肺高血圧症治療ガイドライン - j-circ.or.jp

1 2018 11 30 . 2018 03 23 . 2016 2017 . 2017 . Guidelines for Treatment of Pulmonary Hypertension JCS 2017/JPCPHS 2017 .. II .. 1.. 2018 3 .. 6 . PoPH 42. I. 8 . 1 8 44. 2 8 48. 8 HIV PAH HIV PAH 48. 3 11 PVOD / . 4 11 PCH 49. 11 2 51. 11 51. X 11 52. 12 53. 13 53. 13 54. - 13 3 / . MDCT 14 54. MRI 14 COPD 56. 14 59. 14 61. 16 AHS 62. 17 63. 17 65. 18 4 CTEPH 66. 18 67. 67. II. 20 CTEPH 67. 1 20 68. IPAH / 68. HPAH 20 72. BPA 73. CTD-PAH 34 CTEPH 74. 2.. 5 III. 90. 75. 6 76 IV. 90. / . IPAH/HPAH 76 98. 80. PPHN 86 102.. 6 MWD 6-minute walk distance 6 CHD congenital heart disease . - . A-aDO2 alveolus artery oxygen gradient CI cardiac index.

2 ACC American College of Cardiology CO carbon monoxide . chronic obstructive pulmonary ACD alveolar capillary dysplasia COPD . (lung) disease continuous positive airway ACE angiotensin converting enzyme CPAP . pressure combined pre- and postcapillary ACHD adult congenital heart disease CpcPH. PH. combined pulmonary brosis ACP advance care planning CPFE . and emphysema cardiopulmonary exercise AHA American Heart Association CPX . testing alveolar hypoventilation AHS CRP C-reactive protein C . syndrome cardiac resynchronization ALK activin receptor-like kinase CRT . therapy II . ARB angiotensin II receptor blocker CSA cross sectional area.

3 ASD atrial septal defect CT computed tomography . AVT aortic valve replacement CTD connective tissue disease . chronic thromboembolic . BMI body mass index CTEPH. pulmonary hypertension . BMP bone morphogenic protein CTPA CT pulmonary angiography CT . B-type (brain) natriuretic B . BNP CYC cyclophosphamide . peptide . BPA balloon pulmonary angioplasty DA digital angiography . displacement encoding with BPD bronchopulmonary dysplasia DENSE. stimulated echoes deep hypothermic intermittent BTR bridge to recovery DHCA . circulatory arrest diffusing capacity for carbon BTT bridge to transplantation DLco . monoxide Ca calcium DM dermatomyositis.

4 CDH congenital diaphragmatic hernia DOAC direct oral anticoagulants .. cGMP guanosine monophosphate DPG diastolic pressure gradient .. 3.. index of pulmonary vascular DSA digital subtraction angiography IPVD. disease DVT deep venous (vein) thrombosis IVC inferior vena cava . ECC extracorporeal circulation LGE late gadolinium enhancement . extracorporeal membrane ECMO LHD left heart disease . oxygenation limited cutaneous systemic . eNOS endothelial nitric oxide synthase lcSSc sclerosis . left ventricular end-diastolic ERA endothelin receptor antagonist LVEDP . pressure ERS European Respiratory Society MAP mean airway pressure.

5 Estimate right ventricular mixed connective tissue eRVSP MCTD . systolic pressure disease multi detector-row computed ESC European Society of Cardiology MDCT CT. tomography forced expiratory volume in one FEV1 1 MFH malignant brous histiocytoma . second mean pulmonary arterial FiO2 fraction of inspiratory oxygen mPAP . pressure FVC forced vital capacity NIH National Institutes of Health . highly active anti retroviral HIV . HAART NO nitric oxide . therapy . non-invasive positive airway HE hematoxylin eosin NPPV . pressure ventilation N-terminal pro-brain natriuretic BNP N . HFO high frequency oscillation NT-proBNP.

6 Peptide . heart failure with preserved . HFpEF NYHA New York Heart Association . ejection fraction . heart failure with reduced obesity hypoventilation HFrEF OHS . ejection fraction syndrome hereditary hemorrhagic . HHT OI oxygenation index . telangiectasia .. HHV-8 human herpesvirus 8 PAC pulmonary arterial capacitance . 8. arterial partial pressure of HIV human immunodeficiency virus PaCO2 . carbon dioxide HLA human leukocyte antigen PAG pulmonary angiography . HOT home oxygen therapy PAH pulmonary arterial hypertension . arterial partial pressure of HPAH heritable PAH PaO2 . oxygen high resolution computed HRCT CT PAP pulmonary artery pressure.

7 Tomography partial anomaly of pulmonary HPS hepatopulmonary syndrome PAPVR . vein return pulmonary artery wedge IABP intar aortic balloon pumping PAWP . pressure pulmonary capillary IL interleukin PCH . hemangiomatosis arterial partial pressure of ILD interstitial lung disease pCO2 . carbon dioxide post capillary pulmonary . iNO inhaled NO pcPH. hypertension . percutaneous cardiopulmonary IPAH idiopathic PAH PCPS . support IPF idiopathic pulmonary fibrosis PDA patent ductus arteriosus .. 4.. PDE phosphodiesterase SDB sleep-disordered breathing .. PDGF platelet-derived growth factor sGC soluble guanylate cyclase.

8 PEA pulmonary endarterectomy SI stroke index .. PEEP positive end-expiratory pressure SLE systemic lupus erythematosus . PGI2 prostacyclin SNP single nucleotide polymorphism . percutaneous oxygen . PH pulmonary hypertension SpO2. saturation . PM polymyositis SSc systemic scleroderma . PoPH portopulmonary hypertension SVC superior vena cava . persistent pulmonary mixed venous oxygen PPHN SvO2 . hypertension of newborn saturation pediatric pulmonary systemic vascular resistance PPHVD SVRI . hypertensive vascular disease index peripheral pulmonary artery tricuspid annular plane systolic . PPS TAPSE. stenosis excursion.

9 Transesophageal PTE pulmonary thromboembolism TEE . echocardiography prothrombin time-international . PT-INR TGF- transforming growth factor . normalized ratio . pulmonary veno-occlusive . PVOD TKI tyrosine kinase inhibitor disease . PVR pulmonary vascular resistance TNF- tumor necrosis factor . pulmonary vascular resistance transpulmonary pressure PVRI TPG . index gradient QOL quality of life TRPG transtricuspid pressure gradient . Qp pulmonary blood ow TRV tricuspid regurgitation velocity . pulmonary blood ow/systemic Qp/Qs TTE transthoracic echocardiography . blood ow ratio - . QTc corrected QT QT V-A ECMO veno-arterial ECMO.

10 ECMO. RAP right atrial pressure VC vital capacity .. RCM restrictive cardiomyopathy V O2 oxygen uptake . RCT randomized controlled trial VSD ventricular septal defect . - . RHC right heart catheterization V-V ECMO veno-venous ECMO. ECMO. RVP right ventricular pressure WHO World Health Organization . central venous oxygen ScvO2 . saturation 5.. 20 . 1 5 .. 5 .. I 2 .. 5 2013 . 5 .. European Society of Cardiology; ESC / . European Respiratory Society; ERS . 1, 2 . 2017 .. 1999 .. 2000 . 2006 2012 . Minds . 5 .. ESC/ERS .. 1 . 2 .. 3 .. 2 . I .. II . 6.. 1 2 . A . I . / .. B.. II.. C . IIa .. IIb .. III . III.