P2540Sex-specific differences in the clinical presentation, surgical complications, and course of chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Barco ◽  
F A Klok ◽  
S V Konstantinides ◽  
P Dartevelle ◽  
E Fadel ◽  
...  

Abstract Background Women are more susceptible to develop several forms of pulmonary hypertension, but they may have better survival rates than men. Sparse data are available concerning sex-specific differences in chronic thromboembolic pulmonary hypertension (CTEPH). Purpose and methods We investigated sex-specific differences in the clinical presentation of CTEPH, functional parameters, exposure to pulmonary endarterectomy (PEA), and survival. Results Women constituted half of the study population (N=679 treatment-naïve patients from the European CTEPH registry) and were characterized by a lower prevalence of some cardiovascular risk factors (e.g. prior acute coronary syndrome, smoking habit, chronic obstructive pulmonary disease), but more prevalent obesity, cancer, and thyroid diseases. Median age was 62 (IQR 50–73) years in women and 63 (IQR 53–70) in men. Women underwent PEA less often than men (54% vs 65%; Figure 1, Panel A) and were exposed to fewer additional cardiac procedures, notably coronary artery bypass graft surgery (0.5% vs. 9.5%). The prevalence of specific reasons for not being operated, including the patient's refusal and the proportion of proximal vs. distal lesions, did not differ between sexes. A total of 57 (17.0%) deaths in women and 70 (20.7%) in men were recorded over long-term follow-up. Female sex was positively associated with long-term survival (adjusted Hazard Ratio 0.66; 95% Confidence Interval 0.46–0.94). Short-term mortality was identical in the two groups (Figure 1, Panel B). Conclusions Women with CTEPH had a lower prevalence of cardiovascular risk factors and underwent PEA less frequently than men, who, in turn, were more often exposed to additional major cardiac surgery procedures. Women had more favorable long-term survival. Acknowledgement/Funding The CTEPH registry is supported by a research grant from Actelion Pharmaceuticals Ltd.

2021 ◽  
Vol 25 (3) ◽  
pp. 11
Author(s):  
O. Ya. Vasiltseva ◽  
A. G. Edemskiy ◽  
D. S. Grankin ◽  
E. N. Kliver ◽  
A. M. Chernyavskiy

<p>Chronic thromboembolic pulmonary hypertension is a long-term consequence of acute pulmonary embolism. Gradual obstruction of the pulmonary arteries and secondary changes in the pulmonary microcirculation over time cause progressive increases in pulmonary vascular resistance and pulmonary artery pressure that can result in severe right heart failure. This article provides an overview of pulmonary embolism and chronic thromboembolic pulmonary hypertension scientific literature and national guidelines. We focus on disease and recurrence risk factors and outline future directions of research to improve short- and long-term patient outcomes.</p><p>Received 17 January 2021. Revised 1 March 2021. Accepted 19 April 2021.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> The authors declare no conflicts of interests.</p><p><strong>Contribution of the authors</strong><br />Conception and study design: A.M. Chernyavskiy, A.G. Edemskiy, D.S. Grankin, E.N. Kliver<br />Drafting the article: O.Ya. Vasiltseva<br />Critical revision of the article: E.N. Kliver<br />Final approval of the version to be published: O.Ya. Vasiltseva, A.G. Edemskiy, D.S. Grankin, E.N. Kliver, A.M. Chernyavskiy</p>


2020 ◽  
pp. 089719002096129
Author(s):  
Marianne Kenny ◽  
Megan M. Clarke ◽  
Kristen T. Pogue

Pulmonary hypertension (PH), which includes pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), is a progressive condition with significant morbidity and mortality due to right heart failure if left untreated. Riociguat is a soluble guanylate cyclase (sGC) stimulator and is the only treatment approved for both PAH and CTEPH. The objectives of this review are to describe the epidemiology and pathophysiology of PAH and CTEPH; synthesize the pharmacology, efficacy, safety, and utilization of riociguat; and discuss the role of the pharmacist in managing patients with these conditions. Data presented in this review is supported by peer reviewed literature, using PubMed and key words including pulmonary hypertension, pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and riociguat. The review draws on key studies and review articles that discuss the pathophysiology of PAH and CTEPH, as well as articles discussing the safety and efficacy of riociguat. The overall goal in the treatment of PAH and CTEPH is to improve long-term survival. Treatment planning depends on the type of PH, treatment goals, comorbidities, and risk profiles. Pharmacists serve a valuable role as part of the multidisciplinary team in the care of patients with PH, many of whom may have comorbidities that contribute to high costs and resource utilization. Riociguat is a first-in-class medication and the only approved treatment for both PAH and CTEPH. In clinical trials, riociguat has demonstrated favorable efficacy and tolerability. Riociguat is a valuable addition to the armamentarium of options for treating patients with PH.


2019 ◽  
Vol 70 (1) ◽  
pp. e563
Author(s):  
Anoop Koshy ◽  
Omar Farouque ◽  
Jay Ramchand ◽  
Adam Testro ◽  
Peter Angus ◽  
...  

2017 ◽  
Vol 38 (05) ◽  
pp. 677-685
Author(s):  
Nick Kim ◽  
Micah Fisher

AbstractChronic thromboembolic pulmonary hypertension is a progressive disease characterized by obstruction of the pulmonary vascular bed by insufficient resolution of thromboemboli leading to increased pulmonary vascular resistance with resultant right heart dysfunction and poor long-term survival. Estimates of cumulative incidence after an acute pulmonary embolism range from 1 to 4% within 2 years of an initial event. Current recommendations focus on screening patients being evaluated for pulmonary hypertension with ventilation/perfusion scans and then considering them for potentially curative surgery consisting of a pulmonary endarterectomy. Outcomes at high-volume surgical centers continue to improve and overall perioperative mortality is close to 2 to 4% with excellent reported long-term survival. Unfortunately, adherence with guidelines remains poor and patients with this potentially remediable disease are being missed. In addition, a significant number of patients who are diagnosed are not felt to be operative candidates based on location of disease, hemodynamics, or comorbidities. Several new advances may improve outcomes for such patients who are not operative candidates or who have persistent pulmonary hypertension after surgery, including medical therapy and balloon pulmonary angioplasty. Further studies are needed to answer questions about how best to incorporate these advances into our overall treatment algorithm.


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