Acad Sci U S A19969311460114658876157
- McMullanDMAlterations in endogenous nitric oxide production after cardiopulmonary bypass in lambs with normal and increased pulmonary blood flowCirculation2000102supplIII172III17811082382
- BarrFEEffect of cardiopulmonary bypass on urea cycle intermediates and nitric oxide levels after congenital heart surgeryJ Pediatr2003142263012520250
- GorenfloMPlasma L-arginine and metabolites of nitric oxide synthase in patients with left-to-right shunt after intracardiac repairChest200512741184118915821193
- TanakaEPulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatmentJ Rheumatol200229228228711838845
- HoeperMMDrug treatment of pulmonary arterial hypertension: current and future agentsDrugs200565101337135415977967
- FasnachtMSSwiss Society for Pulmonary Arterial HypertensionThe Swiss registry for pulmonary arterial hypertension: the paediatric experienceSwiss Med Wkly20071373536510513
- MogollónMVCombination therapy with sildenafil and bosentan reverts severe pulmonary hypertension and allows heart transplantation: case reportTransplant Proc20063882522252317097987
- LiuCEndothelin receptor antagonists for pulmonary arterial hypertensionCochrane Database Syst Rev20063CD00443416856046
- DriscollJAMedical therapy for pulmonary arterial hypertensionExpert Opin Pharmacother200891658118076339
- HumbertMTreatment of pulmonary arterial hypertensionN Engl J Med2004351141425143615459304
- McLaughlinVVRandomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertensionAm J Respir Crit Care Med2006174111257126316946127
- HaraldssonAInhaled prostacyclin for treatment of pulmonary hypertension after cardiac surgery or heart transplantation: a pharmacodynamic studyJ Cardiothorac Vasc Anesth19961078648688969392
- BurgessGMutual phar-macokinetic interactions between steady-state bosentan and sildenafilEur J Clin Pharmacol2008641435018040672
- SimonneauGAddition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trialAnn Intern Med2008149852153018936500
- KersteinDBlade balloon atrial septostomy in patients with severe primary pulmonary hypertensionCirculation1995917202820357534664
- RichSAtrial septostomy as palliative therapy for refractory primary pulmonary hypertensionAm J Cardiol1983519156015616189385
- LawMAAtrial septostomy improves survival in select patients with pulmonary hypertensionAm Heart J2007153577978417452153
- HausknechtMJSuccessful palliation of primary pulmonary hypertension by atrial septostomyAm J Cardiol19906515104510461691586
- SandovalJGraded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatmentJ Am Coll Cardiol199822973049708453
- CheeverKHAn overview of pulmonary arterial hypertension: risks, pathogenesis, clinical manifestations, and managementJ Cardiovasc Nurs2005202108116quiz 117–118.15855858
- HuddlestonCBLung transplantation in childrenAnn Surg2002236327027612192313
- AuroraPRegistry of the International Society for Heart and Lung Transplantation: eleventh official pediatric lung and heart/lung transplantation report – 2008J Heart Lung Transplant200827997898318765189
- HaworthSGRole of the endothelium in pulmonary arterial hypertensionVascul Pharmacol200645531732517005453
- Galie’NTreatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomized controlled trialLancet20083712093210018572079
- GalieNA meta-analysis of randomized controlled trials in pulmonary arterial hypertensionEur Heart J20093039440319155250
- RubinLJTreatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trialAnn Intern Med19901124854912107780
- BarstRJA comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study GroupN Engl J Med19963342963028532025
- BadeschDBContinuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trialAnn Intern Med200013242543410733441
- ChannickRNEffects of the dual endothelin receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled studyLancet20013581119112311597664
- LanglebenDEffects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertensionAm Heart J2002143E412040360
- GalieNEffects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomised, double-blind placebo-controlled trialJ Am Coll Cardiol2002391496150211985913
- RubinLJBosentan therapy for pulmonary arterial hypertensionN Engl J Med200234689690311907289
- SastryBKSClinical efficacy of sildenafil in primary pulmonary hypertension. 1: A randomized, placebo-controlled, doubleblind, crossover studyJ Am Coll Cardiol2004431149115315063421
- HumbertMCombination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2Eur Respir J20042435335915358690
- BarstRJSitaxsentan therapy for pulmonary arterial hypertensionAm J Respir Crit Care Med2004169444144714630619
- GalieNThe Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertensionN Engl J Med20053532148215716291984
- WilkinsMRSildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) studyAm J Respir Crit Care Med20051711292129715750042
- SinghTA randomized, placebo controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertensionAm Heart J2006151851.e1851.e5
- BarstRJTreatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentanJ Am Coll Cardiol200647102049205616697324
- McLaughlinVVRandomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertensionAm J Respir Crit Care Med20061741257126316946127
- HoeperMCombining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertensionEur Resp J20064691694
- HislopADevelopmental biology of the pulmonary circulationPaediatr Respir Rev200561354315698814
- TorresFSystematic review of randomised, double-blind clinical trials of oral agents conducted in patients with pulmonary arterial hypertensionInt J Clin Pract200761101756176517877662