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Highlight on the 2015 edition of Pulmonary Hypertension Guidelines

Epidemiological data about Pulmonary Hypertension (PH) are not largely available. Patients belonging to Group 2 (Left Heart Disease - LHD) and to Group 3 (Lung diseases) are often seen in clinical practice, but only data from registries are available. Some estimates consider that up to 60% of people with LHD with systolic dysfunction and 70%  with preserved systolic function may present with PH. Pulmonary Arterial Hypertension of group 1 and Idiopathic Pulmonary Arterial Hypertension (PAH) seem to have a prevalence of 15 and 5.9 cases per million in adult population.
While group 1 PAH is consequently an uncommon clinical problem, group 2 and 3 PH, and also PH related to pulmonary embolism and to some internal medicine illnesses may be frequently encountered in clinical practice.
The new 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension* were presented at ESC Congress 2015 in London and published in January 2016 in the European Heart Journal. Due to the multidisciplinary nature of PH, the composition of the Guidelines Task Force includes members of different medical disciplines.

What is new in the 2015 Pulmonary Hypertension ESC Guidelines

The main novelty of this version in comparison with 2009 edition may be summarized in:

  • The structure, which includes three chapters about classification, basic aspects and differential diagnosis, two chapters about PAH, one chapter about PH due to LHD, one for PH due to lung disease, one about Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and one about unclear/multifactorial mechanisms
  • Modification of the haemodynamic definition
  • Updated clinical classification for adult and paediatric population
  • Updates in pathology, pathobiology, genetics, epidemiology, risk factors
  • Updated diagnostic algorithm with novel screening strategies
  • Underlining of the importance of the expert referral centres in diagnostic and treatment algorithms
  • Updated treatment algorithm according to new PAH severity evaluation and to new treatments and combination therapy
  • Abandon of the term ‘out of proportion PH’.
  • New diagnostic and treatment protocols for treatment of chronic thromboembolic pulmonary hypertension (CTEPH)
  • Short chapter due to unclear and multifactorial mechanisms of PH.


PH is still defined as an increase in mean pulmonary arterial pressure >/= 25 mmHg at rest assessed by right heart catheterization. Due to lack of data ‘PH on exercise’ should not be used. PAH is related to the presence of pre-capillary PH defined as a pulmonary artery wedge pressure </= 15 mmHg and a pulmonary vascular resistance > 3 Wood units in the absence of other causes of precapillary PH due to lung diseases, CTEPH or other specific causes.

PH PAPm >/= 25 mmHg All
Pre-Capillary PH PAPm >/= 25 mmHg
PAWP </= 15 mmHg
1. Pulmonary Arterial Hypertension
3. PH due to lung disease
5. PH unclear/multifactorial mechanisms
Post-Capillary PH PAPm >/= 25 mm
HgPAWP >/= 15 mmHg
2. PH due to LHD
5. PH unclear/multifactorial mechanisms

Isolated capillary PH DPG < 7mmHg and/or PVR </= 3 WU
Combined post and pre-capillary DPG >/= 7mmHg and/or PVR > 3 WU


The new findings of the clinical classification of PH includes: new conditions frequently found in children, gene mutations recently identified and chronic haemolytic anaemia. The structure of the classification remains the same as before with five groups: Group 1 (PAH and pulmonary veno-occlusive and persistent PH of the newborn), Group 2 (LHD), Group 3 (Lung disease and Hypoxia), Group 4 (CTEPH) and Group 5 (PH from unclear/multifactorial mechanisms). In Group 1 the following are still included: PH due to drugs, connective disease, HIV infection, portal hypertension and congenital heart disease.


