Pulmonary Hypertension (Guidelines on Diagnosis and Treatment of)

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 Diagnosis
5.1.1 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 immunology
5.1.10 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 severity
6.2.1 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 status
6.2.6 Treatment goals and follow-up strategy
6.3 Therapy
6.3.1 General measures
6.3.2 Supportive therapy
6.3.3 Specific drug therapy
6.3.4 Combination therapy
6.3.5 Drug interactions
6.3.6 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 issues
7. Specific pulmonary (arterial) hypertension subsets
7.1 Paediatric pulmonary arterial hypertension
7.1.1 Diagnosis
7.1.2 Therapy
7.2 Pulmonary arterial hypertension associated with adult congenital heart disease
7.2.1 Diagnosis
7.2.2 Therapy
7.3 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 Therapy
7.5 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 Therapy
9. 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