肺高血圧症治療ガイドライン - JCS

1 2011 . 2012 . Guidelines for Treatment of Pulmonary Hypertension JCS2012 .. 2012 2 .. 3 18. 5 1. PAH 18. 1. 5 2. 39. 2. 5 3. / 42. 3. 7 4. CTEPH 50. 4. 7 56. 5. 13 57. 6. 15. 1. 2011 .. AASM American Academy of Sleep Medicine HRCT high resolution computed tomography A-aDO2 alveolar-arterial O2 tension difference/gradient ICD implantable cardioverter defibrillator AHA American Heart Association IIP idiopathic interstitial pneumonia ACC American College of Cardiology IPAH idiopathic pulmonary arterial hypertension ACCP American College of Chest Physician IPF idiopathic pulmonary fibrosis ALK1 activin receptor-like kinase-1 JACC Journal of the American College of Cardiology ACVRL1 activin A

2 Receptor type II-like 1 LVEF left ventricular ejection fraction AHI apnea hypopnea index MAS meconium aspiration syndrome ARB angiotensin receptor blocker MCTD mixed connective tissue disease ASD atrial septal defect MRI magnetic resonance imaging BMPR2 bone morphogenetic protein receptor type II NO nitric oxide BNP brain natriuretic peptide NPPV noninvasive positive pressure ventilation BPA balloon pulmonary angioplasty NT-proBNP N-terminal proBNP. CAV1 caveolin-1 NYHA New York Heart Association CCB calcium channel blocker OI Oxygenation Index cGMP cyclic guanosine monophosphate OSAS obstructive sleep apnea syndrome CHD-PAH congenital hear disease-pulmonary arterial OSAHS obstructive sleep apnea-hypopnea syndrome hypertension PAG pulmonary angiography CI cardiac index PAH pulmonary arterial hypertension CO cardiac output PAHS primary alveolar hypoventilation syndrome CO2 carbon dioxide PaCO2 partial pressure of carbon dioxide COPD chronic

3 Obstructive pulmonary disease PaO2 partial pressure of oxygen CTEPH chronic thromboembolic pulmonary hypertension PAP pulmonary arterial pressure CTD-PAH connective heart disease-pulmonary arterial PCH pulmonary capillary hemangiomatosis hypertension PCWP pulmonary capillary wedge pressure CRT cardiac resynchronization therapy PDA patent ductus arteriosus CPAP continuous positive airway pressure PDE5-I phosphodiesterase type 5 inhibitor CPFE combined pulmonary fibrosis and emphysema PDGF platelet-derived growth factor CPX cardio pulmonary exercise test PEA pulmonary endarterectomy CSAS central sleep apnea syndrome PGI2 prostaglandin I2.

4 DIC disseminated intravascular coagulation PM/DM polymyositis/dermatomyositis DLCO carbon monoxide diffusing capacity POPH portopulmonary hypertension EC endotherial cells PPAR- peroxisome proliferator-activated receptor . ECMO extracorporeal membrane oxygenation PPH primary pulmonary hypertension ECD endocardial cushion defect PPHN persistent pulmonary hypertension of the newborn EGFR epidermal growth factor receptor PSG polysomnography EPC endothelial progenitor cells PTE pulmonary thromboendarterectomy ERA endothelin receptor antagonist PTMC percutaneous transluminal mitral commissurotomy ERS European Respiratory Society PTPA percutaneous transluminal pulmonary angioplasty ESC European Society of Cardiology PTTM

5 Pulmonary tumor thrombotic microangiopathy HHT hereditary hemorrhagic telangiectasia PVOD pulmonary veno-occlusive disease HIV human immunodeficiency virus PVR pulmonary vascular resistance HOT home oxygen therapy QOL quality of life HPAH heritable pulmonary arterial hypertension Qp pulmonary blood flow 2.. RA right atrium SvO2 mixed-venous oxygen saturation REM rapid eye movement TAPSE tricuspid annular plane systolic excursion RERA respiratory effort-related arousal TPG transpulmonary pressure gradient SaO2 saturation of arterial oxygen TGF transforming growth factor SAS sleep apnea syndrome VEGF vascular endothelial growth factor SHVS sleep hypoventilation /hypoxic syndrome VSD Ventricular Septal Defect SLE systemic lupus erythematosus VSMC vascular smooth muscle cells SMC smooth muscle cell WHO World

