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Semin Respir Crit Care Med 2000; 21(6): 549-560
DOI: 10.1055/s-2000-13183
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Chronic Thromboembolic Pulmonary Hypertension

Peter F. Fedullo1 , Kim M. Kerr1 , William R. Auger1 , Stuart W. Jamieson2 , David P. Kapelanski2
  • 1Pulmonary and Critical Care Division, University of California, San Diego, UCSD Medical Center, San Diego, California
  • 2Division of Cardiothoracic Surgery, University of California, San Diego, UCSD Medical Center, San Diego, California
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Publication History

Publication Date:
31 December 2000 (online)

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ABSTRACT

Under most circumstances, chronic thromboembolic pulmonary hypertension represents a correctable form of pulmonary hypertension. Approximately 1500 thromboendarterectomy procedures have now been performed worldwide. Mortality rates reported by established programs with experience in the management of patients with this disease process have fallen to a range of 6 to 8%. This reduction in mortality has been contributed to by several factors: improved methods of preoperative evaluation and more selective surgical referral, increased surgical experience and refined techniques, and an increased understanding of the unique postoperative problems that occur following pulmonary thromboendarterectomy. Despite these advances, a great deal more needs to be accomplished. The early natural history and pathophysiologic mechanisms of the disease remain uncertain; improved diagnostic techniques are required; and the most feared complication of the procedure, reperfusion pulmonary edema, remains enigmatic in terms of its pathogenesis, prevention, and therapy.

