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. 1986 May;55(5):449–458. doi: 10.1136/hrt.55.5.449

Factors that influence the outcome of primary pulmonary hypertension.

A Rozkovec, P Montanes, C M Oakley
PMCID: PMC1216380  PMID: 3707785

Abstract

Factors predicting life expectancy in primary pulmonary hypertension have not been well defined. Thirty four cases of primary pulmonary hypertension that had been followed up until death or for at least five years were reviewed retrospectively. Patients were divided into three groups: 18 patients who died within five years of presentation to hospital; 12 who survived more than five years; and four who improved and who lived for more than five years. The age at onset was similar in the three groups and, like symptoms and sex, did not predict life expectancy. Right heart failure during the course of the disease was associated with a poor outcome. Radiographic evidence of cardiac enlargement and evidence of right heart strain on electrocardiogram at presentation was also predictive of survival for less than five years. Pulmonary arteriolar resistance was higher and cardiac output lower in those with the shortest survival times. There was no relation between pulmonary artery pressure and length of survival. Systemic resistance varied directly with pulmonary resistance and served to maintain systemic pressure. Presentation in or after pregnancy and patency of the foramen ovale were associated with longer survival. In four patients there was evidence of regression of the disease by cardiac catheterisation and lung histology. Primary pulmonary hypertension is a heterogeneous condition in which life expectancy varies widely.

