title: Chronic thromboembolic pulmonary arterial hypertension: a review of the literature and novel therapeutic approaches.
abstract: Chronic thromboembolic pulmonary hypertension is defined as pulmonary hypertension (PH) caused by single or recurrent pulmonary emboli and is characterized by chronic obstruction of the pulmonary arteries leading to increased vascular resistance and PH. Also, progressive remodeling may occur in occluded and nonoccluded territories. Better understanding of the underlying mechanisms and risk factors could improve diagnosis and allow appropriate interventions. Pulmonary endarterectomy is an established approach and is considered the definitive treatment for chronic PH, resulting from thromboembolic disease. Furthermore, percutaneous transluminal pulmonary angioplasty is technically feasible, especially for those with peripheral-type of the disease. In addition, several agents, including prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors, have been tested in selected patients yielding promising results. Several novel agents are under investigation, and extensive research is currently in progress aiming to resolve uncertainties in the understanding and treatment of the disease.
authors.affiliation: Department of Cardiology, Royal Free Hospital, Royal Free London NHS Foundation Trust, Pond Street, London, NW3 2QG, London, UK.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Androulakis
authors.forename: Eirini
authors.initials: E
authors.lastname: Lioudaki
authors.forename: Theodoros
authors.initials: T
authors.lastname: Christophides
authors.forename: Mahmood
authors.initials: M
authors.lastname: Ahmad
authors.forename: Hossam
authors.initials: H
authors.lastname: Fayed
authors.forename: Nabila
authors.initials: N
authors.lastname: Laskar
authors.forename: Benjamin
authors.initials: B
authors.lastname: Schreiber
mesh_terms: D017130:Angioplasty; D004691:Endarterectomy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D011655:Pulmonary Embolism / Q000150:complications* / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome
keywords: endarterectomy; medical treatment; percutaneous transluminal pulmonary angioplasty; pulmonary hypertension; thromboembolic disease