KEYWORD

Pulmonary hypertension - thromboembolism - pulmonary embolism

REFERENCES

  • 1 Carroll D. Chronic obstruction of major pulmonary arteries.  Am J Med . 1950;  9 175-185
    CrossrefPubMedGoogle Scholar
  • 2 Jamieson S W, Auger W R, Fedullo P F. Experience and results with 150 pulmonary thromboendarterectomy operations over a 29-month period.  J Thorac Cardiovasc Surg . 1995;  106 116-127
    PubMedGoogle Scholar
  • 3 Fedullo P F, Auger W R, Channick R N. Chronic thromboembolic pulmonary hypertension.  Clin Chest Med . 1995;  16 353-374
    PubMedGoogle Scholar
  • 4 Daily P O, Auger W R. Historical perspectives: surgery for chronic thromboembolic disease.  Semin Thorac Cardiovasc Surg . 1999;  11 143-151
    PubMedGoogle Scholar
  • 5 Daily P O, Dembitsky W P, Jamieson S W. The evolution and the current state of the art of pulmonary thromboendarterectomy.  Semin Thorac Cardiovasc Surg . 1999;  11 152-163
    PubMedGoogle Scholar
  • 6 Jamieson S W, Kapelanski D P. Pulmonary endarterectomy.  Curr Probl Surg . 2000;  37(3) 165-252
    PubMedGoogle Scholar
  • 7 Kapelanski D P, Macoviak J A, Jamieson S W. Surgical interventions in the treatment of pulmonary embolism and chronic thromboembolic pulmonary hypertension. In: Oudkerk M, van Beels EJR. Ten Cate JW, eds. Pulmonary Embolism Berlin: Blackwell Science 1999: 382-397
    Google Scholar
  • 8 Hollister L E, Cull V L. The syndrome of chronic thrombosis of the the major pulmonary arteries.  Am J Med . 1956;  21 312-320
    CrossrefPubMedGoogle Scholar
  • 9 Houk V N, Hufnagel C A, McClenathan J E. Chronic thrombotic obstruction of major pulmonary arteries: Report of a case successfully treated by thromboendarterectomy and a review of the literature.  Am J Med . 1963;  35 269-282
    CrossrefPubMedGoogle Scholar
  • 10 Chitwood W R, Lyerly H K, Sabiston D C. Surgical management of chronic pulmonary embolism.  Ann Surg . 1985;  201 11-26
    PubMedGoogle Scholar
  • 11 Moser K M, Auger W R, Fedullo P F. Chronic major-vessel thromboembolic pulmonary hypertension.  Circulation . 1990;  81 1735-1743
    CrossrefPubMedGoogle Scholar
  • 12 Moser K M, Daily P O, Peterson K L. Thromboendarterectomy for chronic, major vessel thromboembolic pulmonary hypertension: immediate and long-term results in 42 patients.  Ann Intern Med . 1987;  107 560-565
    PubMedGoogle Scholar
  • 13 Levinson R M, Shure D, Moser K M. Reperfusion pulmonary edema after pulmonary thromboendarterectomy.  Am Rev Respir Dis . 1986;  134 1241-1245
    PubMedGoogle Scholar
  • 14 Olman M A, Auger W R, Fedullo P E. Pulmonary vascular steal in chronic thromboembolic pulmonary hypertension.  Chest . 1990;  98 1430-1434
    PubMedGoogle Scholar
  • 15 Moser K M, Metersky M L, Auger W R. Resolution of vascular steal after pulmonary thromboendarterectomy.  Chest . 1993;  104 1441-1444
    PubMedGoogle Scholar
  • 16 Shure D, Gregoratos G, Moser K M. Fiberoptic angioscopy: role in the diagnosis of chronic pulmonary artery obstruction.  Ann Intern Med . 1985;  103 844-850
    CrossrefPubMedGoogle Scholar
  • 17 Moser K M, Bloor C M. Pulmonary vascular lesions occurring in patients with chronic major-vessel thromboembolic pulmonary hypertension.  Chest . 1993;  103 684-692
    PubMedGoogle Scholar
  • 18 Ryan K L, Fedullo P F, Davis G B. Perfusion scan findings understate the severity of angiographic and hemodynamic compromise in chronic thromboembolic pulmonary hypertension.  Chest . 1988;  93 1180-1185
    CrossrefPubMedGoogle Scholar
  • 19 Auger W R, Fedullo P F, Moser K M. Chronic major-vessel chronic thromboembolic pulmonary artery obstruction: appearance of angiography.  Radiology . 1992;  183 393-398
    PubMedGoogle Scholar
  • 20 Kapitan K S, Buchbinder M, Wagner D. Mechanisms of hypoxemia in chronic thromboembolic pulmonary hypertension.  Am Rev Respir Dis . 1989;  139 1149-1154
    PubMedGoogle Scholar
  • 21 Auger W R, Permpikul P, Moser K M. Lupus anticoagulant, heparin use, and thrombocytopenia in patients with chronic thromboembolic pulmonary hypertension. A preliminary report.  Am J Med . 1995;  99 392-396
    CrossrefPubMedGoogle Scholar
  • 22 Archibald C J, Auger W R, Fedullo P F. Long-term outcome after pulmonary thromboendarterectomy.  Am J Respir Crit Care Med . 1999;  160 523-528
