ABSTRACT

Pulmonary hypertension is defined by the elevation of the pulmonary pressure above the normal reference values. Its presence, is related to progressive and often fatal intrinsic modifications in pulmonary circulation, or to the presence of other cardiac, pulmonary and systemic diseases. Diagnosis of the pulmonary hypertension etiology permits to establish the treatment and prognosis of the disease. Patients with pulmonary hypertension, idiopathic or related to other diseases, develop exertional dyspnea. Presence of right heart failure (generally with progressive course) manifested with reduced cardiac output or elevation or right atrial pressure, is associated with bad prognosis. The velocity of the progression is often variable, and frequently related with age and comorbid conditions. Patients with pulmonary hypertension and left cardiac disease have an slow and progressive evolution, whereas patients with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) or portal hypertension develop unresponsive and fatal disease. During last decades, classifications of pulmonary hypertension have been developed, based on etiology, clinical presentation and prognosis, with the objective of clinical investigation on diagnostic and therapeutic options. The first classification described in Geneva in 1973 during the First World Symposium of Pulmonary Hypertension create the bases of the last classification of Venice, Italy in 2003 during the Third World Symposium of Primary Pulmonary Hypertension, actually guides investigation and understanding of pulmonary hypertension.

KEYWORDS

Idiopathic pulmonary arterial hypertension, pulmonary arterial hypertension, pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, Evian classification, Venice classification.

REFERENCES

1. The International Primary Pulmonary Hypertension Study Group. The International Primary Pulmonary Hypertension Study (IPPHS). Chest 1994; 105(Suppl): 37S-41S.

2. Appelbaum L, Yigla M, Bendayan D. Primary pulmonary hypertension in Israel. A national survey. Chest 2001; 119: 1801-6.

3. Sáenz de la Calzada C, Sánchez V, Velásquez MT. Guías de práctica clínica de la Sociedad Española de Cardiología en tromboembolismo e hipertensión pulmonar. Rev Esp Cardiol 2001; 54: 194-210.

4. McQuillan B, Picard M, Leavitt M. Clinical correlates and reference intervals for pulmonary artery systolic pressure among echocardiographical normal subjects. Circulation 2001; 104: 2797-802.

5. Leung JA, Debatin JF. Three-dimensional contrast-enhanced magnetic resonance angiography of the thoracic vasculature. Eur Radiol 1997; 7: 981-9.

6. Raeside D, Smith A, Brown A. Pulmonary artery pressure measurement during exercise testing in patients with suspected pulmonary hypertension. Eur Resp J 2000; 16: 282-7.

7. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet 1998; 352: 719-25.

8. Saldanha MBS. Nossas diretrizes em hipertensão pulmonar. J Bras Pneumol 2005; 31: 20.

9. McLaughlin VV. Classification and epidemiology of pulmonary hypertension. Cardiol Clin 2002; 22: 327-41.

10. Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997; 336: 111-17.

11. Hatano S, Strasser T. Primary pulmonary hypertension. Reporton a WHO meeting. Geneva: World Health Organization. 1975.

12. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BG, Detre KM. Primary pulmonary hypertension: a national prospective study. Ann Intern Med 1987; 107: 216-23.

13. Rich S. Primary pulmonary hypertension. The world symposium of primary pulmonary hypertension 1998. World Health Organization http://www.who.int/cardiovascular_diseases/resources/publications/en

14. Rubin JL. Diagnosis and management of pulmonary arterial hypertension: ACCP Evidence-Based Clinical Practice Guidelines. Chest 2004; 126: 7S-10S.

15. Lane KB, Machado RD, Pauciulo N. Heterozygous germ-line mutations in BMPR2, encoding a TGF-b receptor, causa familial primary pulmonary hypertension. Nat Genet 2000; 26: 81-4.

16. Newman JH, Wheeler L, Lane KB. Mutation in the gene for BMPR2 as a cause for primary pulmonary hypertension in a large kindrer. N Engl J Med 2001; 345: 319-24.

17. Humbert M, Deng Z, Simonneau G. BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine derivates. Eur Resp J 2002; 20: 518-23.

18. Runo JR, Vnencak JCL, Prince M. Pulmonary veno-occlusive disease caused by an inherited mutation in BMPR2. Am J Crit Care Med 2003; 167: 889-94.

19. Eddahibi S, Humbert M, Fadel E. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001; 108: 1141-50.

20. Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43: 5S-12S.

21. Morse JH, Barst RJ, Horn E. Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor-2 mutations. J Rheumatol 2002; 29: 2379-81.

22. Kidd L, Driscoll D, Gersony W. Second natural history study of congenital herath defects. Results of treatment of patients with ventricular septal defects. Circulation 1993; 887: 138-51.

23. Morse JH, Barst RJ, Fotino M. Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. Am Rev Respir Dis 1992; 145: 787-92.

24. Daliento L, Somerville J, Presbitero P. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845-55.

25. Simonson JS, Schiller NB, Petri M. Pulmonary hypertension in systemic lupus erythematosus. J Rheumatol 1989; 16: 918-25.

26. Mukerjee D, St George D, Coleiro B. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003; 62: 1088-93.

27. Hadengue A, Benhayoun MK, Lebrec D. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991; 100: 520-8.

28. Kim KK, Factor SM. Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome. Hum Pathol 1987; 18: 1293-6.

29. Rodríguez-Roisin R, Krowka MJ, Fallon H. Pulmonary-hepatic vascular disorders. Eur Respir J 2004; 24: 861-880.

30. Petitpretz P, Brenot F, Azarian R. Pulmonary hypertension in patients with human immunodeficiency virus infection. Comparison with primary pulmonary hypertension. Circulation 1994; 89: 2722-7.

31. Brenot F, Herve P, Pettipretz P. Primary pulmonary hypertension and fenfluramine use. Br Heart J 1993; 70: 537-41.

32. Kawut SM, Taichman DB, Archer-Chicko CL. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344-50.

33. Humbert M, Maitre S, Capron F. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 1998; 157: 1681-5.

34. Weitzenblum E, Sautegeau A, Ehrhart M. Long-term course of pulmonary arterial pressure in chronic obstructive pulmonary disease. Am Rev Respir Dis 1984; 130: 993-8.

35. British Medical Research Council Party. Long-term domiciliary oxygen therapy in hypoxic cor pulmonale complicating bronchitis and emphysema. Lancet 1981; 1: 681-5.

36. Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease. Ann Intern Med 1980; 93: 391-8.

37. Jamieson SW, Kapelanski DP, Sakahibara N. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 2003; 76: 1457-64.

38. Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 1990; 81: 1735-43.

39. Schraufnagel DE, Sekosan M, McGee T. Human alveolar capillaries undergo angiogenesis in pulmonary veno-occlusive disease. Eur Respir J 1996; 9: 346-50.

40. McLaughlin VV, Genthner DE, Panella MM. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med 1999; 130: 740-3.