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Selected References

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  1. AUSTEN W. G., MORROW A. G., BERRY W. B. EXPERIMENTAL STUDIES OF THE SURGICAL TREATMENT OF PRIMARY PULMONARY HYPERTENSION. J Thorac Cardiovasc Surg. 1964 Sep;48:448–455. [PubMed] [Google Scholar]
  2. Asherson R. A., Mackworth-Young C. G., Boey M. L., Hull R. G., Saunders A., Gharavi A. E., Hughes G. R. Pulmonary hypertension in systemic lupus erythematosus. Br Med J (Clin Res Ed) 1983 Oct 8;287(6398):1024–1025. doi: 10.1136/bmj.287.6398.1024-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. BURCHELL H. B., EDWARDS J. E., SHEPHERD J. T., SWAN H. J., WOOD E. H. Clinical, physiological, and pathological considerations in patients with idiopathic pulmonary hypertension. Br Heart J. 1957 Jan;19(1):70–82. doi: 10.1136/hrt.19.1.70. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bourdillon P. D., Oakley C. M. Regression of primary pulmonary hypertension. Br Heart J. 1976 Mar;38(3):264–270. doi: 10.1136/hrt.38.3.264. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Dreyer N. A., Pizzo S. V. Blood coagulation and idiopathic thromboembolism among fertile women. Contraception. 1980 Aug;22(2):123–135. doi: 10.1016/0010-7824(80)90057-8. [DOI] [PubMed] [Google Scholar]
  6. FitzGerald G. A., Brash A. R., Falardeau P., Oates J. A. Estimated rate of prostacyclin secretion into the circulation of normal man. J Clin Invest. 1981 Nov;68(5):1272–1276. doi: 10.1172/JCI110373. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Fujii A., Rabinovitch M., Matthews E. C. A case of spontaneous resolution of idiopathic pulmonary hypertension. Br Heart J. 1981 Nov;46(5):574–577. doi: 10.1136/hrt.46.5.574. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Fuster V., Steele P. M., Edwards W. D., Gersh B. J., McGoon M. D., Frye R. L. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984 Oct;70(4):580–587. doi: 10.1161/01.cir.70.4.580. [DOI] [PubMed] [Google Scholar]
  9. Gold F. L., Bache R. J. Transmural right ventricular blood flow during acute pulmonary artery hypertension in the sedated dog. Evidence for subendocardial ischemia despite residual vasodilator reserve. Circ Res. 1982 Aug;51(2):196–204. doi: 10.1161/01.res.51.2.196. [DOI] [PubMed] [Google Scholar]
  10. Goodman D. J., Harrison D. C., Popp R. L. Echocardiographic features of primary pulmonary hypertension. Am J Cardiol. 1974 Mar;33(3):438–443. doi: 10.1016/0002-9149(74)90329-4. [DOI] [PubMed] [Google Scholar]
  11. Gurtner H. P. Pulmonary hypertension, "plexogenic pulmonary arteriopathy" and the appetite depressant drug aminorex: post or propter? Bull Eur Physiopathol Respir. 1979 Sep-Oct;15(5):897–923. [PubMed] [Google Scholar]
  12. HEATH D., EDWARDS J. E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958 Oct;18(4 Pt 1):533–547. doi: 10.1161/01.cir.18.4.533. [DOI] [PubMed] [Google Scholar]
  13. HEATH D., HELMHOLZ H. F., Jr, BURCHELL H. B., DUSHANE J. W., KIRKLIN J. W., EDWARDS J. E. Relation between structural change in the small pulmonary arteries and the immediate reversibility of pulmonary hypertension following closure of ventricular and atrial septal defects. Circulation. 1958 Dec;18(6):1167–1174. doi: 10.1161/01.cir.18.6.1167. [DOI] [PubMed] [Google Scholar]
  14. HOWARTH S., LOWE J. B. The mechanism of effort syncope in primary pulmonary hypertension and cyanotic congenital heart disease. Br Heart J. 1953 Jan;15(1):47–54. doi: 10.1136/hrt.15.1.47. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Haworth S. G. Primary pulmonary hypertension. Br Heart J. 1983 Jun;49(6):517–521. doi: 10.1136/hrt.49.6.517. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Higenbottam T., Wheeldon D., Wells F., Wallwork J. Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet. 1984 May 12;1(8385):1046–1047. doi: 10.1016/s0140-6736(84)91452-1. [DOI] [PubMed] [Google Scholar]
  17. Howie P. W. Thromboembolism. Clin Obstet Gynaecol. 1977 Aug;4(2):397–417. [PubMed] [Google Scholar]
  18. Inglesby T. V., Singer J. W., Gordon D. S. Abnormal fibrinolysis in familial pulmonary hypertension. Am J Med. 1973 Jul;55(1):5–14. doi: 10.1016/0002-9343(73)90144-7. [DOI] [PubMed] [Google Scholar]
