Thromb Haemost 2005; 93(03): 512-516
DOI: 10.1160/TH04-10-0657
Blood Coagulation, Fibrinolysis and Cellular Haemostasis
Schattauer GmbH

Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension

Diana Bonderman
1   Medical University of Vienna, Departments of Cardiology, Medical University of Vienna, Vienna, Austria
,
Johannes Jakowitsch
1   Medical University of Vienna, Departments of Cardiology, Medical University of Vienna, Vienna, Austria
,
Christopher Adlbrecht
1   Medical University of Vienna, Departments of Cardiology, Medical University of Vienna, Vienna, Austria
,
Michael Schemper
2   Department of Medical Computer Sciences; Medical University of Vienna, Vienna, Austria
,
Paul A. Kyrle
3   Department of Hematology; Medical University of Vienna, Vienna, Austria
,
Verena Schönauer
3   Department of Hematology; Medical University of Vienna, Vienna, Austria
,
Markus Exner
4   Department of Laboratory Medicine; Medical University of Vienna, Vienna, Austria
,
Walter Klepetko
5   Department of Cardiothoracic Surgery; Medical University of Vienna, Vienna, Austria
,
Meinhard P. Kneussl
6   Department of Pulmonary Medicine; Medical University of Vienna, Vienna, Austria
,
Gerald Maurer
1   Medical University of Vienna, Departments of Cardiology, Medical University of Vienna, Vienna, Austria
,
Irene Lang
1   Medical University of Vienna, Departments of Cardiology, Medical University of Vienna, Vienna, Austria
› Author Affiliations
Grant support: This research was supported by the Austrian fellowship grant FWF P13834-MED (to IML), the österreichischer Selbsthilfeverein Lungenhochdruck and the Ludwig Boltzmann Institutes for Cardiovascular Research and Thrombosis Research.
Further Information

Publication History

Received 10 October 2004

Accepted after revision 28 February 2004

Publication Date:
14 December 2017 (online)

Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by organized thromboemboli that obstruct the pulmonary vascular bed. Although CTEPH is a serious complication of acute symptomatic pulmonary embolism in 4% of cases, signs, symptoms and classical risk factors for venous thromboembolism are lacking. The aim of the present study was to identify medical conditions conferring an increased risk of CTEPH. We performed a case-control-study comparing 109 consecutive CTEPH patients to 187 patients with acute pulmonary embolism that was confirmed by a high probability lung scan. Splenectomy (odds ratio=13, 95% CI 2.7–127), ventriculoatrial (VA-) shunt for the treatment of hydrocephalus (odds ratio=13, 95% CI 2.5–129) and chronic inflammatory disorders, such as osteomyelitis and inflammatory bowel disease (IBD, odds ratio=67, 95% CI 7.9–8832) were associated with an increased risk of CTEPH.

