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Actelion (a Janssen Pharmaceutical company of J&J) announce positive final results of new study

  • Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson has announced the final results of a new study evaluating the effect of OPSUMIT® (macitentan) on the heart function and blood pressure of the lungs, in patients with pulmonary arterial hypertension (PAH)
  • The results have just been published on the International Society for Heart and Lung Transplantation (ISHLT) website
  • PAH is a serious progressive disease with no cure in which the walls of the pulmonary arteries become thick and stiff, narrowing the space for blood to flow, which increases blood pressure. PAH can lead to heart failure and early death[i],[ii],[iii],[iv]


  • The Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension (REPAIR) trial is the first prospective study of macitentan in PAH to use right ventricular stroke volume (RVSV) measured by cardiac MRI (cMRI) as one of the primary endpoints to measure the effect of a PAH therapy on the heart function and blood pressure of the lungs[v],[vi]


  • The results showed an improvement of the right heart function* and a decrease of the blood pressure in the arteries of the lungs,* and the severity of the disease improved in 56.3% of cases. These patients also showed an increase in their exercise capacity** and no patients worsened during the course of the trial[vi]


  • This study shows that cMRI could be selected as a non-invasive option, with reproducible measurements for monitoring the progression of PAH.  Currently, patients not only undergo invasive right heart catherisation at diagnosis, which is when it is required, but also during follow-up. Also, in comparison to other non-invasive tools such as the six-minute walking distance (6MWD), it could show the changes in heart structure[vii]


  • This study demonstrates the use of cMRI could allow physicians to show patients their response to treatment, with a clear physical translation of any improvements. It could also offer a more personalised approach to disease management, where physicians can select and adjust treatment options based on its visible effect on an individual


  • Janssen and Actelion are committed to transforming PAH into a long-term, manageable condition so that patients can live a normal life. We want to bring about a new age of PH research that holistically addresses unmet needs across the patient journey


  • We have a number of clinical trials ongoing which form a core part of our clinical development plan to further investigate the potential of our portfolio


* RVSV was significantly increased by 12mL, which is significant both clinically and statistically, highlighting improved RV function, and pulmonary vascular resistance (PVR) decreased by 38% (p<0.0001)5

** The exercise capacity of the patients improved by 36m as measured by 6MWD (p<0.0001)5

[i] Hoeper MM and Gibbs SR. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev 2014; 23:450−57.

[ii] Vachiéry JL and Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev 2012; 21:313−20.

[iii] McLaughlin VV, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009; 119(16):2250−94.

[iv] Schermuly RT, et al. Mechanisms of disease: pulmonary arterial hypertension. Nat Rev Cardiol 2011; 8(8):443−55.

[v] Vonk-Noordegraaf A, et al. Results from the REPAIR study final analysis: Effects of macitentan on right ventricular (RV) remodeling in pulmonary arterial hypertension (PAH). J Heart Lung Transplant 2020; 39(4):S16–17.

[vi] NCT02310672. Available at Clinical (Last accessed April 2020).

[vi] NCT02310672. Available at Clinical (Last accessed April 2020).

[vii] Galie N, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension European Heart Journal 2016; 37:67−119.


Editor Details

  • Company:
    • Janssen Pharmaceutical company of J&J
  • Name:
    • Janssen Pharmaceutical company of J&J
Last Updated: 05-May-2020