  19. JAMES T. N. On the cause of syncope and sudden death in primary pulmonary hypertension. Ann Intern Med. 1962 Feb;56:252–264. doi: 10.7326/0003-4819-56-2-252. [DOI] [PubMed] [Google Scholar]
  20. Juaneda E., Watson H., Haworth S. G. An unusual case of rapidly progressive primary pulmonary hypertension in childhood. Int J Cardiol. 1985 Mar;7(3):306–309. doi: 10.1016/0167-5273(85)90057-9. [DOI] [PubMed] [Google Scholar]
  21. Kahaleh M. B., Osborn I., Leroy E. C. Elevated levels of circulating platelet aggregates and beta-thromboglobulin in scleroderma. Ann Intern Med. 1982 May;96(5):610–613. doi: 10.7326/0003-4819-96-5-610. [DOI] [PubMed] [Google Scholar]
  22. Kanemoto N., Furuya H., Etoh T., Sasamoto H., Matsuyama S. Chest roentgenograms in primary pulmonary hypertension. Chest. 1979 Jul;76(1):45–49. doi: 10.1378/chest.76.1.45. [DOI] [PubMed] [Google Scholar]
  23. Kanemoto N., Sasamoto H. Pulmonary hemodynamics in primary pulmonary hypertension. Jpn Heart J. 1979 Jul;20(4):395–405. doi: 10.1536/ihj.20.395. [DOI] [PubMed] [Google Scholar]
  24. Kleiger R. E., Boxer M., Ingham R. E., Harrison D. C. Pulmonary hypertension in patients using oral contraceptives. A report of six cases. Chest. 1976 Feb;69(2):143–147. doi: 10.1378/chest.69.2.143. [DOI] [PubMed] [Google Scholar]
  25. Krayenbuehl H. P., Turina J., Hess O. Left ventricular function in chronic pulmonary hypertension. Am J Cardiol. 1978 Jun;41(7):1150–1158. doi: 10.1016/0002-9149(78)90872-x. [DOI] [PubMed] [Google Scholar]
  26. Loogen F., Both A. Primäre pulmonale hypertonie. Z Kardiol. 1976 Jan;65(1):1–14. [PubMed] [Google Scholar]
  27. Loyd J. E., Primm R. K., Newman J. H. Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis. 1984 Jan;129(1):194–197. doi: 10.1164/arrd.1984.129.1.194. [DOI] [PubMed] [Google Scholar]
  28. Lupi-Herrera E., Sandoval J., Seoane M., Bialostozky D. The role of hydralazine therapy for pulmonary arterial hypertension of unknown cause. Circulation. 1982 Apr;65(4):645–650. doi: 10.1161/01.cir.65.4.645. [DOI] [PubMed] [Google Scholar]
  29. MCCAFFREY R. M., DUNN L. J. PRIMARY PULMONARY HYPERTENSION IN PREGNANCY. Obstet Gynecol Surv. 1964 Aug;19:567–591. doi: 10.1097/00006254-196408000-00001. [DOI] [PubMed] [Google Scholar]
  30. Mueh J. R., Herbst K. D., Rapaport S. I. Thrombosis in patients with the lupus anticoagulant. Ann Intern Med. 1980 Feb;92(2 Pt 1):156–159. doi: 10.7326/0003-4819-92-2-156. [DOI] [PubMed] [Google Scholar]
  31. Nelson D. M., Main E., Crafford W., Ahumada G. G. Peripartum heart failure due to primary pulmonary hypertension. Obstet Gynecol. 1983 Sep;62(3 Suppl):58s–63s. [PubMed] [Google Scholar]
  32. Nenci G. G., Berrettini M., Todisco T., Costantini V., Grasselli S. Exhausted platelets in chronic obstructive pulmonary disease. Respiration. 1983;44(1):71–76. doi: 10.1159/000194530. [DOI] [PubMed] [Google Scholar]
  33. Oakley C., Somerville J. Oral contraceptives and progressive pulmonary vascular disease. Lancet. 1968 Apr 27;1(7548):890–893. doi: 10.1016/s0140-6736(68)90240-7. [DOI] [PubMed] [Google Scholar]
  34. Olukotun A. Y. Vasodilator therapy for pulmonary hypertension. N Engl J Med. 1980 May 29;302(22):1261–1262. [PubMed] [Google Scholar]
  35. Pearl R. G., Rosenthal M. H., Schroeder J. S., Ashton J. P. Acute hemodynamic effects of nitroglycerin in pulmonary hypertension. Ann Intern Med. 1983 Jul;99(1):9–13. doi: 10.7326/0003-4819-99-1-9. [DOI] [PubMed] [Google Scholar]
  36. Peters A. M., Rozkovec A., Bell R. N., Hallidie-Smith K. A., Goodwin J. F., Lavender J. P. Platelet kinetics in congenital heart disease. Cardiovasc Res. 1982 Jul;16(7):391–397. doi: 10.1093/cvr/16.7.391. [DOI] [PubMed] [Google Scholar]
  37. RAWSON A. J., WOSKE H. M. A study of etiologic factors in so-called primary pulmonary hypertension. Arch Intern Med. 1960 Feb;105:233–243. doi: 10.1001/archinte.1960.00270140055006. [DOI] [PubMed] [Google Scholar]
  38. Rich S., Ganz R., Levy P. S. Comparative actions of hydralazine, nifedipine and amrinone in primary pulmonary hypertension. Am J Cardiol. 1983 Nov 1;52(8):1104–1107. doi: 10.1016/0002-9149(83)90541-6. [DOI] [PubMed] [Google Scholar]
  39. Rich S., Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol. 1983 May 15;51(9):1560–1561. doi: 10.1016/0002-9149(83)90678-1. [DOI] [PubMed] [Google Scholar]
