Case report: a patient with pulmonary arterial hypertension transitioning from a PDE-5 inhibitor to Riociguat

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Abstract

Background

We present here the case of a patient with pulmonary arterial hypertension and NYHA Class II symptoms who transitioned from PDE-5i therapy to riociguat. No protocol currently exists for transitioning between these PAH medications.

Case presentation

A 59-year old male with a history of anorexigen use initially presented in 2008 and was felt to have non-operable small vessel disease. His care was transitioned to our center after insurance would not cover high-dose sildenafil in addition to ERA therapy.

Conclusion

This case demonstrates a safe and successful transition from higher dose PDE-5is to riociguat with no interruption in therapy.

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Most cited references 6

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Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

Nazzareno Galiè, Joan Barberà, Adaani Frost … (2015)

Data on the effect of initial combination therapy with ambrisentan and tadalafil on long-term outcomes in patients with pulmonary arterial hypertension are scarce.

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Definitions and diagnosis of pulmonary hypertension.

Toru Satoh, Marc R. Wilkins, David Langleben … (2013)

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure ≥ 25 mm Hg at rest, measured during right heart catheterization. There is still insufficient evidence to add an exercise criterion to this definition. The term pulmonary arterial hypertension (PAH) describes a subpopulation of patients with PH characterized hemodynamically by the presence of pre-capillary PH including an end-expiratory pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg and a pulmonary vascular resistance >3 Wood units. Right heart catheterization remains essential for a diagnosis of PH or PAH. This procedure requires further standardization, including uniformity of the pressure transducer zero level at the midthoracic line, which is at the level of the left atrium. One of the most common problems in the diagnostic workup of patients with PH is the distinction between PAH and PH due to left heart failure with preserved ejection fraction (HFpEF). A normal PAWP does not rule out the presence of HFpEF. Volume or exercise challenge during right heart catheterization may be useful to unmask the presence of left heart disease, but both tools require further evaluation before their use in general practice can be recommended. Early diagnosis of PAH remains difficult, and screening programs in asymptomatic patients are feasible only in high-risk populations, particularly in patients with systemic sclerosis, for whom recent data suggest that a combination of clinical assessment and pulmonary function testing including diffusion capacity for carbon monoxide, biomarkers, and echocardiography has a higher predictive value than echocardiography alone. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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Relevant issues in the pathology and pathobiology of pulmonary hypertension.

Rubin M Tuder, Stephen Archer, Peter Dorfmüller … (2013)

Knowledge of the pathobiology of pulmonary hypertension (PH) continues to accelerate. However, fundamental gaps remain in our understanding of the underlying pathological changes in pulmonary arteries and veins in the different forms of this syndrome. Although PH primarily affects the arteries, venous disease is increasingly recognized as an important entity. Moreover, prognosis in PH is determined largely by the status of the right ventricle, rather than the levels of pulmonary artery pressures. It is increasingly clear that although vasospasm plays a role, PH is an obstructive lung panvasculopathy. Disordered metabolism and mitochondrial structure, inflammation, and dysregulation of growth factors lead to a proliferative, apoptosis-resistant state. These abnormalities may be acquired, genetically mediated as a result of mutations in bone morphogenetic protein receptor-2 or activin-like kinase-1, or epigenetically inherited (as a result of epigenetic silencing of genes such as superoxide dismutase-2). There is a pressing need to better understand how the pathobiology leads to severe disease in some patients versus mild PH in others. Recent recognition of a potential role of acquired abnormalities of mitochondrial metabolism in the right ventricular myocytes and pulmonary vascular cells suggests new therapeutic approaches, diagnostic modalities, and biomarkers. Finally, dissection of the role of pulmonary inflammation in the initiation and promotion of PH has revealed a complex yet fascinating interplay with pulmonary vascular remodeling, promising to lead to novel therapeutics and diagnostics. Emerging concepts are also relevant to the pathobiology of PH, including a role for bone marrow and circulating progenitor cells and microribonucleic acids. Continued interest in the interface of the genetic basis of PH and cellular and molecular pathogenetic links should further expand our understanding of the disease. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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Author and article information

Contributors

David S. Poch: dspoch@ucsd.edu

Journal

Journal ID (nlm-ta): BMC Pulm Med

Journal ID (iso-abbrev): BMC Pulm Med

Title: BMC Pulmonary Medicine

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2466

Publication date (Electronic): 18 May 2016

Publication date PMC-release: 18 May 2016

Publication date Collection: 2016

Volume: 16

Electronic Location Identifier: 82

Affiliations

Division of Pulmonary, Critical Care, & Sleep Medicine, University of California, San Diego, CA USA

Article

Publisher ID: 229

DOI: 10.1186/s12890-016-0229-x

PMC ID: 4870744

PubMed ID: 27193179

SO-VID: 9d6cb670-585a-4944-bdba-1b7135bd4f04

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 11 November 2015

Date accepted : 15 April 2016

Custom metadata

ScienceOpen disciplines: Respiratory medicine

Keywords: pulmonary arterial hypertension,sgc stimulators,cyclic guanosine monophosphate,chronic thromboembolic pulmonary hypertension

Data availability:

ScienceOpen disciplines: Respiratory medicine

Keywords: pulmonary arterial hypertension, sgc stimulators, cyclic guanosine monophosphate, chronic thromboembolic pulmonary hypertension

Case report: a patient with pulmonary arterial hypertension transitioning from a PDE-5 inhibitor to Riociguat

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Abstract

Background

Case presentation

Conclusion

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Most cited references 6

Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

Definitions and diagnosis of pulmonary hypertension.

Relevant issues in the pathology and pathobiology of pulmonary hypertension.

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Contributors

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Article

History

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