The description of organized thrombus in major pulmonary arteries can be found in autopsy reports dating back to the late nineteenth and early twentieth centuries. Not until the 1950s was the antemortem diagnosis and clinical syndrome of chronic thrombotic obstruction of the major pulmonary arteries better characterized. The first surgical attempt to remove the adherent thrombus from the vessel wall occurred in 1958. This operation provided the conceptual foundation for the distinction between acute and chronic thromboembolic disease of the pulmonary vascular bed, and established that an endarterectomy, and not an embolectomy, would be necessary if a surgical remedy for this disease was to be successful.

During the past 2 decades, there has been a steady rise in the number of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing surgery and in the number of programs worldwide dedicated to the diagnosis and management of this patient population. This article discusses the natural history and clinical presentation of CTEPH, the evaluation of patients for pulmonary thromboendarterectomy, and the outcomes following surgery, along with a brief review of the procedure as performed at the University of California, San Diego.

The last 4 decades have witnessed dramatic changes in the treatment of patients with pulmonary hypertension. And with the advances in cardiothoracic surgical techniques, selected patients with chronic thromboembolic pulmonary hypertension (CTEPH) are afforded a surgical remedy for their disease. In the vast majority of these patients, surgical endarterectomy of chronic thromboemboli from the pulmonary vascular bed effectively treats even severe pulmonary hypertension, and as a result, the debilitating symptoms of right heart dysfunction. This article reviews the natural history of chronic thromboembolic disease and outline a suggested diagnostic approach to determine whether a patient with chronic thromboembolic disease might be an appropriate surgical candidate. Pulmonary thromboendarterectomy surgery, and the growing information on the use of pharmacotherapy in inoperable CTEPH, are also reviewed.

Chronic thromboembolic pulmonary hypertension is one of the few forms of pulmonary hypertension that is surgically curable. It is likely underdiagnosed and must be considered in every patient presenting with pulmonary hypertension to avoid missing the opportunity to cure these patients. This article discusses the epidemiology, risk factors, natural history, diagnosis, and preoperative evaluation of patients with this disorder. Also covered are putative mechanisms for the conversion of acute emboli into fibrosed thrombembolic residua. Mechanical obstruction of the central pulmonary vasculature is rarely the sole cause of the pulmonary hypertension, and a discussion of the small vessel arteriopathy present in these patients is offered. Technical aspects of pulmonary endartectomy and the data supporting its role are discussed, as are the limited data on pulmonary arterial hypertension specific medical therapies for patients deemed noncandidates for the operation.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a result of an alternate natural history in which there is limited resolution of thromboemboli with pulmonary artery obstruction leading to pulmonary hypertension (PH). CTEPH requires a thorough clinical assessment including pulmonary hemodynamics and radiologic evaluation in addition to consultation with an expert center. Surgical intervention remains the optimal management strategy. Select patients may be candidates for catheter-based intervention with balloon pulmonary angioplasty in centers with clinical expertise. Inoperable patients or those with post-intervention PH are treated with pulmonary hypertension-targeted medical therapy.