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

Thor, M.C.J. van; Klooster, Liesbeth ten; Snijder, Repke J.; Post, Marco C.; Mager, Johannes J.

doi:10.1016/j.ijcha.2019.02.004

Cited by 24 publications

(22 citation statements)

References 24 publications

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“…In one year, the mean change of distance in the 6MWD test was +29 m and improvement by at least one NYHA class was observed in 26.2% of patients 8 . Similar results have been found in smaller comparable registries of patients with CTEPH receiving riociguat therapy 9,10 . Of note, NYHA class and 6MWD are important prognostic markers in CTEPH, and their improvement corresponds with the amelioration of survival in populations of treated patients 11 .…”

Section: Discussionsupporting

confidence: 84%

The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry

Jansa¹,

Ambrož²,

Kuchař³

et al. 2021

BIOMED PAP

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Ales Linhart a Aims. The primary objective of the registry was to assess the impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension (CTEPH) that was inoperable or persistent/recurrent after pulmonary endarterectomy (PEA). In contrast to randomized pivotal trials, this non-interventional registry evaluated the effectiveness and safety of riociguat in a real-world setting. Methods. Retrospective data were collected from patients' charts as recorded in routine clinical practice from the initiation of riociguat therapy up to approximately 5 months and 1 year after this initiation. Results. In total, 51 patients from a single site were enrolled. After 5 months (mean duration) of riociguat treatment, the following improvements from baseline were observed: change of distance in the 6-minute walking distance (6MWD) (P=0.066); change of score from the quality of life questionnaire (EQ5D-5L) (P=0.020), and overall self-assessment of health status (P=0.001). New York Heart Association (NYHA) class improved in 24.3% of patients. After 11.2 months (mean duration) of riociguat treatment, the following improvements from baseline were observed: change of distance in the 6MWD test (P=0.006), and overall self-assessment of health status (P=0.009). NYHA class improved in 46.4% of patients. Riociguat was well tolerated. In total, 4 patients reported side effects, with hospitalization required in one case and 2 patients who had to discontinue the treatment. Annual survival rate was 89.1%. Conclusion. Riociguat improves functional NYHA class, distance in the 6MWD test and quality of life in a real-world patient population.

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Section: Discussionsupporting

confidence: 84%

The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry

Jansa¹,

Ambrož²,

Kuchař³

et al. 2021

BIOMED PAP

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“…In the chest-1 trial mPAP fell by 10% and the PVR by one-third, while 6MWD increased by just over 10% in the riociguat arm and 33% of patients reported a one-grade improvement in FC. The safety and efficacy of riociguat appears to extend beyond 2 years6 15 although longer-term mortality benefits have not been demonstrated and the treatments are lifelong and expensive.…”

Section: Discussionmentioning

confidence: 99%

“…Mechanical obstructions and secondary pulmonary vasculopathy cause pulmonary hypertension, right ventricular (RV) remodelling and dilatation culminating in a worsening exercise tolerance, right heart failure and early death 5. Survival at 3 years in unoperated patients is 70% and is not affected by medical therapy, although preliminary data suggest that the soluble guanylate cyclase (sGC) riociguat may improve medium-term survival 6…”

Section: Introductionmentioning

confidence: 99%

Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

et al. 2020

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ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK opened in October 2015 and we now describe the treatment of our initial patient cohort.MethodsThirty consecutive, inoperable, anatomically suitable, symptomatic patients on stable medical therapy for CTEPH were identified and offered BPA. They initially underwent baseline investigations including Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) quality of life (QoL) questionnaire, cardiopulmonary exercise test, 6 min walk distance (6MWD), transthoracic echocardiography, N-terminal probrain natriuretic peptide (NT pro-BNP) and right heart catheterisation. Serial BPA sessions were then performed and after completion, the treatment effect was gauged by comparing the same investigations at 3 months follow-up.ResultsA median of 3 (IQR 1–6) BPA sessions per patient resulted in a significant improvement in functional status (WHO functional class ≥3: 24 vs 4, p<0.0001) and QoL (CAMPHOR symptom score: 8.7±5.4 vs 5.6±6.1, p=0.0005) with reductions in pulmonary pressures (mean pulmonary artery pressure: 44.7±11.0 vs 34.4±8.3 mm Hg, p<0.0001) and resistance (pulmonary vascular resistance: 663±281 vs 436±196 dyn.s.cm-5, p<0.0001). Exercise capacity improved (minute ventilation/carbon dioxide production: 55.3±12.2 vs 45.0±7.8, p=0.03 and 6MWD: 366±107 vs 440±94 m, p<0.0001) and there was reduction in right ventricular (RV) stretch (NT pro-BNP: 442 (IQR 168–1607) vs 202 (IQR 105–447) pg/mL, p<0.0001) and dimensions (mid RV diameter: 4.4±1.0 vs 3.8±0.7 cm, p=0.002). There were no deaths or life-threatening complications and the mild-moderate per-procedure complication rate was 10.5%.ConclusionsBPA is safe and improves the functional status, QoL, pulmonary haemodynamics and RV dimensions of patients with inoperable CTEPH.

