肺高血圧症治療ガイドライン（2012年改訂版）
Guidelines for Treatment of Pulmonary Hypertension（JCS2012）
Guidelines for Treatment of Pulmonary Hypertension（JCS2012）
AASM：American Academy of Sleep Medicine
A-aDO2：alveolar-arterial O2 tension difference/gradient
AHA：American Heart Association
ACC：American College of Cardiology
ACCP：American College of Chest Physician
ALK1：activin receptor-like kinase-1
ACVRL1：activin A receptor type II-like 1
AHI：apnea hypopnea index
ARB：angiotensin receptor blocker
ASD：atrial septal defect
BMPR2：bone morphogenetic protein receptor type II
BNP：brain natriuretic peptide
BPA：balloon pulmonary angioplasty
CAV1：caveolin-1
CCB：calcium channel blocker
cGMP：cyclic guanosine monophosphate
CHD-PAH：congenital hear disease-pulmonary arterial hypertension
CI：cardiac index
CO：cardiac output
CO2：carbon dioxide
COPD：chronic obstructive pulmonary disease
CTEPH：chronic thromboembolic pulmonary hypertension
CTD-PAH：connective heart disease-pulmonary arterial hypertension
CRT：cardiac resynchronization therapy
CPAP：continuous positive airway pressure
CPFE：combined pulmonary fibrosis and emphysema
CPX：cardio pulmonary exercise test
CSAS：central sleep apnea syndrome
DIC：disseminated intravascular coagulation
DLCO：carbon monoxide diffusing capacity
EC：endotherial cells
ECMO：extracorporeal membrane oxygenation
ECD：endocardial cushion defect
EGFR：epidermal growth factor receptor
EPC：endothelial progenitor cells
ERA：endothelin receptor antagonist
ERS：European Respiratory Society
ESC：European Society of Cardiology
HHT：hereditary hemorrhagic telangiectasia
HIV：human immunodeficiency virus
HOT：home oxygen therapy
HPAH：heritable pulmonary arterial hypertension
HRCT：high resolution computed tomography
ICD：implantable cardioverter defibrillator
IIP： idiopathic interstitial pneumonia
IPAH：idiopathic pulmonary arterial hypertension
IPF：idiopathic pulmonary fibrosis
JACC：Journal of the American College of Cardiology
LVEF：left ventricular ejection fraction
MAS：meconium aspiration syndrome
MCTD：mixed connective tissue disease
MRI：magnetic resonance imaging
NO：nitric oxide
NPPV：noninvasive positive pressure ventilation
NT-proBNP：N-terminal proBNP
NYHA：New York Heart Association
OI：Oxygenation Index
OSAS：obstructive sleep apnea syndrome
OSAHS：obstructive sleep apnea-hypopnea syndrome
PAG： pulmonary angiography
PAH：pulmonary arterial hypertension
PAHS： primary alveolar hypoventilation syndrome
PaCO2：partial pressure of carbon dioxide
PaO2：partial pressure of oxygen
PAP：pulmonary arterial pressure
PCH：pulmonary capillary hemangiomatosis
PCWP：pulmonary capillary wedge pressure
PDA：patent ductus arteriosus
PDE5-I：phosphodiesterase type 5 inhibitor
PDGF：platelet-derived growth factor
PEA：pulmonary endarterectomy
PGI2：prostaglandin I2
PM/DM：polymyositis/dermatomyositis
POPH：portopulmonary hypertension
PPAR- γ：peroxisome proliferator-activated receptor γ
PPH： primary pulmonary hypertension
PPHN：persistent pulmonary hypertension of the newborn
PSG： polysomnography
PTE：pulmonary thromboendarterectomy
PTMC：percutaneous transluminal mitral commissurotomy
PTPA：percutaneous transluminal pulmonary angioplasty
PTTM： pulmonary tumor thrombotic microangiopathy
PVOD：pulmonary veno-occlusive disease
PVR：pulmonary vascular resistance
QOL：quality of life
Qp：pulmonary blood flow
RA：right atrium
REM：rapid eye movement
RERA： respiratory effort-related arousal
SaO2：saturation of arterial oxygen
SAS：sleep apnea syndrome
SHVS： sleep hypoventilation /hypoxic syndrome
SLE：systemic lupus erythematosus
SMC：smooth muscle cell
SNP：single nucleotide polymorphism
SSc：systemic sclerosis
Svo2：mixed-venous oxygen saturation
TAPSE：tricuspid annular plane systolic excursion
TPG： transpulmonary pressure gradient
TGF： transforming growth factor
VEGF：vascular endothelial growth factor
VSD：Ventricular Septal Defect
