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ESC/ERS Guidelines for pulmonary hypertension

ESC Congress News 2015 - London

The new 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (PH) are authored by the Joint Task Force of the ESC and of the European Respiratory Society (ERS) and endorsed by the European Paediatric and Congenital Cardiology, the International Society for Heart and Lung Transplantation, the European League Against Rheumatism, and the European Society of Radiology.

Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Pulmonary Hypertension
Valvular Heart Disease

Access the Guidelines here

Antonio Vaz Carneiro, University of Lisbon School of Medicine, Portugal Victor Aboyans, Kupuytren University Hospital, Limoges, FrancePH is defined as an increase in mean pulmonary arterial pressure (PAPm) ≥25 mmHg at rest as assessed by right heart catheterisation. It is a pathophysiological disorder which may involve multiple clinical conditions and complicate the majority of cardiovascular and respiratory diseases.

This latest edition follows the two previous ESC/ERS guidelines of 2004 and 2009 and is based on systematic literature review to identify new studies published since 2009.

The new clinical classification of PH includes five major types:

  1. Pulmonary arterial hypertension (with two subtypes: pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis and persistent PH of the newborn)
  2. PH due to left heart disease
  3. PH due to lung diseases and/or hypoxia
  4. Chronic thromboembolic PH and other pulmonary artery obstructions
  5. PH with unclear and/or multifactorial mechanisms.

The main changes from the previous 2009 guidelines are:

  • The table of contents has been simplified with three initial general chapters on classifications, basic aspects and differential diagnosis, two chapters on pulmonary arterial hypertension (PAH), and one chapter each for PH due to left heart disease, PH due to lung disease and/or hypoxia, chronic thromboembolic pulmonary hypertension, and PH due to unclear and/or multifactorial mechanisms, respectively.
  • New wordings and parameters for the haemodynamic definition of post-capillary PH sub-groups have been adopted. Pulmonary vascular resistance has been included in the haemodynamic definition of PAH.
  • An updated common clinical classification for adult and paediatric patients is reported.
  • New advances in pathology, pathobiology, genetics, epidemiology and risk factors are reported.
  • An updated diagnostic algorithm has been added in an independent chapter, and novel screening strategies are proposed in the web addenda.
  • The importance of expert referral centres in the management of PH patients has been highlighted in both the diagnostic and treatment algorithms.
  • New developments on PAH severity evaluation and on treatments and treatment goals are reported, including combination therapy and two new drugs recently approved. The treatment algorithm has been updated accordingly.
  • New diagnostic and treatment algorithms are added in the chronic thromboembolic PH chapter, including general criteria for operability and for balloon pulmonary angioplasty.

    Specifically, in what concerns therapy for PH, there are several recommended approaches:

  1. General measures (physical activity and supervised rehabilitation; pregnancy, birth control, and postmenopausal hormonal therapy; elective surgery; infection prevention; psychosocial support; adherence to treatments; travel and genetic counselling).
  2. Supportive therapy (oral anticoagulants, diuretics, oxygen, digoxin and other CV drugs and anaemia and iron status).
  3. Specific drug therapy with calcium channel blockers (nifedipine, diltiazem and amlodipine), endothelin receptor antagonists (ambrisentan, bosentan, macitentan), phosphodiesterase type-5 inhibitors and guanylate cyclase stimulators (sildenafil, tadalafil, vardenafil, riociguat), prostacyclin analogues and prostacyclin receptors agonists (beraprost, epoprostenol, iloprost, treprostinil, selexipag) and experimental compounds and strategies.
  4. Combination therapy.
  5. Drug interactions.
  6. Balloon atrial septostomy.
  7. Advanced right ventricular failure.
  8. Transplantation.

A treatment algorithm is also included and the diagnosis and treatment of PH complications are presented. Finally, end-of-life care and ethical issues are discussed. This same approach is followed for specific PH subsets (paediatric, left heart disease or lung diseases/hypoxia, from chronic thromboembolic pulmonary hypertension, etc).

At the end there is a definition of a PH referral centre and a complete online addenda with extra tables, figures and text with the pathology and pathobiology of PH, a proposal for a screening programme and a set of quality of life measurements.

Access all ESC Clinical Practice Guidelines here