RESUMEN

El diagnóstico de la hipertensión pulmonar requiere de un alto índice de sospecha.
El proceso diagnóstico involucra dos fases, la detección (determinar la etiología de los síntomas, demostrar la presencia de hipertensión pulmonar en pacientes de alto riesgo) y la caracterización (establecer el contexto clínico de la enfermedad, incluyendo los factores causales, enfermedades asociadas, alteraciones hemodinámicas, y secuelas).
En los pacientes sintomáticos se requiere de una evaluación exhaustiva para identificar una estrategia terapéutica adecuada que conlleve a disminuir los síntomas, mejorar la hemodinámica pulmonar y prolongar la sobrevida. En cambio, los pacientes asintomáticos requieren identificación de causas reversibles o tratables y una cuidadosa monitorización de la progresión de la enfermedad, para proponer una estrategia terapéutica conservadora (dependiendo de la severidad de las alteraciones hemodinámicas).
Una vez identificada la población de alto riesgo por medio de evaluación clínica, radiografía de tórax, electrocardiograma y ecocardiograma, se debe realizar el cateterismo cardiaco derecho para confirmar el diagnóstico y documentar el comportamiento hemodinámico inicial.
Las herramientas que permiten determinar la supervivencia incluyen la clase funcional, capacidad al ejercicio, la evolución del comportamiento hemodinámico, la respuesta en el test de reactividad vascular pulmonar, función ventricular derecha, y factores bioquímicos como péptido cerebral natriurético, endotelina 1, niveles de troponina y ácido úrico. El seguimiento estrecho de estos pacientes se recomienda en centros especializados.

PALABRAS CLAVE

Hipertensión arterial pulmonar idiopática, hipertensión arterial pulmonar, ecocadiograma, presión pulmonar media, cateterismo cardiaco derecho.

REFERENCIAS

1. Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewsky H, Gaine S. Diagnosis and differencial assesment of pulmonary arterial hypertension. J Am Coll Cardio 2004; 43: 40s-47s.

2. Sandoval J, Bauerle O, Gomez A, Palomar A, Martinez- Guerra ML, Furuya ME. Primary pulmonary hypertension in children. Clinical characterization and survival. J Am Coll Cardiol 1995; 25: 466-74.

3. Weitzenblum E. Chronic cor pulmonale. Heart 2003; 89: 225-30.

4. Rich S, Dantzker DR, Ayers SM. Primary pulmonary hypertension: a National Prospective Study. Ann Intern Med 1987; 107: 216-23.

5. McGoon MD. The Assessment of Pulmonary Hypertension. Clin Chest Med 2001; 22: 493-508.

6. Butman SM, Ewy GA, Standen JR, Kern KB, Hahn E. Bedside cardiovascular examination in patients with severe chronic heart failure: importance of rest or inducible jugular venous distension. J Am Coll Cardiol 1993; 22: 968-74.

7. Ducas J, Magder S, McGregor M. Validity of the hepatojugular reflux as a clinical test for congestive heart failure. Am J Cardiol 1983; 52: 1299-303.

8. Bull T. Physical Examination in Pulmonary Arterial Hypertension. Adv Pulm Hyperten 2005; 4: 23-30.

9. Newburger JW, Rosenthal A, Williams RG, Fellows K, Miettinen OS. Noninvasive tests in the initial evaluation of heart murmurs in children. N Engl J Med 1983; 308: 6-14.

10. Van de WF, Geboers J, Kesteloot H, De Geest H, Barrios L. The mechanism of disappearance of the physiologic third heart sound with age. Circulation 1986; 73: 877-84.

11. Escribano M, Gómez SMA, Diaz MJ, Palomera FJ, García MI. Lung function testing in patients with pulmonary artery hypertension. Arch Bronchoneumol 2005; 41: 380-4.

12. Viner SM, Bagg BR, Auger WR, Ford GT. The management of pulmonary hypertension secondary to chronic thromboembolic disease. Prog Cardiovasc Dis 1994; 37: 79-92.

13. Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992; 35: 365-70.

14. Lupi E, Horwitz S, Tejada VM, Dumont C, Galland F. Índices y mediciones radiológicas en la valoración de la hipertensión pulmonar. Arch Inst Cardiol Mex 1975; 45: 34-42.

15. Lupi E, Dumont C, Tejada VM, Horwitz S, Galland F. A radiologic index of pulmonary arterial hypertension. Chest 1975; 68: 28-31.

16. Arenas JL, Murillo A, Seoane M, Lupi HE. Índices y mediciones radiológicas en la valoración de la hipertensión arterial pulmonar en la neumopatía obstructiva crónica. Arch Inst Cardiol Mex 1983; 53: 187-90.

17. Kanemoto N, Furuya H, Etoh T, Sasamoto H, Matsuyama S. Chest roentgenograms in primary pulmonary hypertension. Chest 1979; 76: 45-9.

