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Withdrawal of sitaxentan in the treatment of pulmonary arterial hypertension
1. Preamble 2. Introduction 3. Definitions and classifications 3.1 Definitions 3.2. Classifications 4. Epidemiology and genetics of pulmonary hypertension 4.1 Epidemiology and risk factors 4.2 Genetics 5. Pulmonary hypertension diagnosis 5.1 Diagnosis5.1.1 Clinical presentation 5.1.2 Electrocardiogram 5.1.3 Chest radiograph 5.1.4 Pulmonary function tests and arterial blood gases 5.1.5 Echocardiography 5.1.6 Ventilation/perfusion lung scan 5.1.7 High-resolution computed tomography, contrast enhanced computed tomography, and pulmonary angiography 5.1.8 Cardiac magnetic resonance imaging 5.1.9 Blood tests and immunology5.1.10 Abdominal ultrasound scan 5.1.11 Right heart catheterization and vasoreactivity 5.1.12 Genetic testing 5.2 Diagnostic algorithm 6. Pulmonary arterial hypertension (group 1) 6.1 Clinical characteristics 6.2 Evaluation of severity6.2.1 Clinical parameters, imaging and haemodynamics 6.2.2 Exercise capacity 6.2.3 Biochemical markers 6.2.4 Comprehensive prognostic evaluation and riskassessment 6.2.5 Definition of patient status6.2.6 Treatment goals and follow-up strategy 6.3 Therapy 6.3.1 General measures 6.3.2 Supportive therapy 6.3.3 Specific drug therapy6.3.4 Combination therapy 6.3.5 Drug interactions6.3.6 Balloon atrial septostomy 6.3.7 Advanced right ventricular failure 6.3.8 Transplantation 6.3.9 Treatment algorithm 6.3.10 Diagnosis and treatment of pulmonary arterial hypertension complications 6.3.11 End of life care and ethical issues7. Specific pulmonary (arterial) hypertension subsets 7.1 Paediatric pulmonary arterial hypertension7.1.1 Diagnosis 7.1.2 Therapy 7.2 Pulmonary arterial hypertension associated with adult congenital heart disease 7.2.1 Diagnosis7.2.2 Therapy7.3 Pulmonary arterial hypertension associated with connective tissue disease 7.3.1 Diagnosis 7.3.2 Therapy 7.4 Pulmonary arterial hypertension associated with portal hypertension 7.4.1 Diagnosis 7.4.2 Therapy7.5 Pulmonary arterial hypertension associated with human immunodeficiency virus infection 7.5.1 Diagnosis 7.5.2 Therapy 7.6 Pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis 7.6.1 Diagnosis 7.6.2 Therapy 8. Pulmonary hypertension due to left heart disease (group 2)8.1 Diagnosis 8.2 Therapy9. Pulmonary hypertension due to lung diseases and/or hypoxia (group 3) 9.1 Diagnosis 9.2 Therapy 10. Chronic thromboembolic pulmonary hypertension (group 4) 10.1 Diagnosis 10.2 Therapy 10.2.1 Surgical 10.2.2 Medical 10.2.3 Interventional 11. Pulmonary hypertension with unclear and/or multifactorial mechanisms (group 5) 12. Definition of a pulmonary hypertension referral centre 13. To do and not to do messages from the guidelines 14. Appendix 15. Web addenda 16. References
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