title: Chronic thromboembolic pulmonary hypertension.
abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening complication of acute pulmonary embolism. CTEPH results from persistent obstruction of pulmonary arteries and progressive vascular remodelling. Not all patients presenting with CTEPH have a history of clinically overt pulmonary embolism. The diagnostic work-up to detect or rule out CTEPH should include ventilation-perfusion scintigraphy, which has high sensitivity and a negative predictive value of nearly 100%. CT angiography usually reveals typical features of CTEPH, including mosaic perfusion, part or complete occlusion of pulmonary arteries, and intraluminal bands and webs. Patients with suspected CTEPH should be referred to a specialist centre for right-heart catheterisation and pulmonary angiography. Surgical pulmonary endarterectomy remains the treatment of choice for CTEPH and is associated with excellent long-term results and a high probability of cure. For patients with inoperable CTEPH, various medical and interventional therapies are being developed.
authors.affiliation: Department of Respiratory Medicine, Hannover Medical School and German Centre for Lung Research (DZL), Hannover, Germany. Electronic address: hoeper.marius@mh-hannover.de.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.affiliation: Department of Cardiothoracic Surgery, University of California, San Diego, CA, USA.
authors.forename: Michael M
authors.initials: MM
authors.lastname: Madani
authors.affiliation: Department of Cardiovascular Medicine, National Cardiovascular Centre, Osaka, Japan.
authors.forename: Norifumi
authors.initials: N
authors.lastname: Nakanishi
authors.affiliation: Department of Radiology, Hannover Medical School, Hannover, Germany.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Meyer
authors.affiliation: Department of Cardiovascular, Thoracic and Transplantation Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Serghei
authors.initials: S
authors.lastname: Cebotari
authors.affiliation: Department of Respiratory Medicine, University of California, San Diego, CA, USA.
authors.forename: Lewis J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000800:Angioplasty, Balloon; D000077300:Bosentan; D002908:Chronic Disease; D004691:Endarterectomy; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D020536:Enzyme Activators / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011655:Pulmonary Embolism / Q000150:complications*; D011687:Purines / Q000627:therapeutic use; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013923:Thromboembolism / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*
chemical_list: D065128:Endothelin Receptor Antagonists; D020536:Enzyme Activators; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D011720:Pyrazoles; D011743:Pyrimidines; D013449:Sulfonamides; D000068677:Sildenafil Citrate; D000077300:Bosentan; C542595:riociguat