Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the pulmonary vasculature, leading to increased pulmonary vascular resistance and ultimately right ventricular failure, the leading cause of death in non-operated patients. This article reviews the current management of CTEPH. The standard of care in CTEPH is pulmonary endarterectomy (PEA). However, up to 40% of patients with CTEPH are ineligible for PEA, and up to 51% develop persistent/recurrent PH after PEA. Riociguat is currently the only medical therapy licensed for treatment of inoperable or persistent/recurrent CTEPH after PEA based on the results of the Phase III CHEST-1 study. Studies of balloon pulmonary angioplasty (BPA) have shown benefits in patients with inoperable or persistent/recurrent CTEPH after PEA; however, data are lacking from large, prospective, controlled studies. Studies of macitentan in patients with inoperable CTEPH and treprostinil in patients with inoperable or persistent/recurrent CTEPH showed positive results. Combination therapy is under evaluation in CTEPH, and long-term data are not available. In the future, CTEPH may be managed by PEA, medical therapy or BPAalone or in combination, according to individual patient needs. Patients should be referred to experienced centers capable of assessing and delivering all options.

Chronic Thromboembolic Pulmonary Hypertension: An Update

“…CTEPH differs from other PH groups also as to the therapeutic options currently available [ 27 , 28 , 29 ]. Pulmonary endarterectomy (PEA) remains the current potentially curative treatment of choice, especially when the organized thrombi involve the main, lobar, or segmental arteries.…”

“…Pulmonary endarterectomy (PEA) remains the current potentially curative treatment of choice, especially when the organized thrombi involve the main, lobar, or segmental arteries. Although this is a complex surgical procedure involving the removal of obstructive thromboembolic material from the pulmonary vessels, a high percentage of patients have enhanced quality and life expectancy, and it may even be considered a definitive cure for many of them [ 27 , 28 , 29 ]. Therefore, distinguishing operable from inoperable patients is of crucial importance.…”

“…Unfortunately, a certain number of CTEPH (30% to 45%) patients are not candidates for surgery, which is the preferred treatment, and, according to literature data, have a worse prognosis than operated patients [ 10 , 12 , 17 , 26 , 27 , 28 , 29 , 30 ]. The exclusion of the possibility of surgical treatment is more often due to the involvement of the distal pulmonary arteries (technically inaccessible), the presence of severe comorbidities (unfavorable risk-benefit ratio), or the patient’s refusal to undergo surgery.…”

End-stage chronic thromboembolic pulmonary hypertension: is there still room for lung transplantation?—the past, the present and the future

“…This procedure is less invasive than PEA as it only requires the cannulation of the femoral or jugular veins. Eligibility criteria for BPA are based on assessment of the anatomy of PA anatomy and function and lung perfusion (6). Regarding medical treatment in CTEPH, current guidelines approved Riociguat, an oral guanylate cyclase stimulator, and Treprostinil, a subcutaneous prostacyclin analogue, for patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension (PH) after PEA (1).…”

“…If all these treatments are not effective, lung transplantation can be considered as a salvage option only in highly selected patients and in highly specialized centers (6,7).…”