Sequential treatment with sildenafil and riociguat in patients with persistent or inoperable chronic thromboembolic pulmonary hypertension improves functional class and pulmonary hemodynamics

Darocha, Szymon; Banaszkiewicz, Marta; Pietrasik, Arkadiusz; Piłka, Michał; Florczyk, Michał; Wieteska, Maria; Dobosiewicz, Anna; Szmit, Sebastian; Torbicki, Adam; Kurzyna, Marcin

doi:10.1016/j.ijcard.2018.07.015

Cited by 20 publications

(17 citation statements)

References 30 publications

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“…In our cohort we also included patients with a longer CTEPH disease history or who were already on other PAH medication, as patients in daily practice often switch between therapies to achieve maximal treatment effect or due to adverse events. In addition, a recent study reported improved WHO FC and pulmonary hemodynamics after a switch from sildenafil to riociguat [17], although another research showed that a switch may not be as effective as direct initiation of riociguat [18]. However, the patients in this transition group were older and had more severe CTEPH disease [18].…”

Section: Discussionmentioning

confidence: 99%

Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension

Thor

Klooster

Snijder

et al. 2019

IJC Heart & Vasculature

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Background To improve clinical outcome, patients with inoperable and residual chronic thromboembolic pulmonary hypertension (CTEPH) can be treated with riociguat. The aim of this study is to explore long-term outcomes and to compare our ‘real world’ data with previous research. Methods We included all consecutive patients with technical inoperable and residual CTEPH, in whom riociguat therapy was initiated from January 2014 onwards, with patients followed till January 2019. Survival, clinical worsening (CW), functional class (FC), N-terminal pro brain natriuretic peptide (NT-proBNP) and 6-minute walking distance (6MWD) were described yearly after riociguat initiation. Results Thirty-six patients (50% female, mean age 64.9 ± 12.1 years, 54% WHO FC III/IV and 6MWD 337 ± 138 m could be included, with a mean follow-up of 2.3 ± 1.2 years. Survival and CW-free survival three years after initiation of riociguat were 94% and 78%, respectively. The 6MWD per 10 m at baseline was a significant predictor (HR 0.90 [0.83–0.97], p = 0.009) for CW. At three years follow-up the WHO FC and 6MWD improved and NT-proBNP decreased compared to baseline. Conclusion Our study confirms that riociguat is an effective treatment in patients with technical inoperable and residual CTEPH at long-term follow-up. Although our results are consistent with previous studies, more ‘real world’ research is necessary to confirm long-term results.

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Section: Discussionmentioning

confidence: 99%

Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension

Thor

Klooster

Snijder

et al. 2019

IJC Heart & Vasculature

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“…2 Currently riociguat 13,14 is recommended for inoperable, persistent or recurrent CTEPH, 2 as the only approved drug therapy. However, other pulmonary hypertension specific drugs such as sildenafil, 15,16 bosentan, 17 macitentan 18 and treprostinil 19 are also used. 12 BPA may be considered in inoperable patients and patients with unfavourable risk–benefit ratio for surgery.…”

Section: Introductionmentioning

confidence: 99%

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team

Siennicka

Darocha²,

Banaszkiewicz³

et al. 2019

Ther Adv Respir Dis

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Background: Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept. Methods and results: A total of 160 patients were consulted by the CTEPH team between December 2015 and September 2018. Patient baseline characteristics, CTEPH team decisions and implementation rates of diagnostic and therapeutic procedures were analysed. Change in World Health Organization (WHO) functional class and survival rates were evaluated by treatment strategy. A total of 51 (32%) patients were assessed as operable and 109 (68%) were deemed inoperable. Thirty-one (61% of operable patients) underwent PEA. Patients treated with PEA, BPA(+MT) and MT alone were 50.9 ± 14.7, 62.9 ± 15.1 and 68.9 ± 12.7 years old, respectively. At the follow-up, PEA patients had the highest WHO functional class improvement. Patients treated with BPA(+MT) had significantly better survival than PEA (p = 0.04) and MT patients (p = 0.04; 2-year survival of 92%, 79% and 79%, respectively). Conclusions:The CTEPH team ensures that necessary diagnostic procedures are performed. A relatively low proportion of patients was assessed by the CTEPH team as operable and underwent surgery, which in survivors resulted in the best functional improvement. Although patients undergoing BPA(+MT) were older than patients treated with PEA, their survival was better than patients subjected to PEA or MT alone. The reviews of this paper are available via the supplemental material section.

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“…Patients demonstrated reductions in PVR, mPAP, and increased cardiac output. 38 The data from these case studies suggest that switching to riociguat has been considered in the clinical setting, with the suggestion of therapeutic efficacy, although the current role of this therapeutic modality in the treatment algorithm is not yet defined.…”

Section: Case Studies Of Switching To Riociguatmentioning

confidence: 99%

Switching to riociguat: a potential treatment strategy for the management of CTEPH and PAH

et al. 2020

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Currently, five classes of drug are approved for the treatment of pulmonary arterial hypertension (PAH): phosphodiesterase 5 inhibitors (PDE5i); endothelin receptor antagonists; prostacyclin analogs; the IP receptor agonist selexipag; and the soluble guanylate cyclase (sGC) stimulator riociguat. For patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), riociguat is currently the only approved pharmacotherapy. Despite the development of evidence-based guidelines on appropriate use of specific drugs, in clinical practice patients are often prescribed PAH-targeted therapies off label or at inadequate doses. PDE5i are the most often prescribed class of drugs as initial therapy, either alone or in combination with other drug classes. However, a proportion of patients receiving PAH therapies do not reach or maintain treatment goals. As PDE5i and riociguat target different molecules in the nitric oxide-sGC-cyclic guanosine monophosphate (NO-sGC-cGMP) signaling pathway, for patients with PAH without an initial or sustained response to PDE5i, there is a biological rationale for switching to riociguat. However, robust data from randomized controlled trials on the safety and efficacy of switching are lacking, as is formal guidance for clinicians. Here we review studies of sequential combination therapy, and trial data and case studies that have investigated switching between PAH-approved therapies, particularly from PDE5i to riociguat in patients with PAH with an insufficient response to PDE5i, and in patients with CTEPH who were receiving off-label treatment. These studies summarize the current evidence and practical real-life experience on the concept of switching treatments.

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Sequential treatment with sildenafil and riociguat in patients with persistent or inoperable chronic thromboembolic pulmonary hypertension improves functional class and pulmonary hemodynamics

Cited by 20 publications

References 30 publications

Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension

Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team

Switching to riociguat: a potential treatment strategy for the management of CTEPH and PAH

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