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ESC/ERS Pulmonary Hypertension Guidelines: novel treatment algorithm

The 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension are published in the European Heart Journal and the European Respiratory Journal.
The document was written jointly by the ESC and the ERS and incorporates evidence accumulated since the previous joint guidelines were published in 2009.

Pulmonary hypertension is a condition in which blood pressure is raised in the pulmonary arteries, which supply blood to the lungs. It is a serious medical condition that leads to progressive right ventricular dysfunction with symptoms such as shortness of breath, fatigue, weakness, angina and syncope typically induced by exertion.

The guidelines introduce a novel treatment algorithm for pulmonary arterial hypertension ( PAH ) and treatment tables outlining when to use recently approved drugs. Treatment strategies are based on the patient’s risk profile.
Sequential and initial combination therapies are recommended following the emergence of new evidence and early consideration for lung transplantation is recommended for the first time.
A novel treatment algorithm is also presented for chronic thromboembolic pulmonary hypertension ( CTEPH ) that includes surgical, medical and interventional strategies.

Clinical and haemodynamic classifications have been updated using new evidence on genetic and drug causes. Paediatric disorders are also classified and pulmonary vascular resistance ( PVR ) is included in the diagnosis of pulmonary arterial hypertension.
A new diagnostic algorithm starts with the echocardiographic probability of pulmonary hypertension ( low, intermediate, high ) and is followed by identification of the most common causes such as pulmonary hypertension due to heart diseases, lung diseases and chronic thromboembolic pulmonary hypertension.

Expert centres are given a pivotal role in the management of pulmonary hypertension, starting with the diagnostic process.
Right heart catheterization is recommended for confirming the final diagnosis of PAH and CTEPH and this technically demanding procedure provides more accurate information and has lower morbidity when performed in expert centres.
Patients with established hypertension should be referred early to expert centres to ensure they are given the best care.

For the first time, the definition of satisfactory/unsatisfactory clinical responses to therapy is based on the change or maintenance of an individual patient risk profile.
Nine clinical, functional, exercise, biochemical, imaging and haemodynamic parameters are assessed to calculate an estimated one year mortality of low ( less than 5% ), intermediate ( 5-10% ), or high ( more than 10% ).

Therapy for pulmonary arterial hypertension

The current treatment strategy for PAH patients can be divided into three main steps:

1. The initial approach includes general measures ( physical activity and supervised rehabilitation, pregnancy, birth control and post-menopausal hormonal therapy, elective surgery, infection prevention, psychosocial support, adherence to treatments, genetic counselling and travel ), supportive therapy ( oral anticoagulants, diuretics, O2, Digoxin ), referral to expert centres and acute vasoreactivity testing for the indication of chronic therapy with calcium channel blockers.

2. The second step includes initial therapy with high-dose calcium channel blocker in vasoreactive patients or drugs approved for PAH in non-vasoreactive patients according to the prognostic risk of the patient and the grade of recommendation and level of evidence for each individual compound or combination of compounds.

3. The third part is related to the response to the initial treatment strategy; in the case of an inadequate response, the role of combinations of approved drugs and lung transplantation are proposed.

Treatment algorithm description

• After confirmation of the diagnosis of the treatment-naive PAH patient in an expert centre, the suggested initial approach is the adoption of general measures and the initiation of supportive therapy if needed.

• Acute vasoreactivity testing should be performed only in patients with IPAH [ Idiopatic PAH ], HPAH [ Heritable PAH ] and PAH associated with drugs or toxins use. Vasoreactive patients should be treated with high doses ( progressively titrated ) of calcium channel blockers; adequate response should be confirmed after 3-4 months of treatment. Patient responders without an adequate clinical response to calcium channel blockers treatment should be treated with approved PAH medications according to the non-vasoreactive patient’s treatment strategy.

• Non-responders to acute vasoreactivity testing who are at low or intermediate risk can be treated with either initial monotherapy or initial oral combination therapy.

• If initial monotherapy is chosen, since head-to-head comparisons among different compounds are not available, no evidence-based first-line monotherapy can be proposed. In this case the choice of the drug may depend on a variety of factors, including the approval status, labelling, route of administration, side-effect profile, potential interaction with background therapies, patient preferences, co-morbidities, physician experience and cost.

• Since head-to-head comparison between initial combination therapy with Ambrisentan plus Tadalafil has proven to be superior to initial monotherapy with Ambrisentan or Tadalafil in delaying clinical failure, a higher grade of recommendation has been given to this initial combination.

• In non-vasoreactive and treatment-naive patients at high risk, initial combination therapy including i.v. prostacyclin analogues should be considered. I.V. Epoprostenol should be prioritised since it has reduced the 3-month rate of mortality in high-risk PAH patients also as monotherapy. Alternative types of initial combination therapy may be considered.

• In case of inadequate clinical response to initial combination therapy or initial monotherapy, sequential double or triple combination therapy is recommended. The combination of Rociguat and PDE-5i is contraindicated.

• In case of inadequate clinical response with sequential double combination therapy, triple combination therapy should be attempted.

• It seems reasonable to consider eligibility for lung transplantation after an inadequate clinical response to the initial monotherapy or initial combination therapy and to refer the patient for lung transplantation soon after the inadequate clinical response is confirmed on maximal combination therapy. BAS ( balloon atrial septostomy ) should be regarded as a palliative or bridging procedure in patients deteriorating despite maximal medical therapy. ( Xagena )

Source: European Society of Cardiology, 2015