Pulmonary arterial hypertension in systemic sclerosis. 2021

Benoît Lechartier, and Marc Humbert
Lausanne University Hospital, Department of Respiratory Medicine, Lausanne, Switzerland.

Pulmonary arterial hypertension (PAH) is a frequent and severe complication of systemic sclerosis (SSc) due to combined vasculopathy and fibrogenesis. Early diagnosis and treatment are highly challenging in SSc-PAH and require referral to an expert PAH centre. Diagnostic algorithms evolved in the last decade. Novel therapeutic options notably targeting pulmonary vascular remodeling are needed.

UI MeSH Term Description Entries
D011659 Pulmonary Gas Exchange The exchange of OXYGEN and CARBON DIOXIDE between alveolar air and pulmonary capillary blood that occurs across the BLOOD-AIR BARRIER. Exchange, Pulmonary Gas,Gas Exchange, Pulmonary
D012017 Referral and Consultation The practice of sending a patient to another program or practitioner for services or advice which the referring source is not prepared to provide. Consultation,Gatekeepers, Health Service,Hospital Referral,Second Opinion,Consultation and Referral,Health Service Gatekeepers,Hospital Referrals,Referral,Referral, Hospital,Referrals, Hospital,Consultations,Gatekeeper, Health Service,Health Service Gatekeeper,Opinion, Second,Opinions, Second,Referrals,Second Opinions
D004719 Endomyocardial Fibrosis A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE). Endomyocardial Fibroses,Fibroses, Endomyocardial,Fibrosis, Endomyocardial
D005080 Exercise Test Controlled physical activity which is performed in order to allow assessment of physiological functions, particularly cardiovascular and pulmonary, but also aerobic capacity. Maximal (most intense) exercise is usually required but submaximal exercise is also used. Arm Ergometry Test,Bicycle Ergometry Test,Cardiopulmonary Exercise Testing,Exercise Testing,Step Test,Stress Test,Treadmill Test,Cardiopulmonary Exercise Test,EuroFit Tests,Eurofit Test Battery,European Fitness Testing Battery,Fitness Testing,Physical Fitness Testing,Arm Ergometry Tests,Bicycle Ergometry Tests,Cardiopulmonary Exercise Tests,Ergometry Test, Arm,Ergometry Test, Bicycle,Ergometry Tests, Arm,Ergometry Tests, Bicycle,EuroFit Test,Eurofit Test Batteries,Exercise Test, Cardiopulmonary,Exercise Testing, Cardiopulmonary,Exercise Tests,Exercise Tests, Cardiopulmonary,Fitness Testing, Physical,Fitness Testings,Step Tests,Stress Tests,Test Battery, Eurofit,Test, Arm Ergometry,Test, Bicycle Ergometry,Test, Cardiopulmonary Exercise,Test, EuroFit,Test, Exercise,Test, Step,Test, Stress,Test, Treadmill,Testing, Cardiopulmonary Exercise,Testing, Exercise,Testing, Fitness,Testing, Physical Fitness,Tests, Arm Ergometry,Tests, Bicycle Ergometry,Tests, Cardiopulmonary Exercise,Tests, EuroFit,Tests, Exercise,Tests, Step,Tests, Stress,Tests, Treadmill,Treadmill Tests
D006328 Cardiac Catheterization Procedures in which placement of CARDIAC CATHETERS is performed for therapeutic or diagnostic procedures. Catheterization, Cardiac,Catheterization, Heart,Heart Catheterization,Cardiac Catheterizations,Catheterizations, Cardiac,Catheterizations, Heart,Heart Catheterizations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000081029 Pulmonary Arterial Hypertension A progressive rare pulmonary disease characterized by high blood pressure in the PULMONARY ARTERY. Arterial Hypertension, Pulmonary,Hypertension, Pulmonary Arterial
D000465 Algorithms A procedure consisting of a sequence of algebraic formulas and/or logical steps to calculate or determine a given task. Algorithm
D012595 Scleroderma, Systemic A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D015150 Echocardiography, Doppler Measurement of intracardiac blood flow using an M-mode and/or two-dimensional (2-D) echocardiogram while simultaneously recording the spectrum of the audible Doppler signal (e.g., velocity, direction, amplitude, intensity, timing) reflected from the moving column of red blood cells. Doppler Echocardiography,Echocardiography, Continuous Doppler,Echocardiography, Two-Dimensional Doppler,2-D Doppler Echocardiography,2D Doppler Echocardiography,Continuous Doppler Echocardiography,Doppler Echocardiography, 2-D,Doppler Echocardiography, 2D,Doppler Echocardiography, Continuous,Doppler Echocardiography, Two-Dimensional,Echocardiography, 2-D Doppler,Echocardiography, 2D Doppler,Two-Dimensional Doppler Echocardiography,2 D Doppler Echocardiography,Doppler Echocardiography, 2 D,Doppler Echocardiography, Two Dimensional,Echocardiography, 2 D Doppler,Echocardiography, Two Dimensional Doppler,Two Dimensional Doppler Echocardiography

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