18. Parent JG, Schrijen F, Viana RA. Radiological indices of pulmonary hypertension in chronic obstructive pulmonary disease. Bull Eur Physiopathol Respir 1976; 12: 637-50.

19. Cueto RG, Mares GY, Escosura RG, Torres PE, Cicero SR. Diagnóstico de la hipertensión pulmonar. Rev Inst Nal Enf Resp Mex 2004; 17: s28-s39.

20. Kuriyama K, Gamsu G, Stern RG, Cann CE, Herfkens RJ, Brundage BH. CT-determined pulmonary artery diameters in predicting pulmonary hypertension. Invest Radiol 1984; 19: 16-22.

21. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Medical College of Wisconsin Lung Transplant Group. Chest 1998; 113: 1250-6.

22. Haimovici JB, Trotman-Dickenson B, Halpern EF, Dec GW, Ginns LC, Shepard JA, McLoud TC. Relationship between pulmonary artery diameter at computed tomography and pulmonary artery pressures at right-side heart catheterization. Acad Radiol 1997; 4: 327-34.

23. Alva L, Falcon V, Sotelo R. Hallazgos por imagen en la hipertensión pulmonar. Rev Inst Nal Enf Resp Mex 2004; 17: s40-s45.

24. Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imaging 1999; 14: 270-8.

25. Bossone E, Paciocco G, Iarussi D, Agretto A, Iacono A, Gillespie BW, Rubenfire M. The prognostic role of the ECG in primary pulmonary hypertension. Chest 2002; 121: 513-8.

26. Ahern GS, Tapson VF, Rebeiz A. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest 2002; 122: 524-7.

27. Beard JT, Bryd BF. Saline contrast enhancement of trivial Doppler tricuspid regurgitation signals for estimating pulmonary arterial pressure. Am J Cardiol 1988; 62: 486-488.

28. Rubin JL. Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines. Chest 2004; 126: 7S–10S.

29. Yeo TC, Dujardin KS, Tei C, Mahoney DW, McGoon MD, Seward JB. Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension. Am J Cardiol 1998; 81: 1157-61.

30. Eysmann SB, Palevsky HI, Reichek N, Hackney K, Douglas PS. Two dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension. Circulation 1989; 80: 353-60.

31. Raymond RJ, Hinderliter AL, Willis PW. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002; 39: 1214-9.

32. Meluzin J, Spinarova L, Bakala J. Pulsed Doppler tissue imaging of the velocity of tricuspid annular systolic motion; a new, rapid, and non-invasive method of evaluating right ventricular systolic function. Eur Heart J 2001; 22: 340-8.

33. Galie N, Hinderliter AL, Torbicki A. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and Doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 1380-6.

34. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension. ACCP evidence based clinical practice guidelines. Chest 2004; 126: 14S-34S.

35. American Thoracic Society. ATS Statement: Guidelines for the Six-Minute Walk Test. Am J Respir Crit Care Med 2002, 166: 111-17.

36. American Thoracic Society, American College of Chest Physicians. ATS/ACCP Statement on Cardiopulmonary Exercise Testing. Am J Respir Crit Care Med 2003; 167: 211-77.

37. Hoeper MM, Oudiz RJ, Peacock A, Tapson VF, Haworth SG, Frost AE, Torbicki A. End points and clinical trial designs in pulmonary arterial hypertension: clinical and regulatory perspectives. J Am Coll Cardiol 2004; 43: S48-S55.

38. Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, McLaughlin V, Hill N, Tapson VF, Robbins IM, Zwicke D, Duncan B, Dixon RA, Frumkin LR. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004; 169: 441-7.

39. Wensel R, Opitz C, Anker SD. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 2002; 106: 319-24.

40. Sun X-G, Oudiz RJ, Hansen, JE, Wasserman, K. Exercise pathophysiology in primary pulmonary vascular hypertension. Circulation 2001; 104: 429-35.

41. Hoeper MM, Taha N, Bekjarova A, Gatzke R, Spiekerkoetter E. Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids. Eur Respir J 2003; 22: 330-4.

42. Williamson TL, Kim NH, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Prog Cardiovasc Dis 2002; 45: 203-212.

43. Pengo V, Lensing AWA, Prins MH. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257-64.

44. Viner SM, Bagg BR, Auger WR. The management of pulmonary hypertension secondary to chronic thromboembolic disease. Prog Cardiovasc Dis 1994; 37: 79-82.

45. D’Alonzo GE, Bower JS, Dantzker DR. Differentiation of patients with primary and thromboembolic pulmonary hypertension. Chest 1984; 85: 457-61.

46. Ryan KL, Fedullo PF, Davis GB, et al. Perfusion scan findings understate the severity of angiographic and hemodynamic compromise in chronic thromboembolic pulmonary hypertension. Chest 1988; 93: 1180-5.

47. Fishman AP. The enigma of hypoxic pulmonary vasoconstriction. In: Fishman AP, Ed. The Pulmonary Circulation: Normal and Abnormal. Philadelphia: University of Pennsylvania Press; 1990:109-30.