6 Health Organization SNP single nucleotide polymorphism 6 MWT 6 minutes walk test SSc systemic sclerosis .. 2008 . 4 . Eisenmenger . primary pulmonary hypertension: PPH 1 2 . European Society of Cardiology: ESC / . European Respiratory Society: ERS . 3 4 . 2012 .. 2013. 2 5 .. 1999 2000 . 2006 .. 6 .. Minds . 2007 .. AHA American Heart 5 Association /ACC American College of Cardiology . ESC/ERS 1. 2 .. 3. 2011 . 1 1 2008 . Level A .. 2013 2 . Level B .. Level C .. 2 .. Class .. 2 . Class .. Class a .. Class b 3.

7 Class PPH .. 2009 10 PPH .. PAH .. PAH Eisenmenger . PPH .. 2 .. 4 .. 4.. 2 . PPH Eisenmenger . 1 1998 . WHO PPH . 2008 . mean PAP 5 . 25mmHg 3 5 . 2003 . mean PAP 30mmHg NIH PAH .. 7 8 2008 4 . pulmonary capillary wedged . pressure: PCWP 15mmHg . pulmonary arterial hypertension: PAH 9 ESC/ERS . mean PAP 14 3mmHg 3 4 . 20mmHg 6 mean PAP 20 . 25mmHg . 2013 . 5 .. 1 . 5 . 3 .. 3 . 1 PAH 2 . 1 idiopathic PAH: IPAH 1 . 2 . 2 heritable PAH: HPAH 3 . 1 BMPR2 4 / / . 2 ALK1 endoglin SMAD9 CAV1 3 . 3 . 1 . 2 . 3.

8 3 . 4 . 4 associated PAH: APAH . 5 . 1 . 6 . 2 . 7 . 3 . 4 4 CTEPH . 5 5 . 1' PVOD 1 . PCH 2 . 1'' PPHN . 3 . 4 .. 5. 2011 . 2006 10 . 1 PAH 12 . 5 . 1 PAH 2 PAH . 3 . 4 chronic 3 PAH connective tissue disease - thromboembolic pulmonary hypertension: CTEPH 5 PAH: CTD-PAH .. PAH . PAH: CTD-PAH . CTD-PAH . 1 PAH . PAH . SSc PAH 7 12. PAH PAH idiopathic PAH: 13 14 I PAH . IPAH PAH heritable PAH: HPAH SSc . PAH PAH 1 MCTD . pulmonary veno-occlusive disease: PVOD SLE 15 . pulmonary capillary hemangiomatosis: PAH.

9 PCH . PAH HIV PAH .. 4 HIV . 1 IPAH/HPAH HIV PAH . 1998 PAH HIV PAH PAH 6 . 2003 12 HIV PAH . 16 HIV PAH . PAH PAH HIV . PAH . HPAH 2000 5 portopulmonary hypertension: PAH BMPR2 POPH . 10 2001 BMPR2 TGF PAH POPH 10 . ACVRL1 ALK1 12 5 . 11 . 17 POPH . HPAH IPAH . BMPR2 ACVRL1 ENG SMAD9 18 . 6 congenital heart disease - PAH: PAH CHD-PAH . CHD- HPAH IPAH PAH . PAH PAH . 100 25 . 2 PAH 50 CHD-PAH Eisenmenger . PAH 19 Eisenmenger .. PAH . 6.. 22 . 7 . 4 chronic PAH . thromboembolic pulmonary hypertension: CTEPH.

10 PAH . IPAH CTEPH . 20 . PAH . CTEPH CTEPH . 8 pulmonary veno-occlusive . disease: PVOD . pulmonary capillary hemangiomatosis: PCH . 5 . PAH .. 1 . PCH PVOD . 21 .. PAH PVOD/PCH a . PVOD/PCH .. 9 persistent pulmonary 1 . hypertention of the newborn: PPHN .. 3 . 1 PAH . 1 .. 2 .. 3 . Rivero-Carvallo . Graham Steel . click .. PAH .. 3 . 4 . 3 . COPD . 1 .. 7. 2011 .. B . 23 24 26 . BNP NT-proBNP 27 28 .. PAH CTD-PAH 2 . POPH . HIV PAH HIV 30 . PAH 30 eccentricity index 30 .. 31 . tricuspid annular plane systolic excursion TAPSE.