    CrossrefPubMedGoogle Scholar
  • 23 Bradley S P, Auger W R, Moser K M, Fedullo P F. Right ventricular pathology in chronic pulmonary hypertension.  Am J Cardiol . 1996;  78 584-587
    CrossrefPubMedGoogle Scholar
  • 24 Morris T A, Auger W R, Ysrael M Z. Parenchymal scarring is associated with restrictive spirometric defects in patients with chronic thromboembolic pulmonary hypertension.  Chest . 1996;  110 399-403
    PubMedGoogle Scholar
  • 25 Kapitan K S, Clausen J L, Moser K M. Gas exchange in chronic thromboembolism after pulmonary thromboendarterectomy.  Chest . 1990;  98 14-19
    PubMedGoogle Scholar
  • 26 Wragg R E, Dimsdale J E, Moser K M. Operative predictors of delirium after pulmonary thromboendarterectomy. A model for postcardiotomy delirium?.  J Thorac Cardiovasc Surg . 1988;  96 524-529
    PubMedGoogle Scholar
  • 27 Moser K M, Page G T, Ashburn W L, Fedullo P F. Perfusion lung scans provide a guide to which patients with apparent primary pulmonary hypertension merit angiography.  West J Med . 1988;  148 167-170
    PubMedGoogle Scholar
  • 28 Jault F, Cabrol C. Surgical treatment for chronic pulmonary thromboembolism.  Herz . 1989;  14 192-196
    PubMedGoogle Scholar
  • 29 Hartz R S, Byrne J G, Levitsky S. Predictors of mortality in pulmonary thromboendarterectomy.  Ann Thorac Surg . 1996;  62 1255-1259
    CrossrefPubMedGoogle Scholar
  • 30 Mayer E, Dahm M, Hake U. Mid-term results of pulmonary thromboendarterecomy for chronic thromboembolic pulmonary hypertension.  Ann Thorac Surg . 1996;  61 1788-1792
    CrossrefPubMedGoogle Scholar
  • 31 Dalen J E, Alpert J S. Natural history of pulmonary embolism.  Prog Cardiovasc Dis . 1975;  17 259-270
    CrossrefPubMedGoogle Scholar
  • 32 Peterson K L. Acute pulmonary embolism. Has its evolution been redefined?.  Circulation . 1999;  99 1280-1283
    PubMedGoogle Scholar
  • 33 Ribeiro A, Lindmarker P, Johnsson H, Juhlin-Dannfelt A, Jorfeldt L. Pulmonary embolism. One-year follow-up with echocardiography Doppler and five-year survival analysis.  Circulation . 1999;  99 1325-1330
    CrossrefPubMedGoogle Scholar
  • 34 Riedel M, Stanek V, Widimsky J, Prevovsky I. Longterm follow-up of patients with pulmonary thromboembolism: late prognosis and evolution of hemodynamic and respiratory data.  Chest . 1982;  81 151-158
    CrossrefPubMedGoogle Scholar
  • 35 Stein P D, Terrin M L, Hales C A. Clinical, laboratory, roentgenographic, and electrocardiographic findings in patients with acute pulmonary embolism and no pre-existing cardiac or pulmonary disease.  Chest . 1991;  100 598-603
    CrossrefPubMedGoogle Scholar
  • 36 Meignan M, Rosso J, Gauthier H. Systematic lung scans reveal a high frequency of silent pulmonary embolism in patients with proximal deep venous thrombosis.  Arch Intern Med . 2000;  160 159-164
    CrossrefPubMedGoogle Scholar
  • 37 Hansson Per-Olaf, Sorbo J, Eriksson H. Recurrent venous thromboembolism after deep venous thrombosis.  Arch Intern Med . 2000;  160 769-774
    CrossrefPubMedGoogle Scholar
  • 38 Wolf M, Boyer-Neumann C, Parent F. Thrombotic risk factors in pulmonary hypertension.  Eur Respir J . 2000;  15 395-399
    CrossrefPubMedGoogle Scholar
  • 39 Karwinski B, Svendsen E. Comparison of clinical and postmortem diagnosis of pulmonary embolism.  J Clin Pathol . 1989;  42 135-139
    PubMedGoogle Scholar
  • 40 Fedullo P F, Rubin L J, Kerr K M. The natural history of acute and chronic thromboembolic disease: the search for the missing link.  Eur Respir J . 2000;  15 435 (editorial)-437 (editorial)
    PubMedGoogle Scholar
  • 41 Auger W R, Moser K M. Pulmonary flow murmurs: a distinctive physical sign found in chronic pulmonary thromboembolic disease.  Clin Res . 1989;  37 145A (abstr)
    PubMedGoogle Scholar
  • 42 Voelkel M A, Wynne K M, Badesch D B. Hyperuricemia in severe pulmonary hypertension.  Chest . 2000;  117 19-24
    CrossrefPubMedGoogle Scholar
  • 43 Nagaya N, Uematsu M, Satoh T. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension.  Am J Respir Crit Care Med . 1999;  160 487-492
    CrossrefPubMedGoogle Scholar
  • 44 Bernstein R J, Ford R L, Clausen J L, Moser K M. Membrane diffusion and capillary blood volume in chronic thromboembolic pulmonary hypertension.  Chest . 1996;  110 1430-1436