48. Barberà JA, Peinado VI, Santos S. Pulmonary hypertension in chronic obstructive pulmonary disease. Eur Respir J 2003; 21: 892-905.

49. Leuchte HH, Neurohr C, Baumgartner R, Holzapfel M, Giehrl W, Vogeser M. Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 2004; 170: 360-5.

50. Nootens M, Kaufmann E, Rector T, et al. Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension: relation to hemodynamic variables and endothelin levels. J Am Coll Cardiol 1995; 26: 1581-5.

51. Rubens C, Ewert R, Halank M, et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001; 120: 1562-9.

52. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 2003; 108: 844-8.

53. Stupi AM, Steen VD, Owens GR. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum 1986; 29: 515-24.

54. Dawson JK, Goodson NC, Graham DR. Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology 2000; 39: 1320-5.

55. Raeside DA, Chalmers G, Clelland J. Pulmonary artery pressure variation in patients with connective tissue disease: 24 hour ambulatory pulmonary artery monitoring . Thorax 1998; 53: 857-62.

56. Falcoa CA, Alves IC, Chahade WH. Echocardiographic abnormalities and antiphospholipid antibodies in patients with systemic lupus erythematosus. Arq Bras Cardiol 2002; 79: 285-91.

57. Asherson RA, Higgenbottam TW, Dinh Xuan AT. Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. J Rheumatol 1990; 17: 1292-8.

58. 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.

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

60. Laffon E, Vallet C, Bernard V. A computed method for noninvasive MRI assessment of pulmonary hypertension. J Appl Physiol 2004; 96: 463-8.

61. Saba TS, Foster J, Cockburn M. Ventricular mass index using magnetic resonance imaging accurately estimates pulmonary artery pressure. Eur Respir J 2002; 20: 1519-24.

62. Hoeper MM, Tongers J, Leppert A. Evaluation of right ventricular performance with a right ventricular ejection fraction thermodilution catheter and MRI in patients with pulmonary hypertension. Chest 2001; 120: 502-7.

63. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Eng J Med 2001; 345: 1465-1472.

64. Palevsky HI, Schloo BL, Pietra GG, et al. Primary pulmonary hypertension: Vascular structure, morphometry, and responsiveness to vasodilator agents. Circulation 1989; 80: 1207-21.

65. Fletcher EC, Luckett RA, Miller T, Fletcher JG. Exercise hemodynamics and gas exchange in patients with chronic obstructive pulmonary disease, sleep desaturation, and a daytime PaO2 above 60 mmHg. Am Rev Respir Dis 1989; 140: 1237-45.

66. Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal. Philadelphia: University of Pennsylvania Press; 1990: 1-551.

67. Adamson PB, Reynolds D, Luby A, Magalski A, Steinhaus D, Linde C, Braunschweig F, Rydén L, Böhm M, Stäblein A, Takle T, Bennett T. Ongoing right ventricular hemodynamics in heart failure: clinical value of measurements derived from an implantable monitoring system. J Am Coll Cardiol 2003; 41: 565-71.

68. Ohlsson A, Steinhaus D, Kjellstrom B, Ryden L, Bennett T. Serial exercise testing in heart failure patients with central hemodynamic recording using implantable hemodynamic monitors. Eur J Heart Failure 2003; 5: 253-9.

69. Fruhwald F, Kjellström B, Perthold W, Watzinger N, Maier R, Grandjean P, Klein W. Continuous haemodynamic monitoring in pulmonary hypertensive patients treated with inhaled iloprost. Chest 2003; 124: 351-9.

70. McGoon M, Frantz R, Benza R, Bourge R, Severson C, Roettger A, Kjellstrom B, Bennett T. Continuous monitoring of pulmonary hemodynamics with an implantable device: initial experience in patients with pulmonary arterial hypertension. Abstract, PHA 6th International Conference, 2004.

71. Benza R, McGoon M, Frantz R, Bourge R, Severson C, Kjellstrom B, Roettger A, Bennett T. Monitoring pulmonary hemodynamic responses to epoprostenol infusions with an implantable hemodynamic monitoring device. Abstract, PHA 6th International Conference, 2004.

72. Frantz R, McGoon M, Benza R, Bourge R, Severson C, Kjellstrom B, Roettger A, Bennett T. Monitoring pulmonary artery and right ventricular hemodynamic responses to six-minute walk tests with an implantable monitoring device in PAH patients. Abstract, PHA 6th International Conference, 2004.

73. McGoon M, Frantz R, Benza R, Bourge R, Severson C, Kjellstrom B, Roettger A, Bennett T. Range of pulmonary artery-right heart pressures in ambulatory patients with pulmonary arterial hypertension: preliminary results from an implanted monitoring system. Abstract, PHA 6th International Conference, 2004.

74. Naeije R. Pulmonary vascular function. In Peacock A, Rubin L. Pulmonary Circulation. Diseases and their treatment. 2th edition. London Edward Arnold 2004: 3-13.