VSMC：vascular smooth muscle cells
WHO：World Health Organization
6MWT：6 minutes walk test
A-aDO2：alveolar-arterial O2 tension difference/gradient
AHA：American Heart Association
ACC：American College of Cardiology
ACCP：American College of Chest Physician
ALK1：activin receptor-like kinase-1
ACVRL1：activin A receptor type II-like 1
AHI：apnea hypopnea index
ARB：angiotensin receptor blocker
ASD：atrial septal defect
BMPR2：bone morphogenetic protein receptor type II
BNP：brain natriuretic peptide
BPA：balloon pulmonary angioplasty
CAV1：caveolin-1
CCB：calcium channel blocker
cGMP：cyclic guanosine monophosphate
CHD-PAH：congenital hear disease-pulmonary arterial hypertension
CI：cardiac index
CO：cardiac output
CO2：carbon dioxide
COPD：chronic obstructive pulmonary disease
CTEPH：chronic thromboembolic pulmonary hypertension
CTD-PAH：connective heart disease-pulmonary arterial hypertension
CRT：cardiac resynchronization therapy
CPAP：continuous positive airway pressure
CPFE：combined pulmonary fibrosis and emphysema
CPX：cardio pulmonary exercise test
CSAS：central sleep apnea syndrome
DIC：disseminated intravascular coagulation
DLCO：carbon monoxide diffusing capacity
EC：endotherial cells
ECMO：extracorporeal membrane oxygenation
ECD：endocardial cushion defect
EGFR：epidermal growth factor receptor
EPC：endothelial progenitor cells
ERA：endothelin receptor antagonist
ERS：European Respiratory Society
ESC：European Society of Cardiology
HHT：hereditary hemorrhagic telangiectasia
HIV：human immunodeficiency virus
HOT：home oxygen therapy
HPAH：heritable pulmonary arterial hypertension
HRCT：high resolution computed tomography
ICD：implantable cardioverter defibrillator
IIP： idiopathic interstitial pneumonia
IPAH：idiopathic pulmonary arterial hypertension
IPF：idiopathic pulmonary fibrosis
JACC：Journal of the American College of Cardiology
LVEF：left ventricular ejection fraction
MAS：meconium aspiration syndrome
MCTD：mixed connective tissue disease
MRI：magnetic resonance imaging
NO：nitric oxide
NPPV：noninvasive positive pressure ventilation
NT-proBNP：N-terminal proBNP
NYHA：New York Heart Association
OI：Oxygenation Index
OSAS：obstructive sleep apnea syndrome
OSAHS：obstructive sleep apnea-hypopnea syndrome
PAG： pulmonary angiography
PAH：pulmonary arterial hypertension
PAHS： primary alveolar hypoventilation syndrome
PaCO2：partial pressure of carbon dioxide
PaO2：partial pressure of oxygen
PAP：pulmonary arterial pressure
PCH：pulmonary capillary hemangiomatosis
PCWP：pulmonary capillary wedge pressure
PDA：patent ductus arteriosus
PDE5-I：phosphodiesterase type 5 inhibitor
PDGF：platelet-derived growth factor
PEA：pulmonary endarterectomy
PGI2：prostaglandin I2
PM/DM：polymyositis/dermatomyositis
POPH：portopulmonary hypertension
PPAR- γ：peroxisome proliferator-activated receptor γ
PPH： primary pulmonary hypertension
PPHN：persistent pulmonary hypertension of the newborn
PSG： polysomnography
PTE：pulmonary thromboendarterectomy
PTMC：percutaneous transluminal mitral commissurotomy
PTPA：percutaneous transluminal pulmonary angioplasty
PTTM： pulmonary tumor thrombotic microangiopathy
PVOD：pulmonary veno-occlusive disease
PVR：pulmonary vascular resistance
QOL：quality of life
Qp：pulmonary blood flow
RA：right atrium
REM：rapid eye movement
RERA： respiratory effort-related arousal
SaO2：saturation of arterial oxygen
SAS：sleep apnea syndrome
SHVS： sleep hypoventilation /hypoxic syndrome
SLE：systemic lupus erythematosus
SMC：smooth muscle cell
SNP：single nucleotide polymorphism
SSc：systemic sclerosis
Svo2：mixed-venous oxygen saturation
TAPSE：tricuspid annular plane systolic excursion
TPG： transpulmonary pressure gradient
TGF： transforming growth factor
VEGF：vascular endothelial growth factor
VSD：Ventricular Septal Defect
VSMC：vascular smooth muscle cells
WHO：World Health Organization
6MWT：6 minutes walk test