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“…Given that riociguat is currently the only approved drug with a label to treat inoperable CTEPH in the United States, we also analyzed the subset of pulmonary vasodilator studies that evaluated riociguat usage 10,11,50,51 . The mean change in 6MWD in these studies was 49.4 meters (95% CI: 24.1-74.6 meters).…”

Section: Resultsmentioning

confidence: 99%

Comparison of Balloon Pulmonary Angioplasty and Pulmonary Vasodilators for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis

Kalra

Duval

Thenappan

et al. 2020

Sci Rep

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treatment options for chronic thromboembolic pulmonary hypertension (ctepH) that is not amenable to thromboendarterectomy or is recurrent/persistent after thromboendarterectomy (inoperable CTEPH) include pulmonary vasodilators or balloon pulmonary angioplasty (BPA). We compared efficacy and safety outcomes of BpA with or without pulmonary vasodilators to pulmonary vasodilator therapy alone in patients with inoperable ctepH. observational and randomized trial data reporting outcomes for >5 patients with inoperable CTEPH were sought. Single-arm random effects meta-analyses were performed. The primary outcome was change in six-minute walk distance (6MWD). Secondary outcomes included safety; World Health organization functional class (WHo fc); and change in mean pulmonary arterial pressure (mpAp), pulmonary vascular resistance (pVR), and cardiac index. thirtyfour studies with 1604 patients were eligible for analyses. Both treatments resulted in significant improvement in 6MWD (71.0 meters, 95% CI: 47.4-94.5 meters with BPA versus 47.8 meters, 95% CI: 34.5-61.2 meters with pulmonary vasodilators), PVR [−3.1 Wood Units (WU), 95% CI: −4.9 to −1.4 WU versus −1.6 WU, 95% CI: −2.4 to −0.8 WU] and mPAP (−14.8 mmHg, 95% CI: −18.2 to −11.5 mmHg versus −4.9 mmHg, 95% CI: −6.9 to −2.8 mmHg). Cardiac index was similar and most patients were WHo fc ii and iii after their respective interventions. More complications occurred in the BpA arm. In conclusion, BPA and pulmonary vasodilators both improve 6MWD and hemodynamics in patients with inoperable CTEPH. While BPA may offer greater functional and hemodynamic improvements, this technique carries the accompanying risks of an invasive procedure. Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by macrovascular obstruction due to thromboemboli with an accompanying small vessel pulmonary arteriopathy 1. Pulmonary thromboendarterectomy is the gold standard treatment for CTEPH with numerous centers of excellence worldwide 2,3. However, over 40% of CTEPH patients are ineligible for pulmonary thromboendarterectomy, due to a combination of technical inaccessibility of thromboemboli, poor surgical candidacy, patient choice, or recurrent/persistent pulmonary hypertension after the operation 4,5. CTEPH patients who are not amenable to thromboendarterectomy, develop CTEPH recurrence after thromboendarterectomy, or have persistent CTEPH despite thromboendarterectomy (inoperable CTEPH) have worse outcomes than patients who successfully undergo operative intervention 6,7. In view of this, new treatments have emerged for this patient population. Balloon pulmonary angioplasty (BPA) is a percutaneous approach that employs sequential pulmonary artery angioplasty to relieve the macrovascular obstruction associated with CTEPH 6. This approach improves exercise capacity and hemodynamics 8. Furthermore, pulmonary vasodilators

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

Cited by 24 publications

References 24 publications

The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry

The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry

Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

Comparison of Balloon Pulmonary Angioplasty and Pulmonary Vasodilators for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis

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