Pulmonary Hypertension

High pressure in the lungs, or pulmonary hypertension, is most commonly due to smoking-related lung disease and conditions affecting the left heart, for example, high blood pressure in the arteries, faulty heart valves, and a weakened heart muscle. Pulmonary arterial hypertension (PAH), is a rare and complex disease, associated with many different medical conditions, which is caused by a growth of cells lining the inside of the lung arteries, resulting in high pressure in areas of the body. Both conditions lead to strain and potential failure of the right heart, and therefore awareness and early diagnosis of the condition is needed to lead to better outcomes: however this depends on education of all specialists and primary care clinicians about PAH. This practical handbook covers both PH and PAH, providing information required by the wide range of clinicians who will encounter these conditions, including cardiologists, respiratory physicians, rheumatologists, specialists in liver and AIDS medicine, and haematologists. It covers what it is, who it is likely to affect, how it is diagnosed, including the pitfalls of the various diagnostic tests, the different and evolving forms of treatment, long term management of this often devastating chronic disease, and the crucial role of multidisciplinary management in optimizing clinical care of these patients who usually have multi-system diseases. It also covers the up new classification for both PH and PAH, and new drugs that have recently become available.1. History of pulmonary hypertension and the circulation ; 2. Definitions of pulmonary hypertension and pulmonary arterial hypertension ; 3. Clinical classification of pulmonary hyptertension ; 4. Pathology of pulmonary hyptertension ; 5. Pathology of chronic thromboembolic pulmonary hypertension ; 6. Pathobiology of pulmonary arterial hypertension ; 7. Inflammation, growth factors and thrombosis in pulmonary arterial hypertension ; 8. Genetics and genomics of pulmonary arterial hypertension ; 9. Epidemiology of pulmonary arterial hypertension and pulmonary hypertension ; 10. Risk factors for pulmonary arterial hypertension ; 11. Pathophysiology of pulmonary hypertension in non-pulmonary arterial hypertension groups ; 12. Pulmonary arterial hypertension associated with connective tissue diseases ; 13. Systemic sclerosis ; 14. Systemic lupus erythematosus ; 15. Sjogren's syndrome ; 16. Polymyositis and dermatomyositis ; 17. HIV ; 18. Porto-pulmonary hypertension ; 19. Venous thromboembolism and acute pulmonary embolism ; 20. Acute pulmonary embolism ; 21. Chronic thromboembolic pulmonary hypertension ; 22. Pulmonary hypertension associated with congenital systemic-to-pulmonary cardiac shunts ; 23. Schistomiasis ; 24. Chronic haemolytic anaemias and pulmonary arterial hypertension ; 25. Interstitial lung disease and pulmonary hypertension ; 26. Prognosis of pulmonary arterial hypertension ; 27. The right ventricle ; 28. Clinical features of pulmonary arterial hypertension ; 29. Haemodynamics and treatment approaches in pulmonary hypertension due to left heart disease ; 30. Diagnostic algorithm ; 31. Simple investigations ; 32. Ventilation/perfusion scanning ; 33. Echocardiography ; 34. Non-invasive exercise testing ; 35. Cardiac catheterisation ; 36. Management of pulmonary arterial hypertension ; 37. General measures ; 38. Contraception in pulmonary arterial hypertension ; 39. Risks of pregnancy and contraception ; 40. Lifestyle issues ; 41. Elective surgery in patients with pulmonary arterial hypertension ; 42. Supportive therapy ; 43. Management of arrhythmias ; 44. Advanced therapies for pulmonary arterial hypertension ; 45. Endothelin and endothelin receptor antagonists ; 46. Phosphodiesterase-5 inhibitors ; 47. Classes of recommendations for procedures and treatments ; 48. Drug interactions with pulmonary arterial hypertension targeted therapies ; 49. Combination therapy in pulmonary arterial hypertension ; 50. Problems in conducting pulmonary arterial hypertension drug trials ; 51. Atrial septostomy ; 52. Translation