title: Treatment of Chronic Thromboembolic Pulmonary Hypertension: The Role of Medical Therapy and Balloon Pulmonary Angioplasty.
abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable disease when treated with pulmonary thromboendarterectomy (PTE). However, even at experienced surgical centers, nearly one-third of patients with CTEPH will be deemed inoperable for reasons including distal disease, comorbidities, or out-of-proportion pulmonary hypertension. It is in these patients with inoperable CTEPH that pulmonary hypertension (PH)-targeted medical therapy and balloon pulmonary angioplasty have potential therapeutic value. Previous unblinded cohort trials have assessed PH-targeted medical therapy in various subpopulations of CTEPH patients using epoprostenol, treprostinil, sildenafil, bosentan, and iloprost, each demonstrating measurable pulmonary hemodynamic effects. However, riociguat, a soluble guanylate cyclase stimulator, is the first FDA-approved therapy for inoperable CTEPH to demonstrate both an improvement in functional capabilities (6-minute walk time) as well as significant gains in secondary pulmonary hemodynamic end points in a large placebo-controlled trial. Balloon pulmonary angioplasty is an interventional procedure using telescoping catheters placed in the pulmonary arteries, through which wires and balloons are used to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction. Contemporary case series from multiple centers worldwide have demonstrated pulmonary hemodynamic improvement with this approach. As a result of these advances, patients with inoperable CTEPH who had few options as recently as 5 years ago now have alternatives with emerging evidence of therapeutic efficacy.
authors.affiliation: University of California-San Diego, School of Medicine, La Jolla, California.
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Fernandes
authors.affiliation: University of California-San Diego, School of Medicine, La Jolla, California.
authors.forename: David S
authors.initials: DS
authors.lastname: Poch
authors.affiliation: University of California-San Diego, School of Medicine, La Jolla, California.
authors.forename: William R
authors.initials: WR
authors.lastname: Auger
mesh_terms: D000800:Angioplasty, Balloon / Q000009:adverse effects; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D062186:Arterial Pressure / Q000187:drug effects*; D002908:Chronic Disease; D017079:Exercise Tolerance / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D011651:Pulmonary Artery / Q000187:drug effects* / Q000503:physiopathology; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D020127:Recovery of Function; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents
keywords: balloon pulmonary angioplasty; chronic thromboemboli; pulmonary hypertension; pulmonary hypertension targeted medical therapy