Chronic thromboembolic pulmonary hypertension☆
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
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Cited by (16)
Thrombosis and Embolism
2021, Molecular Imaging: Principles and PracticeAcute haemodynamic responses to inhaled nitric oxide and intravenous sildenafil in distal chronic thromboembolic pulmonary hypertension (CTEPH)
2007, Vascular PharmacologyCitation Excerpt :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.
Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties
2006, ChestCitation Excerpt :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
Surrogate markers for adverse outcomes in normotensive patients with pulmonary embolism
2006, Critical Care Medicine
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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.