Clinical presentation, electrocardiogram, chest X-ray and pulmonary function tests with arterial blood gases are shown in the chapter about the diagnosis of PH. A specific paragraph about echocardiography underlines the probability of PH in patients with suspicion of PH, based on peak tricuspid regurgitation velocity and on other signs regarding the ventricles, the pulmonary artery, the right atrium and the inferior vena cava; the management of the patients combining the echocardiographic data and the clinical data of the patient are summarized in a specific table. Other imaging methods are analysed with their indications and peculiarities. A specific paragraph about right heart catheterization and vaso-reactivity test recalls a series of issues about technical aspects and gives specific recommendations for catheterization and the use of vaso-reactivity tests.
The diagnostic algorithm, after suspicion of PH identified at echocardiography, continues with an attempt to identify the more common groups of PH: group 2, 3, and 4. The investigation of choice for suspected acute pulmonary embolism is computer tomography, while planar ventilation/perfusion lung scan is still the essential test in suspicion of CTEPH.  If the diagnostic trial doesn’t lead to a diagnosis, at the end of the process there is an attempt for the recognition of groups 1 and 5 PH. 

Careful evaluation of severity is based on clinical parameters, imaging and haemodynamic findings, on the evaluation of exercise capacity, on biochemical markers; these findings should help to define the patient’s status and consequently make a comprehensive prognostic evaluation and risk assessment.


Therapy is based on general measures to support the patient’s status, such as oral anticoagulants, diuretics, oxygen administration, digoxin and other drugs. In group 2 and 3 patients with cardiac and lung diseases specific treatment may be required such as valve surgery, coronary artery disease treatment, HR control of AF, and so on. Specific recommendations are reported for group 4 PH, secondary to CTEPH, where the treatment of choice is pulmonary endoarteriectomy and inoperable situations should be treated with balloon pulmonary angioplasty.
Group 1 PAH therapy has improved impressively in the last years with larger efficacy but also with complex algorithms. Therapy may begin with single or with combination therapy, based on specific drugs active on each of the different signalling pathways involved in the pathophysiology of the illness (prostacyclin pathway, endothelin pathway and nitric oxide pathway). The text provides specific tables about monotherapy and about indications and contraindications to combination and sequential combination therapy in relation to the WHO functional class. High risk patients should undergo sequential combination therapy including an intravenous prostacyclin analogue.
Drug therapy should be periodically submitted to careful reappraisal of its efficacy. After an inadequate clinical response the patient's elibility for lung transplantation should be considered.  


Referral centres where organized multi-professional teams of cardiologists, pneumologists, internal medicine physicians, radiologists, psychologists and also specialized nurses, usually obtain the best performance in diagnostic and therapeutic management of PAH. Due to the high volumes of  patients treated these centres tend to optimize the use of resources and attain the best outcomes.
The task of these centres is to receive new referrals, fully assess the causes of PH according to the diagnostic algorithm, implement correct therapeutic strategies and periodically re-evaluate by means of careful clinical and instrumental follow-up of the patients. They should also provide audits, research and education in the complex field of pulmonary hypertension.  


In the same issue of the European Heart Journal the Authors provide a Decalogue of recommendations about pulmonary hypertension.

  1. Right heart catheterization is recommended to confirm the diagnosis and to guide treatment
  2. Vasoreactivity tests is recommended in idiopathic PAH, inherited PAH and drugs and toxin induced PAH to guide high dosed calcium-channel blockers therapy
  3. Evaluation of severity should be done every 3-6 months with clinical assessment, exercise tests, biochemical markers, echocardiography and catheterization.
  4. Avoid pregnancy in PAH
  5. PAH patients should be followed in referral centres managed by multi-professional teams
  6. Initial drug mono or combination therapy is recommended in PAH patients naïve or at low or intermediate risk
  7. Sequential combination therapy is recommended in PAH patients with inadequate response to initial mono or combination therapy
  8. Initial combination therapy + intravenous prostacyclin analogue is recommended in PAH high risk patien
  9. PAH drugs use is not recommended in patients with group 2 or 3  PH
  10. Surgical pulmonary endoarteriectomy is the treatment of choice in patients with CTEPH


* Pulmonary Hypertension (Guidelines on Diagnosis and Treatment of)

The Joint Task Force for the Diagnosis and the Treatment of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS)
Nazzareno Galiè, Marc Humbert, Jean-Luc Vachiery  (Eur Heart J 2016: 37; 67-119)

** The Ten Commandments for 2015 European Society of Cardiology – European Respiratory Society Guidelines on Pulmonary Hypertension
Marc Humbert, Nazzareno Galiè (Eur Heart J 2016: 37; 5)