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Change of right heart size and function by long-term therapy with riociguat in patients with PAH and CTEPH

Benjamin Egenlauf, Alberto Marra, Nicola Ehlken, Christine Fischer, Christina Eichstaedt, Christian Nagel, Eduardo Bossone, Antonio Cittadini, Michael Halank, Henning Gall, Karen Olsson, Tobias Lange, Ekkehard Grünig
European Respiratory Journal 2015 46: PA2455; DOI: 10.1183/13993003.congress-2015.PA2455
Benjamin Egenlauf
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
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Alberto Marra
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
22Internal Medicine Section-Department of Translational Medical Sciences, "Federico II" University School of Medicine, Naples, Italy
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Nicola Ehlken
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
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Christine Fischer
3Institute of Human Genetics, University of Heidelberg, Heidelberg, Germany
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Christina Eichstaedt
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
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Christian Nagel
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
4Department of Pneumology, Clinic Mittelbaden, Baden-Baden, Germany
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Eduardo Bossone
5Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, University of Salerno, Salerno, Italy
6Cardiac Surgery Dept., IRCCS Policlinico San Donato, Milan, Italy
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Antonio Cittadini
22Internal Medicine Section-Department of Translational Medical Sciences, "Federico II" University School of Medicine, Naples, Italy
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Michael Halank
7Medical Clinic I, University Hospital of TU Dresden, Dresden, Germany
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Henning Gall
8Department of Pneumology, University of Giessen Lung Center, Giessen, Germany
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Karen Olsson
9Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany
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Tobias Lange
10Internal Medicine II, University Medical Center Würzburg, Würzburg, Germany
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Ekkehard Grünig
1Centre for Pulmonary Hypertension, Thoraxclinic at the University of Heidelberg, Heidelberg, Germany
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Abstract

Background: Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTPEH).

Objectives: Aim of this study was to evaluate the change of right heart size and function assessed by echocardiography during long-term treatment with riociguat.

Methods: We assessed patients who started riociguat treatment within the trials PATENT, PATENTplus, EAS, CHEST and continued for 3-12 months. Echocardiography, 6-minute walking distance (6MWD) and further clinical parameters were analyzed at baseline, after 3, 6 and 12 months. Right heart catheterization was performed at baseline and after 3 months. For missing data we performed the last and baseline observation carried forward (LOCF, BOCF) method.

Results: Thirty-nine patients (21 PAH, 18 CTEPH, 56.4% treatment-naïve) were included. Mean right ventricular (RV) area significantly decreased after 3 (-2.1±3.9cm2, equals 7.4±15.3%, p<0.01), 6 (-4.2±3.2cm2, equals 16.1±11.5%, p<0.001) and 12 months (-5.9±4.6cm2, equals 22.1±14.2%, p<0.001) compared to baseline. Right atrial area significantly decreased after 12 months (-3.5±4.1cm2, equals 16.8±19.2%, p<0.001). TAPSE significantly improved after 6 (+2±4.7, equals 12±25.8%, p=0.03) and 12 months (+3.6±5.4, equals 21.0±29.6%, p<0.01). RV wall thickness and 6MWD significantly improved after 3, 6 and 12 months (p<0.05). Invasive hemodynamics significantly improved after 3 months. LOCF and BOCF showed similar significance and lower effect sizes.

Conclusion: Treatment with riociguat significantly reduced right heart size and improved RV function in PAH and CTEPH. Further prospective studies are needed.

  • Pulmonary hypertension
  • Treatments
  • Longitudinal study
  • Copyright ©ERS 2015
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Change of right heart size and function by long-term therapy with riociguat in patients with PAH and CTEPH
Benjamin Egenlauf, Alberto Marra, Nicola Ehlken, Christine Fischer, Christina Eichstaedt, Christian Nagel, Eduardo Bossone, Antonio Cittadini, Michael Halank, Henning Gall, Karen Olsson, Tobias Lange, Ekkehard Grünig
European Respiratory Journal Sep 2015, 46 (suppl 59) PA2455; DOI: 10.1183/13993003.congress-2015.PA2455

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Change of right heart size and function by long-term therapy with riociguat in patients with PAH and CTEPH
Benjamin Egenlauf, Alberto Marra, Nicola Ehlken, Christine Fischer, Christina Eichstaedt, Christian Nagel, Eduardo Bossone, Antonio Cittadini, Michael Halank, Henning Gall, Karen Olsson, Tobias Lange, Ekkehard Grünig
European Respiratory Journal Sep 2015, 46 (suppl 59) PA2455; DOI: 10.1183/13993003.congress-2015.PA2455
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