C hronic thromboembolic pulmonary hypertension (CTEPH) has emerged as one of the leading causes of severe pulmonary hypertension. The disease is notoriously underdiagnosed, and the true prevalence is still unclear. CTEPH is characterized by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries. 1 The consequence is an increased pulmonary vascular resistance resulting in pulmonary hypertension and progressive right heart failure. Vascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for patients with CTEPH, 2 but not all patients are eligible for surgery. Recent research has provided evidence suggesting that the mechanistic view of CTEPH as a disease caused solely by obliteration of central pulmonary arteries due to organized thrombi may have been too simplistic. Pulmonary embolism, either as a single episode or as recurrent episodes, is thought to be the initiating event followed by progressive pulmonary vascular remodeling. This concept explains the clinical observation that CTEPH patients may have severe pulmonary hypertension out of proportion to the pulmonary vascular obliteration seen on a pulmonary angiogram. Thus, treatment of CTEPH often requires a multidisciplinary approach and may involve surgery, medical treatment, or both. However, many aspects of the pathogenesis of CTEPH are poorly understood, the diagnostic approach to these patients has not been standardized, and neither randomized controlled trials nor guidelines have been published in this field.In July 2005, a group of international experts met in Zurich, Switzerland, for a comprehensive discussion of CTEPH. The members of this group are listed in the Appendix (in the online-only Data Supplement). This report will review the issues surrounding CTEPH that were discussed during that meeting: its etiology and pathogenesis, epidemiology, diagnosis, treatment options, and associated prognosis. Current issues, including challenges in assessing surgical candidacy and predicting postoperative outcome, will be introduced. Finally, potential ways of resolving uncertain and controversial issues, such as the role of medical therapy and the requirement for randomized controlled trials, will be discussed. Algorithms for the diagnosis and management of CTEPH will be proposed. EpidemiologyThe true incidence and prevalence of CTEPH are unknown. Originally, it was believed that 0.1% to 0.5% of patients who survive an episode of acute pulmonary embolism develop CTEPH. [3][4][5] However, in a prospective follow-up study of 78 survivors of acute pulmonary embolism, echocardiographic findings suggestive of persistent pulmonary hypertension and/or right ventricular dysfunction were present in 44% of the patients after 1 year. 6 Four patients (5.1%) developed definite CTEPH, and 3 of these subsequently underwent successful PEA. The only identifiable risk factors for persistent pulmonary hypertension were an age Ͼ70 years and a systolic pulmonary artery pressure Ͼ50 mm Hg at the initial p...
C hronic thromboembolic pulmonary hypertension (CTEPH) has emerged as one of the leading causes of severe pulmonary hypertension. The disease is notoriously underdiagnosed, and the true prevalence is still unclear. CTEPH is characterized by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries. 1 The consequence is an increased pulmonary vascular resistance resulting in pulmonary hypertension and progressive right heart failure. Vascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for patients with CTEPH, 2 but not all patients are eligible for surgery. Recent research has provided evidence suggesting that the mechanistic view of CTEPH as a disease caused solely by obliteration of central pulmonary arteries due to organized thrombi may have been too simplistic. Pulmonary embolism, either as a single episode or as recurrent episodes, is thought to be the initiating event followed by progressive pulmonary vascular remodeling. This concept explains the clinical observation that CTEPH patients may have severe pulmonary hypertension out of proportion to the pulmonary vascular obliteration seen on a pulmonary angiogram. Thus, treatment of CTEPH often requires a multidisciplinary approach and may involve surgery, medical treatment, or both. However, many aspects of the pathogenesis of CTEPH are poorly understood, the diagnostic approach to these patients has not been standardized, and neither randomized controlled trials nor guidelines have been published in this field.In July 2005, a group of international experts met in Zurich, Switzerland, for a comprehensive discussion of CTEPH. The members of this group are listed in the Appendix (in the online-only Data Supplement). This report will review the issues surrounding CTEPH that were discussed during that meeting: its etiology and pathogenesis, epidemiology, diagnosis, treatment options, and associated prognosis. Current issues, including challenges in assessing surgical candidacy and predicting postoperative outcome, will be introduced. Finally, potential ways of resolving uncertain and controversial issues, such as the role of medical therapy and the requirement for randomized controlled trials, will be discussed. Algorithms for the diagnosis and management of CTEPH will be proposed. EpidemiologyThe true incidence and prevalence of CTEPH are unknown. Originally, it was believed that 0.1% to 0.5% of patients who survive an episode of acute pulmonary embolism develop CTEPH. [3][4][5] However, in a prospective follow-up study of 78 survivors of acute pulmonary embolism, echocardiographic findings suggestive of persistent pulmonary hypertension and/or right ventricular dysfunction were present in 44% of the patients after 1 year. 6 Four patients (5.1%) developed definite CTEPH, and 3 of these subsequently underwent successful PEA. The only identifiable risk factors for persistent pulmonary hypertension were an age Ͼ70 years and a systolic pulmonary artery pressure Ͼ50 mm Hg at the initial p...
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