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Pulmonary Hypertension
Updated classification and management of pulmonary hypertension
  1. Holger M Nef1,2,
  2. Helge Möllmann1,
  3. Christian Hamm1,
  4. Friedrich Grimminger1,2,
  5. Hossein-Ardeschir Ghofrani1,2
  1. 1Kerckhoff Heart and Thorax Center, Departments of Cardiology (Franz-Groedel-Institute) and Pneumology, Bad Nauheim, Germany
  2. 2University Hospital Giessen and Marburg GmbH, Klinikstrasse 36, Giessen, Germany
  1. Correspondence to Dr med. Holger M Nef, Kerckhoff Heart and Thorax Center, Benekestr. 2-8, D-61231 Bad Nauheim, Germany; h.nef{at}

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Pulmonary arterial hypertension (PAH) is a severe disease characterised by a progressive increase of pulmonary pressure and resistance leading to right heart failure. PAH is commonly diagnosed at a late stage of the disease and is associated with progressive clinical deterioration and premature death. According to the most recent consensus conference, pulmonary hypertension (PH) is categorised into five main groups: group 1, PAH; group 2, PH associated with left sided heart diseases; group 3, PH associated with lung disease and/or hypoxaemia; group 4, PH due to chronic thrombotic and/or embolic disease; and group 5, miscellaneous—summarising a variety of rare and not well characterised disorders characterised by non-specific signs and symptoms.1 Screening for PH is usually made by transthoracic echocardiography on the basis of the velocity of the regurgitant tricuspid jet, and final confirmation is done by right heart catheterisation.2

Without treatment, the prognosis for patients with significant PH is poor. The reported median life expectancy of idiopathic PAH is 2.8 years from the diagnosisw1; however, recent meta-analysis of trials in the field of PAH have provided indications of a beneficial influence of PAH treatments on survival.3 Current PAH specific medications, including prostanoids, endothelin receptor antagonists (ERA), and phosphodiesterase 5 inhibitors (PDE5i), have sought to address the pulmonary vascular endothelial dysfunction and vasoconstriction associated with this condition. Several novel drugs that have already produced encouraging results in animal models aim to target more directly the structural vascular changes (remodelling) and are currently under clinical investigation (eg, soluble guanylyl cyclase activators/stimulators, tyrosine kinase inhibitors, 5-HT2B receptor antagonists).

This article reviews the clinical classification, which has been recently updated. Moreover, established approaches to evaluate PH and the currently recommended treatments for PAH are summarised.

Definition of PH

The haemodynamic definition of PH has not been evidence based. Under normal circumstances, resting …

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  • Competing interests In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. HA Ghofrani has received honoraria and research funds from Actelion, Bayer Schering, ErgoNex Pharma, GlaxoSmithKline, Novartis, and Pfizer. F Grimminger has received honoraria and research funds from Actelion, Bayer Schering, Novartis, and Pfizer. C Hamm received speaker fees from Actelion and GlaxoSmith Kline. H Moellmann and H Nef received speakers fee from Actelion.

  • Provenance and peer review Not commissioned; not externally peer reviewed.