Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) has been estimated to occur in 0.1–0.5% of patients who survive a pulmonary embolism (PE), but more recent prospective studies suggest that its incidence may be much higher. The absence of initial haemodynamic evaluation at the time of PE should explain this discrepancy. We performed a prospective multicentre study including patients with PE in order to assess the prevalence and to describe risk factors of CTEPH. Follow-up every year included an evaluation of dyspnea and echocardiography using a predefined algorithm. In case of suspected CTEPH, the diagnosis was confirmed using right heart catheterisation (RHC). Signs of CTEPH were searched on the multidetector computed tomography (CT) and echocardiography performed at the time of PE. Of the 146 patients analysed, eight patients (5.4%) had suspected CTEPH during a median follow-up of 26 months. CTEPH was confirmed using RHC in seven cases (4.8%; 95%CI, 2.3 – 9.6) and ruled-out in one. Patients with CTEPH were older, had more frequently previous venous thromboembolic events and more proximal PE than those without CTEPH. At the time of PE diagnosis, patients with CTEPH had a higher systolic pulmonary artery pressure and at least two signs of CTEPH on the initial CT. After acute PE, the prevalence of CTEPH appears high. However, initial echocardiography and CT data at the time of the index PE suggest that a majority of patients with CTEPH had previously unknown pulmonary hypertension, indicating that a first clinical presentation of CTEPH may mimic acute PE.

Keywords

Pulmonary embolism - chronic thromboembolic pulmonary hypertension - epidemiology - echocardiography

• References

• 1 Galie N, Hoeper MM, Humbert M. et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. The task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Respir J 2009; 34: 1219-1263.
• 2 Lang IM, Simonneau G, Pepke-Zaba JW. et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study. Thromb Haemost 2013; 110: 83-91.
• 3 Lang IM. Chronic thromboembolic pulmonary hypertension--not so rare after all. N Engl J Med 2004; 350: 2236-2238.
• 4 Fedullo PF, Auger WR, Kerr KM. et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345: 1465-1472.
• 5 Pengo V, Lensing AW, Prins MH. et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257-2264.
• 6 Hachulla E, Gressin V, Guillevin L. et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicentre study. Arthritis Rheum 2005; 52: 3792-3800.
• 7 Sitbon O, Lascoux-Combe C, Delfraissy JF. et al. Prevalence of HIV-Related Pulmonary Arterial Hypertension in the Current Antiretroviral Therapy Era. Am J Respir Crit Care Med 2008; 177: 108-113.
• 8 Jaff MR, McMurtry MS, Archer SL. et al. Management of Massive and Submassive Pulmonary Embolism, Iliofemoral Deep Vein Thrombosis, and Chronic Thromboembolic Pulmonary Hypertension: A Scientific Statement From the American Heart Association. Circulation 2011; 123: 1788-1830.
• 9 Fedullo P, Kerr KM, Kim NH. et al. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2011; 183: 1605-1613.
• 10 Reichelt A, Hoeper MM, Galanski M. et al. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur J Radiol 2009; 71: 49-54.
• 11 Revel MP, Petrover D, Hernigou A. et al. Diagnosing Pulmonary Embolism with Four-Detector Row Helical CT: Prospective Evaluation of 216 Outpatients and Inpatients. Radiology 2005; 234: 265-273.
• 12 Becattini C, Agnelli G, Pesavento R. et al. Incidence of chronic thromboembolic pulmonary hypertension after a first episode of pulmonary embolism. Chest 2006; 130: 172-175.
• 13 Dentali F, Donadini M, Gianni M. et al. Incidence of chronic pulmonary hypertension in patients with previous pulmonary embolism. Thromb Res 2009; 124: 256-258.
• 14 Klok FA, van Kralingen KW, van Dijk AP. et al. Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica 2010; 95: 970-975.
• 15 Korkmaz A, Ozlu T, Ozsu S. et al. Long-term outcomes in acute pulmonary thromboembolism: the incidence of chronic thromboembolic pulmonary hypertension and associated risk factors. Clin Appl Thromb Haemost 2012; 18: 281-288.
• 16 Miniati M, Monti S, Bottai M. et al. Survival and restoration of pulmonary perfusion in a long-term follow-up of patients after acute pulmonary embolism. Medicine 2006; 85: 253-262.
• 17 Poli D, Grifoni E, Antonucci E. et al. Incidence of recurrent venous thromboembolism and of chronic thromboembolic pulmonary hypertension in patients after a first episode of pulmonary embolism. J Thromb Thrombolysis 2010; 30: 294-299.
• 18 Surie S, Gibson NS, Gerdes VE. et al. Active search for chronic thromboembolic pulmonary hypertension does not appear indicated after acute pulmonary embolism. Thromb Res 2010; 125: e202-205.
• 19 Parent F, Bachir D, Inamo J. et al. A haemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011; 365: 44-53.
• 20 Hoeper MM, Mayer E, Simonneau G. et al. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113: 2011-2020.
• 21 McDonald IG, Hirsh J, Hale GS. et al. Major pulmonary embolism, a correlation of clinical findings, haemodynamics, pulmonary angiography, and pathological physiology. Br Heart J 1972; 34: 356-364.
• 22 McIntyre KM, Sasahara AA. The haemodynamic response to pulmonary embolism in patients without prior cardiopulmonary disease. Am J Cardiol 1971; 28: 288-294.
• 23 Pepke-Zaba J, Delcroix M, Lang I. et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124: 1973-1981.
• 24 Sanchez O, Helley D, Couchon S. et al. Perfusion defects after pulmonary embolism: risk factors and clinical significance. J Thromb Haemost 2010; 08: 1248-1255.
• 25 Morris TA, Marsh JJ, Chiles PG. et al. Fibrin derived from patients with chronic thromboembolic pulmonary hypertension is resistant to lysis. Am J Respir Crit Care Med 2006; 173: 1270-1275.
• 26 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: 1325-1330.