  40. Rich S., Martinez J., Lam W., Rosen K. M. Captopril as treatment for patients with pulmonary hypertension. Problem of variability in assessing chronic drug treatment. Br Heart J. 1982 Sep;48(3):272–277. doi: 10.1136/hrt.48.3.272. [DOI] [PMC free article] [PubMed] [Google Scholar]
  41. Riedel M., Stanek V., Widimsky J., Prerovsky I. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest. 1982 Feb;81(2):151–158. doi: 10.1378/chest.81.2.151. [DOI] [PubMed] [Google Scholar]
  42. Rodnan G. P., Myerowitz R. L., Justh G. O. Morphologic changes in the digital arteries of patients with progressive systemic sclerosis (scleroderma) and Raynaud phenomenon. Medicine (Baltimore) 1980 Nov;59(6):393–408. doi: 10.1097/00005792-198011000-00001. [DOI] [PubMed] [Google Scholar]
  43. Rubin L. J., Groves B. M., Reeves J. T., Frosolono M., Handel F., Cato A. E. Prostacyclin-induced acute pulmonary vasodilation in primary pulmonary hypertension. Circulation. 1982 Aug;66(2):334–338. doi: 10.1161/01.cir.66.2.334. [DOI] [PubMed] [Google Scholar]
  44. Rubin L. J., Nicod P., Hillis L. D., Firth B. G. Treatment of primary pulmonary hypertension with nifedipine. A hemodynamic and scintigraphic evaluation. Ann Intern Med. 1983 Oct;99(4):433–438. doi: 10.7326/0003-4819-99-4-433. [DOI] [PubMed] [Google Scholar]
  45. SAMET P., BERNSTEIN W. H. LOSS OF REACTIVITY OF THE PULMONARY VASCULAR BED IN PRIMARY PULMONARY HYPERTENSION. Am Heart J. 1963 Aug;66:197–199. doi: 10.1016/0002-8703(63)90034-6. [DOI] [PubMed] [Google Scholar]
  46. SLEEPER J. C., ORGAIN E. S., McINTOSH H. D. Primary pulmonary hypertension. Review of clinical features and pathologic physiology with a report of pulmonary hemodynamics derived from repeated catheterization. Circulation. 1962 Dec;26:1358–1369. doi: 10.1161/01.cir.26.6.1358. [DOI] [PubMed] [Google Scholar]
  47. Steer M. L., MacIntyre D. E., Levine L., Salzman E. W. Is prostacyclin a physiologically important circulating anti-platelet agent? Nature. 1980 Jan 10;283(5743):194–195. doi: 10.1038/283194a0. [DOI] [PubMed] [Google Scholar]
  48. Stein P. D., Sabbah H. N., Anbe D. T., Marzilli M. Performance of the failing and nonfailing right ventricle of patients with pulmonary hypertension. Am J Cardiol. 1979 Nov;44(6):1050–1055. doi: 10.1016/0002-9149(79)90168-1. [DOI] [PubMed] [Google Scholar]
  49. THILENIUS O. G., NADAS A. S., JOCKIN H. PRIMARY PULMONARY VASCULAR OBSTRUCTION IN CHILDREN. Pediatrics. 1965 Jul;36:75–87. [PubMed] [Google Scholar]
  50. Vlahakes G. J., Turley K., Hoffman J. I. The pathophysiology of failure in acute right ventricular hypertension: hemodynamic and biochemical correlations. Circulation. 1981 Jan;63(1):87–95. doi: 10.1161/01.cir.63.1.87. [DOI] [PubMed] [Google Scholar]
  51. Voss H., Feigel H., Bücking J. Langzeitverlauf der primär vaskulären pulmonalen Hypertonie mit und ohne Einnahme von Appetitzüglern. Z Kardiol. 1983 Apr;72(4):215–221. [PubMed] [Google Scholar]
  52. Wagenvoort C. A., Nauta J., van der Schaar P. J., Weeda H. W., Wagenvoort N. Effect of flow and pressure on pulmonary vessels. A semiquantitative study based on lung biopsies. Circulation. 1967 Jun;35(6):1028–1037. doi: 10.1161/01.cir.35.6.1028. [DOI] [PubMed] [Google Scholar]
  53. Walcott G., Burchell H. B., Brown A. L., Jr Primary pulmonary hypertension. Am J Med. 1970 Jul;49(1):70–79. doi: 10.1016/s0002-9343(70)80115-2. [DOI] [PubMed] [Google Scholar]
  54. Watanabe S., Ogata T. Clinical and experimental study upon primary pulmonary hypertension. Jpn Circ J. 1976 Jun;40(6):603–610. doi: 10.1253/jcj.40.603. [DOI] [PubMed] [Google Scholar]
  55. Wilson A. G., Harris C. N., Lavender J. P., Oakley C. M. Perfusion lung scanning in obliterative pulmonary hypertension. Br Heart J. 1973 Sep;35(9):917–930. doi: 10.1136/hrt.35.9.917. [DOI] [PMC free article] [PubMed] [Google Scholar]
  56. YU P. N. Primary pulmonary hypertension: report of six cases and review of literature. Ann Intern Med. 1958 Nov;49(5):1138–1161. doi: 10.7326/0003-4819-49-5-1138. [DOI] [PubMed] [Google Scholar]
  57. Young D., Mark H. Fate of the patient with the Eisenmenger syndrome. Am J Cardiol. 1971 Dec;28(6):658–669. doi: 10.1016/0002-9149(71)90054-3. [DOI] [PubMed] [Google Scholar]

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