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El. knyga: Pulmonary Hypertension

(Consultant Cardiologist, Royal Free Hospital, Director of the National Pulm), (Consultant in Pulmonary Hypertension, Royal Free Hospital London, Honorary Senior Lecturer in Medicine, Royal Free and University College Medical School, UK)

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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 haemolyticanaemias 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
Clive Handler BSc, MD, MRCP, FACC FESC qualified from Guy's Hospital Medical School. He trained in cardiology at Guy's Hospital and at University College and Middlesex Hospitals. He was consultant Cardiologist at Northwick Park and St Mary's Hospitals and is now consultant in pulmonary hypertension in the National Pulmonary Hypertension Unit, Royal Free Hospital, London, and Honorary Consultant Cardiologist at Guy's and St Thomas' Hospitals and Honorary Senior Lecturer in Medicine, University College London, Medical School. He also works at both Highgate Hospital and the Hospital of St John and St Elizabeth.