Elsevier

Progress in Cardiovascular Diseases

Volume 45, Issue 3, November–December 2002, Pages 203-212
Progress in Cardiovascular Diseases

Chronic thromboembolic pulmonary hypertension

https://doi.org/10.1053/pcad.2002.130159Get rights and content

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) should be differentiated from other etiologies of pulmonary hypertension since surgical intervention may be potentially curative. The presentation of this illness is nonspecific and may mimic other cardiopulmonary disease states. Without treatment, progressive pulmonary hypertension, right heart failure, and death will ensue. Echocardiography, lung ventilation-perfusion scan, right heart catheterization, and angiography are required for proper diagnosis and preoperative assessment. Definitive treatment requires surgical resection of thromboembolic material. The role of medical therapy remains to be defined. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Pathophysiology and natural history

The etiology of CTEPH has been subject to some controversy but is believed to be a consequence of pulmonary emboli that fail to be adequately resorbed with subsequent failure to recanalize the vessel lumen. The embolus reorganizes, with residual obstruction of laminar flow, although full incorporation into the vessel wall has also been described.5 Central pulmonary artery thrombus has been reported in other pulmonary disorders, including PAH, and pulmonary emboli as the source of CTEPH has been

Clinical presentation

With rare exception, it is almost impossible to distinguish CTEPH from other causes of PAH on the basis of symptoms, electrocardiogram (ECG) findings, physical examination, or pulmonary function tests (PFTs).16 Symptoms may mimic other cardiopulmonary disorders such as coronary artery disease, exercise-induced asthma, or even psychogenic dyspnea, resulting in an average delay of 2 to 3 years from the onset of symptoms to the establishment of a correct diagnosis.17 Progressive dyspnea on

Diagnosis

The diagnosis of CTEPH can be elusive and requires a high index of suspicion that leads to the appropriate diagnostic studies. No single test is likely to be diagnostic; rather, a comprehensive evaluation must provide hemodynamic, physiologic, and anatomic details that can be integrated to address 3 key questions: (1) Does pulmonary arterial hypertension exist? (2) Is it caused by chronic thromboembolic material? and (3) Is that material surgically accessible?

As with pulmonary function tests,

Treatment and prognosis

The cornerstone of therapy for CTEPH remains pulmonary thromboendarterectomy (PTE). Candidates for this surgery have significant dyspnea in addition to hemodynamic or ventilatory impairment at rest or with exercise. Pulmonary vascular resistance (PVR) is invariably elevated, usually between 300 to 2,000 dynes/s/cm−5 (dsc-5), averaging 800 to 1,000 dsc-5.36 Pulmonary angiograms should demonstrate at least obstruction of 50% of the pulmonary artery vasculature, with preoperative evidence of

References (62)

  • WR Auger et al.

    Evaluation of patients with suspected chronic thromboembolic pulmonary hypertension

    Semin Thorac Cardiovasc Surg

    (1999)
  • S Kruger et al.

    Diagnosis of pulmonary arterial hypertension and pulmonary embolism with magnetic resonance angiography

    Chest

    (2001)
  • TJ Kroencke et al.

    Three-dimensional gadolinium-enhanced magnetic resonance venography in suspected thrombo-occlusive disease of the central chest veins

    Chest

    (2001)
  • PO Daily et al.

    Modifications of techniques and early results of pulmonary thromboendarterectomy for chronic pulmonary embolism

    J Thorac Cardiovasc Surg

    (1987)
  • RE Wragg et al.

    Operative predictors of delirium after pulmonary thromboendarterectomy. A model for postcardiotomy delirium?

    J Thorac Cardiovasc Surg

    (1988)
  • T Menzel et al.

    Improvement of tricuspid regurgitation after pulmonary thromboendarterectomy

    Ann Thorac Surg

    (2002)
  • E Mayer et al.

    Mid-term results of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension

    Ann Thorac Surg

    (1996)
  • PO Daily et al.

    Historical perspective: Surgery for chronic thromboembolic disease

    Semin Thorac Cardiovasc Surg

    (1999)
  • MA Olman et al.

    Pulmonary vascular steal in chronic thromboembolic pulmonary hypertension

    Chest

    (1990)
  • PF Fedullo et al.

    Postoperative management of the patient undergoing pulmonary thromboendarterectomy

    Semin Thorac Cardiovasc Surg

    (1999)
  • PF Fedullo et al.

    Chronic thromboembolic pulmonary hypertension

    Clin Chest Med

    (1995)
  • M Mo et al.

    Reoperative pulmonary thromboendarterectomy

    Ann Thorac Surg

    (1999)
  • KM Moser et al.

    Chronic major-vessel thromboembolic pulmonary hypertension

    Circulation

    (1990)
  • SW Jamieson et al.

    Pulmonary endarterectomy

    Curr Probl Surg

    (2000)
  • ES Hurwitt et al.

    A surgical approach to the problem of chronic pulmonary artery obstruction due to thrombosis or stenosis

    Ann Surg

    (1958)
  • KM Moser et al.

    Do patients with primary pulmonary hypertension develop extensive central thrombi?

    Circulation

    (1995)
  • E Arbustini et al.

    Plaque composition in plexogenic and thromboembolic pulmonary hypertension: The critical role of thrombotic material in pultaceous core formation

    Heart

    (2002)
  • PF Fedullo et al.

    The natural history of acute and chronic thromboembolic disease: The search for the missing link

    Eur Respir J

    (2000)
  • CC Colorio et al.

    Thrombophilic factors in chronic thromboembolic pulmonary hypertension

    Blood Coagul Fibrinolysis

    (2001)
  • M Wolf et al.

    Thrombotic risk factors in pulmonary hypertension

    Eur Respir J

    (2000)
  • H Kim et al.

    Pulmonary vascular remodeling distal to pulmonary artery ligation is accompanied by upregulation of endothelin receptors and nitric oxide synthase

    Exp Lung Res

    (2000)
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      In patients with predominantly ‘proximal’ disease surgical intervention (pulmonary endarterectomy or PEA) can be very effective (Tscholl et al., 2001; Jamieson et al., 2003). However, when the degree of haemodynamic compromise is out of proportion to the quantity of surgically accessible disease, surgery is often inappropriate (Williamson et al., 2002). In addition, a proportion of surgical patients continue to have pulmonary hypertension post-operatively as a result of residual inaccessible disease.

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      In recent years, a better understanding of the mechanisms responsible for elevated pulmonary arterial pressure and vascular resistance in the different types of PH has led to the suggestion that the thromboembolic and nonthromboembolic types of PH may share common pathophysiologic features.25 This concept is supported by data indicating that the mechanisms leading to PH in patients who have experienced repeated pulmonary thromboembolic events are not only mechanistic (ie, nonrecanalized thrombotic vessel occlusion),26,27 but possibly also are related to vascular remodeling that is located distal to the occluded artery28 and in noninvolved adjacent pulmonary vessels,6 possibly leading to endothelial dysfunction. The vascular lesions in these noninvolved vessel segments were histologically indistinguishable from those in PAH patients.6

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    Address reprint requests to Lewis J. Rubin, MD, Division of Pulmonary and Critical Care Medicine, University of California, San Diego, 9300 Campus Point Drive, La Jolla, CA 92037.

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