Chronic major-vessel thromboembolic pulmonary hypertension.

Moser, Kenneth Μ.; Auger, William R.; Fedullo, Peter F.

doi:10.1161/01.cir.81.6.1735

Cited by 417 publications

(240 citation statements)

References 45 publications

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“…In CTEPH, a reduction in TL,CO has been described [12,13]. In patients with CTEPH, BERNSTEIN et al [13] observed a reduction in TL,CO, caused by a reduction in pulmonary membrane diffusion capacity (Dm) and, to a lesser extent, a reduction in pulmonary capillary blood volume (Vc).…”

mentioning

confidence: 99%

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Steenhuis¹,

Groen²,

Koeter³

et al. 2000

Eur Respir J

100

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The transfer factor of the lung for carbon monoxide (TL,CO) is decreased in patients with pulmonary hypertension. The pulmonary membrane diffusion capacity (Dm) and pulmonary capillary blood volume (Vc), were studied to establish: 1) the relative contribution of the components of the transfer factor to the decrease in TL,CO; 2) whether differences exist between primary pulmonary hypertension (PPH) and chronic thromboembolic pulmonary hypertension (CTEPH); and 3) the relationship between these parameters and haemodynamic parameters.Dm and Vc were determined in 19 patients with PPH and in eight patients with CTEPH. The patients had been referred for consideration for lung transplantation. Haemodynamic parameters were assessed by heart catheterization.In the PPH group, Vc was reduced in 12 of 19 patients (mean SD Vc 72 14% of the predicted value) and Dm in 17 of 19 patients (60 22% pred). In the CTEPH group, Vc was reduced in six of eight patients and Dm in seven of eight patients. The mean TL,CO Dm and Vc values were similar to those in the PPH group.The reduction in pulmonary membrane diffusion capacity was significantly greater than that in pulmonary capillary blood volume. No differences in pulmonary and cardiovascular functional values were found between the groups. Right atrial pressure showed a significant negative correlation with pulmonary capillary blood volume and an increased pulmonary vascular resistance was associated with a decrease in pulmonary membrane diffusion capacity. These results suggest pronounced functional impairment of the alveolocapillary membrane in these patients. Eur Respir J 2000; 16: 276±281.

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mentioning

confidence: 99%

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Steenhuis¹,

Groen²,

Koeter³

et al. 2000

Eur Respir J

100

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“…Most patients with severe CTEPH have episodic dyspnoea, and this often raises the possibility of PE. However when such patients have previously had lung scans there are usually no scintigraphic changes to support a diagnosis of recurrence [46].…”

Section: Do Recurrent Episodes Of Major Pulmonary Embolism Cause Chromentioning

confidence: 99%

“…"CTEPH is the result of single or recurrent pulmonary emboli arising from sites of venous thrombosis" [9]. The term "chronic PE" is also frequently employed as a synonym for CTEPH although the more descriptive term "chronic major vessel thromboembolic PH" may be preferable, provided it is understood that the term "thromboembolism" may also include cases of pulmonary artery thrombosis.…”

mentioning

confidence: 99%

Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis

Egermayer¹,

Peacock²

2000

Eur Respir J

151

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Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. P Egermayer, A.J. Peacock. #ERS Journals Ltd 2000. ABSTRACT: The hypothesis that chronic thromboembolic pulmonary hypertension results from unresolved pulmonary embolism has strongly influenced the diagnosis and management of this disease since the 1960s. However, it is nearly impossible to induce chronic pulmonary hypertension in any animal species by means of repeated embolization of thrombotic material. The haemodynamic effects of thrombotic pulmonary embolism of different degrees of magnitude have also been studied in humans and there is little to suggest that chronic pulmonary hypertension is a likely long term outcome. Furthermore many conditions which predispose to venous thromboembolism do not appear to cause thromboembolic pulmonary hypertension. Other arteriopathic and atherosclerotic risk factors, are found in patients with chronic thromboembolic pulmonary hypertension, but not in those with venous thrombosis, suggesting that these may be unrelated conditions.Thrombosis in situ of the pulmonary arteries is common in severe pulmonary hypertension of any cause. Such thrombosis cannot usually be distinguished from pulmonary embolism. It is hypothesized that in situ thrombosis and pulmonary arteriopathy are common causes of vascular occlusion which is usually diagnosed as "chronic thromboembolic pulmonary hypertension" and that venous thromboembolism is unlikely to be a common cause of chronic pulmonary hypertension. It is further hypothesized that pulmonary embolism is seldom the sole cause of "chronic thromboembolic pulmonary hypertension". Eur Respir J 2000; 15: 440±448.

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“…[1][2][3] CTEPH is a rare disease with an estimated annual incidence of 500-2500 patients in USA. 2 It is further rare in Japan, 4 where the prevalence of CTEPH was estimated to be 450 patients, which is about one-tenth of that in the United States (http://www.nanbyou.or.jp/sikkan/081.htm).…”

Section: Introductionmentioning

confidence: 99%

“…It is accepted that continuous influx of venous thrombi mainly leads to the formation and progression of pulmonary thromboembolism, and that deep vein thrombosis (DVT) and/or coagulation factor variants are predisposing factors of CTEPH. [1][2][3] From this point of view, anticoagulants are often prescribed to CTEPH patients, but the effect is not always sufficient for the prevention of disease progression. In addition, more than half of the patients do not show coincidence or apparent history of DVT.…”

Section: Introductionmentioning

confidence: 99%

HLA-DPB1 and NFKBIL1 may confer the susceptibility to chronic thromboembolic pulmonary hypertension in the absence of deep vein thrombosis

et al. 2009

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Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by pulmonary hypertension caused by thromboembolism of the pulmonary artery. Etiology of CTEPH may be heterogeneous and is largely unknown, but genetic factors are considered to be involved in the etiology. It has been reported that deep vein thrombosis (DVT) and/or coagulation factor variants are predisposing factors to CTEPH. However, more than half of the CTEPH patients, especially the Japanese, do not have prior DVT or coagulation abnormality, suggesting that there should be other risk factors for CTEPH. Moreover, there are several reports on the association between CTEPH and human leukocyte antigen (HLA). To further clarify the HLA-linked gene(s) controlling the susceptibility to CTEPH, 160 patients (99 without DVT and 61 with DVT) and 380 healthy controls were analyzed for polymorphisms in 15 microsatellite markers and 5 genes in the HLA region. We found a strong association of HLA markers with the DVT-negative CTEPH, DPB1*0202 (odds ratio (OR)¼5.07, 95% confidence interval (CI)¼2.52-10.19, P¼0.00000075, corrected P-value (Pc)¼0.00014), IKBL-p*03 (OR¼2.33, 95% CI¼1.49-3.66, P¼0.00017, Pc¼0.033) and B*5201 (OR¼2.47, 95% CI¼1.56-3.90, P¼0.000086, Pc¼0.016), whereas no significant association was observed for the DVT-positive CTEPH. The comparison of clinical characteristics of patients stratified by the presence of susceptibility genes implied that the DPB1 gene controlled the severity of the vascular lesion, whereas the IKBL gene (NFKBIL1) was associated with a relatively mild phenotype.

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Chronic major-vessel thromboembolic pulmonary hypertension.

Cited by 417 publications

References 45 publications

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis

HLA-DPB1 and NFKBIL1 may confer the susceptibility to chronic thromboembolic pulmonary hypertension in the absence of deep vein thrombosis

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