 
  • References

  • 1 Pengo V, Lensing AW, Prins MH. et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257-64.
  • 2 Ribeiro A, Lindmarker P, Johnsson H. et al. Pulmonary embolism: one-year follow-up with echocardiography doppler and five-year survival analysis. Circulation 1999; 99: 1325-30.
  • 3 Lang IM. Chronic thromboembolic pulmonary hypertension-- not so rare after all. N Engl J Med 2004; 350: 2236-8.
  • 4 Wolf M, Boyer-Neumann C, Parent F. et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J 2000; 15: 395-9.
  • 5 Fedullo PF, Auger WR, Kerr KM. et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345: 1465-72.
  • 6 Bonderman D, Turecek PL, Jakowitsch J. et al. High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. Thromb Haemost 2003; 90: 372-6.
  • 7 Cappellini MD, Robbiolo L, Bottasso BM. et al. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol 2000; 111: 467-73.
  • 8 Stewart GW, Amess JA, Eber SW. et al. Thromboembolic disease after splenectomy for hereditary stomatocytosis. Br J Haematol 1996; 93: 303-10.
  • 9 Chou R, DeLoughery TG. Recurrent thromboembolic disease following splenectomy for pyruvate kinase deficiency. Am J Hematol 2001; 67: 197-9.
  • 10 Favara BE, Paul RN. Thromboembolism and cor pulmonale complicating ventriculovenous shunt. JAMA 1967; 199: 668-71.
  • 11 Unnithan RR, Bahuleyan CG, Sambasivan M. et al. Ventriculo-atrial shunt producing pulmonary hypertension. J Assoc Physicians India 1984; 32: 1000-1.
  • 12 Trowitzsch E, Ostrejz M, Evers D. et al. Echocardiographic proof of pulmonary hypertension with irreversible increased resistance in the pulmonary circulation as a complication after placement of a ventriculo- atrial shunt for internal hydrocephalus. Eur J Pediatr Surg 1992; 2: 361-4.
  • 13 Haasnoot K, van Vught AJ. Pulmonary hypertension complicating a ventriculo-atrial shunt. Eur J Pediatr 1992; 151: 748-50.
  • 14 Drucker MH, Vanek VW, Franco AA. et al. Thromboembolic complications of ventriculoatrial shunts. Surg Neurol 1984; 22: 444-8.
  • 15 Pascual JM, Prakash UB. Development of pulmonary hypertension after placement of a ventriculoatrial shunt. Mayo Clin Proc 1993; 68: 1177-82.
  • 16 Rao PS, Molthan ME, Lipow HW. Cor pulmonale as a complication of ventriculoatrial shunts. Case report. J Neurosurg 1970; 33: 221-5.
  • 17 Ralston DR, St John RC. Progressive shortness of breath in a 50-year-old man with ulcerative colitis. Chest 1996; 110: 1608-10.
  • 18 Schlesselmann JJ. Case control studies: design, conduct, analysis. In: Press OU, ed; New York, Oxford: 1982
  • 19 Jamieson SW. Pulmonary thromboendarterectomy [editorial]. Heart 1998; 79: 118-20.
  • 20 Thistlethwaite PA, Mo M, Madani MM. et al. Operative classification of thromboembolic disease determines outcome after pulmonary endarterectomy. J Thorac Cardiovasc Surg 2002; 124: 1203-11.
  • 21 Seligsohn U, Lubetsky A. Genetic susceptibility to venous thrombosis. N Engl J Med 2001; 344: 1222-31.
  • 22 Value of the ventilation/perfusion scan in acute pulmonary embolism Results of the prospective investigation of pulmonary embolism diagnosis (PIOPED). The PIOPED Investigators. JAMA 1990; 263: 2753-9.
  • 23 Lennard-Jones J, Shivananda S. Clinical uniformity of inflammatory bowel disease at presentation and during the first year of disease in the north and south of Europe. Eur J Gastroenterol Hepatol 1997; 9: 353-9.
  • 24 Freeman HJ. Osteomyelitis and osteonecrosis in inflammatory bowel disease. Can J Gastroenterol 1997; 11: 601-6.
  • 25 Heinze G, Schemper M. A solution to the problem of separation in logistic regression. Stat Med 2002; 21: 2409-19.
  • 26 Heinze G, Ploner M. Fixing the nonconvergence bug in logistic regression with SPLUS and SAS. Comput Methods Programs Biomed 2003; 71: 181-7.
  • 27 Miyamoto S, Nagaya N, Satoh T. et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000; 161: 487-92.
  • 28 Hoeper MM, Niedermeyer J, Hoffmeyer F. et al. Pulmonary hypertension after splenectomy?. Ann Intern Med 1999; 130: 506-9.
  • 29 Moser KM, Auger WR, Fedullo PF. Chronic majorvessel thromboembolic pulmonary hypertension. Circulation 1990; 81: 1735-43.
  • 30 Schoenbaum SC, Gardner P, Shillito J. Infections of cerebrospinal fluid shunts: epidemiology, clinical manifestations, and therapy. J Infect Dis 1975; 131: 543-52.
  • 31 Ejstrud P, Kristensen B, Hansen JB. et al. Risk and patterns of bacteraemia after splenectomy: a population- based study. Scand J Infect Dis 2000; 32: 521-5.
  • 32 Olman MA, Marsh JJ, Lang IM. et al. Endogenous fibrinolytic system in chronic large-vessel thromboembolic pulmonary hypertension. Circulation 1992; 86: 1241-8.
  • 33 Lang IM, Marsh JJ, Olman MA. et al. Parallel analysis of tissue-type plasminogen activator and type 1 plasminogen activator inhibitor in plasma and endothelial cells derived from patients with chronic pulmonary thromboemboli. Circulation 1994; 90: 706-12.
  • 34 Lang IM, Marsh JJ, Olman MA. et al. Expression of type 1 plasminogen activator inhibitor in chronic pulmonary thromboemboli. Circulation 1994; 89: 2715-21.
  • 35 Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81.
  • 36 Abenhaim L, Moride Y, Brenot F. et al. Appetitesuppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 335: 609-16.
  • 37 Humbert M, Nunes H, Sitbon O. et al. Risk factors for pulmonary arterial hypertension. Clin Chest Med 2001; 22: 459-75.
  • 38 McGoon MD. Task force on diagnosis and assessment, Third World Symposium on Pulmonary Arterial Hypertension, Venice. 2003
  • 39 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 (Suppl.) 14S-34S.