p.3 抽出テキスト

5.1.6. Ventilation/perfusion lung scan ................................................ 3643 5.1.7. Non-contrast and contrast-enhanced chest computed tomography examinations, and digital subtraction angiography ................................................................................................... 3644 5.1.8. Cardiac magnetic resonance imaging .................................... 3645 5.1.9. Blood tests and immunology .................................................... 3646 5.1.10. Abdominal ultrasound ............................................................... 3646 5.1.11. Cardiopulmonary exercise testing ....................................... 3646 5.1.12. Right heart catheterization, vasoreactivity, exercise, and fluid challenge .............................................................................................. 3646 5.1.12.1. Right heart catheterization ............................................. 3646 5.1.12.2. Vasoreactivity testing ........................................................ 3647 5.1.12.3. Exercise right heart catheterization ........................... 3647 5.1.12.4. Fluid challenge ...................................................................... 3647 5.1.13. Genetic counselling and testing ............................................ 3648 5.2. Diagnostic algorithm ............................................................................ 3648 5.2.1 Step 1 (suspicion) ........................................................................... 3648 5.2.2. Step 2 (detection) ......................................................................... 3648 5.2.3. Step 3 (confirmation) ................................................................... 3650 5.3. Screening and early detection .......................................................... 3652 5.3.1. Systemic sclerosis .......................................................................... 3653 5.3.2. BMPR2 mutation carriers ........................................................... 3653 5.3.3. Portal hypertension ...................................................................... 3653 5.3.4. Pulmonary embolism ................................................................... 3653 6. Pulmonary arterial hypertension (group 1) ......................................... 3655 6.1. Clinical characteristics ......................................................................... 3655 6.2. Severity and risk assessment ............................................................ 3655 6.2.1. Clinical parameters ....................................................................... 3655 6.2.2. Imaging ................................................................................................ 3656 6.2.2.1. Echocardiography .................................................................. 3656 6.2.2.2. Cardiac magnetic resonance imaging ........................... 3656 6.2.3. Haemodynamics ............................................................................. 3656 6.2.4. Exercise capacity ............................................................................ 3657 6.2.5. Biochemical markers .................................................................... 3658 6.2.6. Patient-reported outcome measures ................................... 3658 6.2.7. Comprehensive prognostic evaluation, risk assessment, and treatment goals .................................................................................. 3659 6.3. Therapy ..................................................................................................... 3660 6.3.1. General measures .......................................................................... 3660 6.3.1.1. Physical activity and supervised rehabilitation .......... 3660 6.3.1.2. Anticoagulation ...................................................................... 3660 6.3.1.3. Diuretics .................................................................................... 3661 6.3.1.4. Oxygen ...................................................................................... 3661 6.3.1.5. Cardiovascular drugs ........................................................... 3661 6.3.1.6. Anaemia and iron status .................................................... 3661 6.3.1.7. Vaccination ............................................................................... 3661 6.3.1.8. Psychosocial support ........................................................... 3661 6.3.1.9. Adherence to treatments .................................................. 3661 6.3.2. Special circumstances ................................................................... 3662 6.3.2.1. Pregnancy and birth control ............................................. 3662 6.3.2.1.1. Pregnancy ......................................................................... 3662 6.3.2.1.2. Contraception ................................................................ 3662 6.3.2.2. Surgical procedures .............................................................. 3662 6.3.2.3. Travel and altitude ................................................................ 3662 6.3.3. Pulmonary arterial hypertension therapies ........................ 3663 6.3.3.1. Calcium channel blockers .................................................. 3663 6.3.3.2. Endothelin receptor antagonists .................................... 3664 6.3.3.2.1. Ambrisentan ................................................................... 3665 6.3.3.2.2. Bosentan ........................................................................... 3665 6.3.3.2.3. Macitentan ....................................................................... 3666 6.3.3.3. Phosphodiesterase 5 inhibitors and guanylate cyclase stimulators ............................................................................................... 3666

6.3.3.3.1. Sildenafil ............................................................................ 3667 6.3.3.3.2. Tadalafil ............................................................................. 3667 6.3.3.3.3. Riociguat ........................................................................... 3667 6.3.3.4. Prostacyclin analogues and prostacyclin receptor agonists ...................................................................................................... 3667 6.3.3.4.1. Epoprostenol .................................................................. 3667 6.3.3.4.2. Iloprost .............................................................................. 3667 6.3.3.4.3. Treprostinil ...................................................................... 3667 6.3.3.4.4. Beraprost ......................................................................... 3667 6.3.3.4.5. Selexipag ........................................................................... 3667 6.3.4. Treatment strategies for patients with idiopathic, heritable, drug-associated, or connective tissue diseaseassociated pulmonary arterial hypertension .................................. 3667 6.3.4.1. Initial treatment decision in patients without cardiopulmonary comorbidities ...................................................... 3668 6.3.4.2. Treatment decisions during follow-up in patients without cardiopulmonary comorbidities .................................... 3669 6.3.4.3. Pulmonary arterial hypertension with cardiopulmonary comorbidities ...................................................... 3670 6.3.5. Drug interactions ........................................................................... 3671 6.3.6. Interventional therapy ................................................................. 3671 6.3.6.1. Balloon atrial septostomy and Potts shunt ................ 3671 6.3.6.2. Pulmonary artery denervation ........................................ 3671 6.3.7. Advanced right ventricular failure .......................................... 3672 6.3.7.1. Intensive care unit management ..................................... 3672 6.3.7.2. Mechanical circulatory support ....................................... 3672 6.3.8. Lung and heart–lung transplantation ..................................... 3672 6.3.9. Evidence-based treatment algorithm .................................... 3673 6.3.10. Diagnosis and treatment of pulmonary arterial hypertension complications ................................................................... 3673 6.3.10.1. Arrhythmias .......................................................................... 3673 6.3.10.2. Haemoptysis ......................................................................... 3673 6.3.10.3. Mechanical complications ................................................ 3673 6.3.11. End-of-life care and ethical issues ........................................ 3674 6.3.12. New drugs in advanced clinical development (phase 3 studies) ........................................................................................................... 3674 7. Specific pulmonary arterial hypertension subsets ............................ 3674 7.1. Pulmonary arterial hypertension associated with drugs and toxins ................................................................................................................... 3674 7.2. Pulmonary arterial hypertension associated with connective tissue disease .................................................................................................... 3675 7.2.1. Epidemiology and diagnosis ....................................................... 3675 7.2.2. Therapy .............................................................................................. 3675 7.3. Pulmonary arterial hypertension associated with human immunodeficiency virus infection ............................................................ 3676 7.3.1. Diagnosis ........................................................................................... 3676 7.3.2. Therapy .............................................................................................. 3676 7.4. Pulmonary arterial hypertension associated with portal hypertension .................................................................................................... 3677 7.4.1. Diagnosis ........................................................................................... 3677 7.4.2. Therapy .............................................................................................. 3677 7.4.2.1. Liver transplantation ............................................................ 3677 7.5. Pulmonary arterial hypertension associated with adult congenital heart disease .............................................................................. 3678 7.5.1. Diagnosis and risk assessment ................................................. 3678 7.5.2. Therapy .............................................................................................. 3679 7.6. Pulmonary arterial hypertension associated with schistosomiasis ................................................................................................ 3680 Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026