    PubMedGoogle Scholar
  • 45 Torbicki A, Kurzyna M, Ciurzynski M. Proximal pulmonary emboli modify right ventricular ejection fraction.  Eur Respir J . 1999;  13 616-621
    CrossrefPubMedGoogle Scholar
  • 46 D'Alonzo G E, Bower J S, Dantzker D R. Differentiation of patients with primary and thromboembolic pulmonary hypertension.  Chest . 1984;  85 457-461
    PubMedGoogle Scholar
  • 47 Lisbona R, Kreisman H, Novales-Diaz J. Perfusion lung scanning: differentiation of primary from thromboembolic pulmonary hypertension.  Am J Roentgenol . 1985;  144 27-30
    PubMedGoogle Scholar
  • 48 Fishmann A J, Moser K M, Fedullo P F. Perfusion lung scans vs pulmonary angiography in evaluation of suspected primary pulmonary hypertension.  Chest . 1983;  84 679-683
    PubMedGoogle Scholar
  • 49 Fedullo P F, Auger W R, Moser K M. Hemodynamic response to exercise in patients with chronic, major vessel thromboembolic pulmonary hypertension.  Am Rev Respir Dis . 1990;  141 A890 (abstr)
    PubMedGoogle Scholar
  • 50 Nicod P, Peterson K, Levine M. Pulmonary angiography in severe chronic pulmonary hypertension.  Ann Intern Med . 1987;  107 565-568
    PubMedGoogle Scholar
  • 51 Pitton M B, Duber C, Mayer E, Thelen M. Hemodynamic effects of nonionic contrast bolus injection and oxygen inhalation during pulmonary angiography in patients with chronic major-vessel thromboembolic pulmonary hypertension.  Circulation . 1996;  94 2485-2491
    CrossrefPubMedGoogle Scholar
  • 52 Sompradeekul S, Fedullo P F, Kerr K M, Channick R N, Auger W R. The role of pulmonary angioscopy in the preoperative assessment of patients with thromboembolic pulmonary hypertension (CTEPH).  Am J Respir Crit Care Med . 1999;  159(3) A456 (abstr)
    PubMedGoogle Scholar
  • 53 Channick R N, Auger W R, Fedullo P F. Angioscopy. In: Feinsilver SH, Fein AM, eds. Textbook of Bronchoscopy Baltimore: Williams & Wilkins, 1995: 477-485
    Google Scholar
  • 54 Bergin C J, Sirlin C B, Hauschildt J P. Chronic thromboembolism: diagnosis with helical CT and MR imaging with angiographic and surgical correlation.  Radiology . 1997;  204 695-702
    PubMedGoogle Scholar
  • 55 King M A, Ysrael M, Bergin C J. Chronic thromboembolic pulmonary hypertension: CT findings.  Am J Roentgenol . 1998;  170 955-960
    PubMedGoogle Scholar
  • 56 Bergin C J, Rios G, King M A. Accuracy of high-resolution CT in identifying chronic pulmonary thromboembolic disease.  Am J Roentgenol . 1996;  166 1371-1377
    PubMedGoogle Scholar
  • 57 Bergin C J, Hauschildt J P, Brown M A. Identifying the cause of unilateral hypoperfusion in patients suspected to have chronic pulmonary thromboembolism: diagnostic accuracy of helical CT and conventional angiography.  Radiology . 1999;  213 743-749
    PubMedGoogle Scholar
  • 58 Fedullo P F, Auger W R, Dembitsky W P. Postoperative management of the patient undergoing pulmonary thromboendarterectomy.  Semin Thorac Cardiovasc Surg . 1999;  11 172-178
    PubMedGoogle Scholar
  • 59 Fadel E, Riou J Y, Maxmanian M. Pulmonary thromboendarterectomy for chronic thromboembolic obstruction of the pulmonary artery in piglets.  J Thorac Cardiovasc Surg . 1999;  117 787-793
    PubMedGoogle Scholar
  • 60 Troncy E, Collett J P, Shapiro S. Inhaled nitric oxide in acute respiratory distress syndrome: a pilot randomized controlled study.  Am J Respir Crit Care Med . 1998;  157 1483-1488
    CrossrefPubMedGoogle Scholar
  • 61 Dupont H, Le Corre F, Fierobe L. Efficiency of inhaled nitric oxide as rescue therapy during severe ARDS: survival and factors associated with the first response.  J Crit Care . 1999;  14 107-113
    CrossrefPubMedGoogle Scholar
  • 62 Kramm T, Mayer E, Dahm M. Long-term results after thromboendarterectomy for chronic pulmonary embolism.  Eur J Cardiothoracic Surg . 1999;  15 579-583
    CrossrefPubMedGoogle Scholar
  • 63 Mo M, Kapelanski D P, Mitruka S N. Reoperative pulmonary thromboendarterectomy.  Ann Thorac Surg . 1999;  68 1770-1776
    CrossrefPubMedGoogle Scholar
  • 64 Egermayer P, Peacock A J. Is pulmonary embolism a common cause of pulmonary hypertension?.  <~>Limitations of the embolic hypothesis. Eur Respir J . 2000;  15 440-448
    PubMedGoogle Scholar
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