Risk factors detection in chronic thromboembolic pulmonary hypertension, a tool for risk quantification?

Abstract

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic thromboembolic obstruction in the pulmonary bed. The defi nitive pathogenesis remains incompletely explained, although multiple risk factors of CTEPH have been identifi ed. The purpose of the study was to evaluate the risk profi le of patients with CTEPH and the representativeness of risk factors, identify possible new CTEPH risk factors and specify the epidemiology of CTEPH in our country. METHODS: In 81 patients with CTEPH, well-known risk factors were analyzed, and a detailed analysis of selected hematological parameters was investigated at a specialized hematology laboratory. RESULTS: CTEPH risk factors were identifi ed as follows: pulmonary embolism (PE), deep vein thrombosis (DVT), thyreopathy, blood type other than "0", infl ammatory bowel disease, malignancy, splenectomy, pacemaker. When compared to healthy controls, the following was observed: a signifi cant decrease in platelet count, higher mean platelet volume, higher spontaneous platelet aggregation, increase in von Willebrand factor and fi brinogen. The median of risk factors representativeness was 3 (PE, DVT, blood type other than "0"). The prevalence of CTEPH in adult population in our country is estimated to be 1.8 per 100,000 inhabitants. CONCLUSION: In the study we confi rmed multiple established risk factors of CTEPH, set their representativeness, identifi ed some platelet abnormalities which could be a potential new risk marker and specifi ed the prevalence of CTEPH (Tab. 5, Ref. 35). Text in PDF www.elis.sk.

Risk factors for chronic thromboembolic pulmonary hypertension – Importance of thyroid disease and function

“…Based on laboratory measurements, further 4.8% of patients were identified with thyroid dysfunction resulting in a total proportion of 28.9% of patients with either thyroid disease or dysfunction. The observed prevalence of CTEPH patients receiving THR therapy was comparable to previous studies (ranging from 8.4% to 19.9%; [11,13,19,20,32]). Interestingly, hypothyroidism was independently associated with a diagnosis of CTEPH during followup (OR 4.3, 95% CI 1.4-13) in 772 patients with acute PE [12].…”

“…Furthermore, in a retrospective study comparing 433 CTEPH patients with 254 patients with pulmonary arterial hypertension, blood group non-0 was associated with CTEPH (OR 2.0, 95% CI 1.1-3.9) [10]. In the present study, 80.7% of patients with CTEPH had blood group non-0 -a slightly higher prevalence than previously reported (ranging from 67.3 to 76.0%; [11,19,21]) and considerably higher compared to the reported prevalence of 59.0% of blood group non-0 in the general population in Germany.…”

“…In the present study, 86.0% of patients with operable CTEPH reported a history of symptomatic PE. Of note, previous studies observed a lower prevalence ranging from 35.8% to 79.0% [11,[18][19][20][21]. For example, in the international CTEPH registry, a history of acute PE was reported by 74.8% of the 678 CTEPH patients included between 02/2007 and 01/2009 with a higher incidence in operable (77.5%) compared to non-operable (70.0%) patients [11].…”

Regulation of VWF (Von Willebrand Factor) in Inflammatory Thrombosis

“…40 Furthermore, 77% of patients with CTEPH were found to have non-O blood groups, which may be associated with increased VWF levels. 109 Although venous thromboembolism populations are also associated with elevated VWF levels which are for non-O blood groups, 110 VWF levels were higher in CTEPH relative to acute PE. 104…”

Association between splenectomy and chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis

“…The majority of patients included were from Europe and there was an equal distribution between genders. All trials reported the prevalence of splenectomy in patients with CTEPH, but three observational studies [23][24][25] did not report the incidence of splenectomy between the CTEPH group and the PAH or thromboembolism disease (venous thromboembolism or pulmonary embolism) group, so only the prevalence of splenectomy in patients with CTEPH was included. Only two trials reported the causes of splenectomies.…”