Elsevier

Cardiology Clinics

Volume 22, Issue 3, August 2004, Pages 453-466
Cardiology Clinics

Review article
Chronic thromboembolic pulmonary hypertension

https://doi.org/10.1016/j.ccl.2004.04.006Get rights and content

Section snippets

Natural history

The pathophysiologic events leading to CTEPH are not entirely understood. Contributing to the gaps in the understanding of this process has been the typical presentation of patients late in the course of the disease, often without a history of a venous thromboembolic event or events. Consequently, a retrospective piecing together of historical and clinical clues, along with a knowledge of the natural history of acute thromboembolic events, has by necessity become the foundation of the

Clinical presentation and patient evaluation

At initial presentation, CTEPH patients most commonly complain of exertional dyspnea and an unexpected decline in exercise capabilities. Although individual tolerances vary, the physiologic basis for these complaints relates to limitations in cardiac performance caused by an elevated pulmonary vascular resistance and increased minute ventilatory needs from an elevated alveolar dead space. Patients can experience a nonproductive cough, especially with exertion. Hemoptysis occurs rarely and is

Surgical selection and preoperative management

Patients with suspected chronic thromboembolic pulmonary hypertension undergo evaluation with the goals of establishing the need for surgical intervention, determining the surgical accessibility of the chronic thromboemboli, and estimating the risk of surgery and anticipated hemodynamic outcome for the individual patient. Most patients who ultimately go on to surgery exhibit a pulmonary vascular resistance greater than 300 dynes/second/cm−5. At centers reporting their experience with pulmonary

Pulmonary thromboendarterectomy

Because the details of pulmonary thromboendarterectomy are reviewed by Thistlethwaite and Jamieson in an accompanying article in this issue and in other publications [20], [83], [84], only selected features of the operation are highlighted here. Surgical success requires a true endarterectomy to remove the organized thrombi, not an embolectomy. The chronic thromboembolic material is fibrotic and is incorporated into the native vascular wall. An endarterectomy involves identification of the

Outcome following pulmonary thromboendarterectomy

Meticulous management of patients following pulmonary thromboendarterectomy and an understanding of the physiologic changes that occur postoperatively are essential to achieving successful outcomes [87]. In addition to complications seen in other forms of cardiac surgery involving cardiopulmonary bypass, such as arrhythmias, coagulation disorders, wound infections, delirium, and nosocomial pneumonia, postendarterectomy patients often experience two unique problems that adversely effect

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      Similarly, Pengo and colleagues49 found a cumulative incidence of symptomatic CTEPH of 3.8% after 2 years in patients with an acute episode of PE. Instead of thrombolysis, the thromboembolic material follows an aberrant path of organization and recanalization, leading to characteristic abnormalities such as intraluminal webs and bands, pouchlike endings of arteries, irregularities of the arterial wall, and stenotic lesions (Figs. 5 and 6).50–52 This aberrant path of obstruction and reopening occurs in repeated cycles over many years.

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