Chronic thromboembolic pulmonary hypertension
Keywords:
PULMONARY HYPERTENSION, THROMBOEMBOLISM, CHRONIC DISEASE
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is an unusual entity that may be observed in up to 4% of patients surviving an acute pulmonary thromboembolism (APTE). It is a disease with a poor prognosis unless there is an early diagnosis and a timely treatment. Paradoxically, it is the only form of pulmonary hypertension that may be cured by means of a pulmonary endarterectomy (PE). It is initially diagnosed by non-invasive methods such as the Doppler echocardiogram and the ventilation/perfusion pulmonary scintigraphy (V/Q). Diagnostic and preoperative assessment is completed with a right cardiac catheterization and a pulmonary angiography. These techniques confirm the chronic thromboembolic pulmonary hypertension and define whether it is possible to perform a pulmonary endarterectomy.
References
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(31) Lewczuk J, Piszko P, Jagas J, Porada A, Wójciak S, Sobkowicz B, et al. Prognostic factors in medically treated patients with chronic pulmonary embolism. Chest 2001; 119(3): 818-23.
(32) McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation 2006; 114(13): 1417-31.
(2) Tapson VF, Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc 2006; 3(7): 564-7.
(3) Wolf M, Boyer-Neumann C, Parent F, Eschwege V, Jaillet H, Meyer D, et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J 2000; 15(2): 395-9.
(4) Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med 2007; 48(5): 680-4.
(5) Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. European Society of Cardiology. 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) andthe European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009; 30(20): 2493-537. Disponible en: http://www.escardio.org/guidelines-surveys/esc-guidelines/GuidelinesDocuments/guidelines-PH-FT.pdf. Consulta: 28 agosto 2009.
(6) Jamieson SW. Pulmonary thromboendarterectomy. Heart 1998; 79(2): 118-20.
(7) Jamieson SW, Kapelanski DP, Sakakibara N, Manecke GR, Thistlethwaite PA, Kerr KM, et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 2003; 76(5): 1457-62.
(8) Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345(20): 1465-72.
(9) Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350(22): 2257-64.
(10) Bonderman D, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Dunkler D, Taghavi S, et al. Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 2007; 115(16): 2153-8.
(11) Bonderman D, Wilkens H, Wakounig S, Schäfers HJ, Jansa P, Lindner J, et al. Lang Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 2009; 33(2): 325-31.
(12) Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113(16): 2011-20.
(13) Bonderman D, Jakowitsch J, Redwan B, Bergmeister H, Renner MK, Panzenböck H, et al. Role for staphylococci in misguided thrombus resolution of chronic thromboembolic pulmonary hypertension. Arterioscler Thromb Vasc Biol 2008; 28(4): 678-84.
(14) Egermayer P, Peacock AJ. Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J 2000; 15(3): 440-8.
(15) Moser KM, Braunwald NS. Successful surgical intervention in severe chronic thromboembolic pulmonary hypertension. Chest 1973; 64(1): 29-35.
(16) Lang IM, Klepetko W. Actualización sobre la hipertensión pulmonar tromboembólica crónica, una enfermedad que a menudo no se detecta. Rev Esp Cardiol 2009; 62(2): 120-5.
(17) Keogh AM, Mayer E, Benza RL, Corris P, Dartevelle PG, Frost AE, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 Suppl): S67-77.
(18) Lorente DS, Macchiarini P. Tratamiento quirúrgico en pacientes con hipertensión pulmonar tromboembólica crónica. Arch Bronconeumol 2009; 45(Supl 6): 30-4.
(19) Corsico AG, D'Armini AM, Cerveri I, Klersy C, Ansaldo E, Niniano R, et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med 2008; 178(4): 419-24.
(20) Kim NH, Fesler P, Channick RN, Knowlton KU, Ben-Yehuda O, Lee SH, et al. Preoperative partitioning of pulmonary vascular resistance correlates with early outcome after thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Circulation 2004; 109(1): 18-22.
(21) Dartevelle P, Fadel E, Mussot S, Chapelier A, Hervé P, de Perrot M, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2004; 23(4): 637-48.
(22) Jais X, D'Armini AM, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronic Thromboembolic pulmonary hypertension), a randomized, placebo controlled trial. J Am Coll Cardiol 2008; 52(25): 2127-34.
(23) Suntharalingam J, Treacy CM, Doughty NJ, Goldsmith K, Soon E, Toshner MR, et al. Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest 2008; 134(2): 229-36.
(24) Bhatia S, Frantz RP, Severson CJ, Durst LA, McGoon MD. Immediate and long-term hemodynamic and clinical effects of sildenafil in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc 2003; 78(10): 1207-13.
(25) Michelakis ED, Tymchak W, Noga M, Webster L, Wu XC, Lien D, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003; 108(17): 2066-9.
(26) Ghofrani HA, Schermuly RT, Rose F, Wiedemann R, Kohstall MG, Kreckel A, et al. Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2003; 167(8): 1139-41.
(27) Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension: the Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334(5): 296-302.
(28) Cabrol S, Souza R, Jais X, Fadel E, Ali RH, Humbert M,et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2007; 26(4): 357-62.
(29) Olschewski H, Simonneau G, Galiè N, Higenbottam T, Naeije R, Rubin LJ, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347(5): 322-9.
(30) Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346(12): 896-903.
(31) Lewczuk J, Piszko P, Jagas J, Porada A, Wójciak S, Sobkowicz B, et al. Prognostic factors in medically treated patients with chronic pulmonary embolism. Chest 2001; 119(3): 818-23.
(32) McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation 2006; 114(13): 1417-31.
Published
2011-09-30
How to Cite
1.
Salisbury JP, Curbelo P, Arcaus M, Cáneva J. Chronic thromboembolic pulmonary hypertension. Rev. Méd. Urug. [Internet]. 2011Sep.30 [cited 2024Nov.25];27(3):166-74. Available from: http://www2.rmu.org.uy/ojsrmu311/index.php/rmu/article/view/385
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