Quality of life in patients with chronic thromboembolic pulmonary hypertension

Abstract

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) experience debilitating symptoms that have a negative impact on their quality of life (QoL) in terms of physical capability, psychological wellbeing and social relationships. The use of QoL measurement tools is important in the assessment of treatment efficacy and in guiding treatment decisions. However, despite the importance of QoL, particularly to the patient, it remains under-reported in clinical studies of CTEPH therapy. CTEPH is unique in pulmonary hypertension in that it is potentially curable by surgery; however, a proportion of patients either have residual PH following surgery or are not operable. Although some patients with CTEPH have been treated off-label with pulmonary arterial hypertension-specific therapies, there have been few randomised controlled trials of these therapies in patients with CTEPH. Moreover, in these trials QoL outcomes are variably assessed, and there is little consistency in the tools used. Here we review the assessment of QoL in patients with CTEPH and the tools that have been used. We also discuss the effect of surgical intervention and medical therapies on QoL. We conclude that further studies of QoL in patients with CTEPH are needed to further validate the optimal QoL tools.

Pulmonary rehabilitation in moderate pulmonary hypertension

“…25 It has been reported that patients with PH have debilitating symptoms that negatively impact HRQoL, which mainly generates a progressive deterioration in physical capacity and psychological status. 32 These statements are very similar to those found in our study because the patient presented greater problems in the fatigue and dyspnea domain of the CRQ-SAS questionnaire, clearly evidencing the commitment in the respiratory physical capacity. In turn, as mentioned by some authors, the interventions based on aerobic exercise, muscle strengthening of upper and lower limbs and respiratory muscle exercises, have clear benefits in the quality of life of patients with pathology of pulmonary origin, 33 which benefits for patients, not only in the domains related to the symptomatology, but also in the emotional domains and the control of the disease (Table 2).…”

A meta‐analysis of randomized controlled trials in targeted treatments of chronic thromboembolic pulmonary hypertension

“…CTEPH patients experience debilitating symptoms that severely impact their life quality. 39 Figure 6 demonstrated the effect of PAH-specific treatments on Borg dyspnea scale in this population, which was reported in four studies. [8][9][10]13 There was a larger improvement with PAH-targeted therapies, as compared to control (MD, −0.70; 95% CI, −1.08 to −0.32; P < 0.001).…”

“…CTEPH patients experience debilitating symptoms that severely impact their life quality . Figure demonstrated the effect of PAH‐specific treatments on Borg dyspnea scale in this population, which was reported in four studies .…”

An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan

“…The second study in patients with PAH also identified a significant deterioration in FC associated with a prolonged time between patient-described symptom onset and diagnosis of PAH by right heart catheterisation, which the authors considered could potentially impact on mortality [69], although it should be considered that the natural history of PAH is not the same as that of CTEPH. The consequence of late diagnosis is that treatment options may become limited due to comorbidities and organ damage, and the patient's quality of life may be severely compromised [70]. There appeared to be regional variations in the numbers of patients in NYHA FC III/IV at diagnosis in this study, particularly between Japan and Europe.…”