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Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation

Paul Corris, Bruno Degano
European Respiratory Review 2014 23: 488-497; DOI: 10.1183/09059180.00007214
Paul Corris
1Institute of Cellular Medicine, Newcastle University and the Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK. 2Service de Physiologie-Exploration Fonctionnelles, CHRU, et EA 3920, Université de Franche-Comté, Besançon, France
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  • For correspondence: paul.corris@ncl.ac.uk
Bruno Degano
1Institute of Cellular Medicine, Newcastle University and the Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK. 2Service de Physiologie-Exploration Fonctionnelles, CHRU, et EA 3920, Université de Franche-Comté, Besançon, France
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    Figure 1.

    Updated treatment algorithm for patients with pulmonary arterial hypertension (PAH) in World Health Organization (WHO) functional class IV. ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase type-5 inhibitor; sGCS: soluble guanylate cyclase stimulators; BAS: balloon atrial septostomy. #: based on the WHO functional class of the majority of patients in the studies; ¶: morbidity and mortality were the primary end-point in a randomised controlled study or reduction in all-cause mortality (prospectively defined). Reproduced from [7] with permission from the publisher.

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    Figure 2.

    Use of parenteral prostanoids in 192 patients with pulmonary arterial hypertension (PAH) in functional class IV at the time of all-cause mortality. Results from the US REVEAL (Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management) registry. Data from [30].

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  • Table 1. Atrial septostomy improves haemodynamics in patients with pulmonary arterial hypertension
    First author [ref.]Patients nInitial RAP mmHgRAP changeLAP changeCardiac index changeSOT change
    Rich [60]61741NC5827
    Nihill [61]1412NC369
    Kerstein [62]15111155155
    Sandoval [59]151529553522
    Rothman [63]122322202418
    • Data are presented as %, unless otherwise stated. RAP: right atrial pressure; LAP: left atrial pressure; SOT: systemic organ transplant; NC: no change. Reproduced from [63] with permission from the publisher.

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    • P. Corris
    • B. Degano
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Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation
Paul Corris, Bruno Degano
European Respiratory Review Dec 2014, 23 (134) 488-497; DOI: 10.1183/09059180.00007214

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Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation
Paul Corris, Bruno Degano
European Respiratory Review Dec 2014, 23 (134) 488-497; DOI: 10.1183/09059180.00007214
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Jump To

  • Article
    • Abstract
    • Introduction
    • Treatment options for patients with severe PAH
    • PAH and lung transplantation
    • Identifying patients most likely to benefit from lung transplantation
    • PAH and lung transplantation: bridging the gap
    • Outcomes following lung transplantation
    • Conclusion
    • Acknowledgments
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

Subjects

  • Acute lung injury and critical care
  • Pulmonary pharmacology and therapeutics
  • Pulmonary vascular disease
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