The role of pharmacotherapy in the treatment of chronic thromboembolic pulmonary hypertension (RCD code: II-1A.5)

Barbara Widlińska, Grzegorz Kopeć, Piotr Podolec

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating disease caused by chronic obstruction of major pulmonary arteries. The median age of patients at diagnosis of CTEPH is 63 years and both genders are equally affected. The symptoms are usually nonspecific, particularly in the early stages of the disease, which significantly delays the diagnosis. All patients with pulmonary hypertension (PH) of the unknown aetiology should be diagnosed for CTEPH, even without the information on pulmonary embolism in the medical history. Ventilation-perfusion scintigraphy of the lungs has the highest sensitivity in the diagnosis of CTEPH. A positive result of scintigraphy suggestive of CTEPH is not enough to diagnose the disease. Right heart catheterization and angiography of the pulmonary arteries definitively confirm the diagnosis. All patients with CTEPH should receive chronic oral anticoagulation using generally vitamin K antagonists (warfarin, acenocumarol). Pulmonary endarterectomy (PEA) is the only effective treatment for eliminating the cause of the disease. However, not all patients can be referred for pulmonary endarterectomy surgery. According to data from the European CTEPH Registry of the years 2007-2009, over 40% of patients is not subjected to the operation mainly due to the distal location of thromboembolic changes. Currently, riociguat is the only approved therapeutic agent for the pharmacological treatment of inoperable/persistent CTEPH. In some cases, Off-lebel use of drugs approved for PAH may be considered in symptomatic patients with inoperable CTEPH/persistent PH after PEA. Recent advances in balloon pulmonary angioplasty make it a promising therapeutic alternative for selected patients with non-operable CTEPH. JRCD 2015; 2 (2): 4–8


riociguat; pulmonary endarterectomy; pulmonary embolism


Wytyczne ESC dotyczące rozpoznawania i leczenia nadciśnienia płucnego 2009.

Simonneau G, Gatzoulis MA, Adatia I et all: Updated Clinical Classification of Pulmonary Hypertension. JACC 2013; 62: 25, 34–41.

Wieteska M, Biederman A, Torbicki A. Chronic thromboembolic pulmonary hypertension: epidemiology, pathogenesis and natural history. Hematologia 2010; 1: 136–141.

Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001; 345: 1465–1472.

Fijalkowska A, Biderman A. Przewlekłe zakrzepowo-zatorowe nadciśnienie płucne. Kardiologia po Dyplomie 2012; 11: 34–40.

Dartevelle P, Fadel E, Mussot S et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004; 23: 637–648.

Karen M. Olsson, Bernhard Meyer, Jan Hinrichs, Jens Vogel-Claussen, Marius M. Hoeper, Serghei Cebotari. Chronic Thrombembolic pulmonary hypertension.

Marius M. Hoeper, Pharmacological therapy for patients with chronic thromboembolic pulmonary hypertension, Marius M. Hoeper

Egermayer P, Peacock AJ. Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J. 2000; 15: 440–448.

Owen WR, Thomas WA, Castelman B, Bland EF. Unrecognized emboli to the lungs with subsequent cor pulmonale. N Engl J Med. 1953; 249: 919–926.

Cabrol S, Souza R, Jais X et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2007; 26: 357–362.

Suntharalingam J, Treacy CM, Doughty NJ et al. Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest 2008; 134: 229–236.

Jais X, D’Armini AM, Jansa P et al. Bosentan Effects in iNopErable Forms of chronic Thromboembolic pulmonary hypertension Study Group. 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: 2127–2134.

Ghofrani HA, Hoeper MM, Halank M et al. Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study. Eur Respir J. 2010; 36: 792–799.

Ghofrani HA, Hoeper MM, Halank M et al. Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study. N Engl J Med. 2013; 369: 2268.

Olschewski H, Simonneau G, Galie N et al. Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002; 347: 322–329.

Skoro-Sajer N, Bonderman D, Wiesbauer F, et al. Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2007; 5: 483–489.

Hoeper MM, Kramm T, Wilkens H et al. Bosentan Therapy for Inoperable Chronic Thromboembolic Pulmonary Hypertension. Chest 2005; 128: 2363–2367.

Lang IM, Plank C, Sadushi-Kolici R et al. Imaging in pulmonary hypertension. JACC Cardiovasc Imaging 2010; 3: 1287–1295.

Konstantinides SV, Torbicki A, Agnelli G et al. Task Force for the Diagnosis and Management of Acute Pulmonary Embolism of the European Society of Cardiology (ESC). Eur Heart J. 2014; 35: 3033–3069.

Kopeć G, Waligóra M, Stępniewski J et al. In vivo characterization of changes in composition of organized thrombus in patient with chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty. International Journal of Cardiology 2015; 186: 279–281.



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