European Society of Cardiology

European Society of Cardiology

August 29, 2015
2 min read

Joint guideline offers new algorithm for treatment of pulmonary hypertension

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LONDON — In a new guideline, the European Society of Cardiology and the European Respiratory Society outline a treatment algorithm for pulmonary hypertension and offer advice on when to use newly approved drugs.

Since the release of previous guidelines in 2009, “a huge amount of evidence has accumulated on the pharmacological treatment of patients with PAH [pulmonary arterial hypertension]. We have reconciled all the available data in a comprehensive treatment algorithm and in multiple tables that give recommendations for different patient risk profiles,” Nazzareno Galiè, MD, associate professor of cardiology at the University of Bologna, Italy, and chairperson of the task force that wrote the document, said in a press release. “The information is inclusive enough to be applicable in different health care systems with heterogeneous drug availability. The algorithm for [chronic thromboembolic pulmonary hypertension] is more detailed and recommends specific imaging tests to better define the treatment strategy."

New algorithm

After the PAH diagnosis is confirmed by an expert center, general measures such as avoidance of excessive physical activity and supportive therapy such as oxygen, diuretics or anticoagulants should be taken first, according to the document.

Patients with idiopathic, heritable or drug-induced PAH can undergo an acute vasoreactor test if necessary. If the patient is vasoreactive, he or she should be prescribed high-dose calcium channel blockers, the task force wrote.

Patients who are not vasoreactive and are at low or intermediate risk should be treated with drug monotherapy or oral combination therapy, while those at high risk should have combination therapy including IV prostacyclin analogs, according to the guideline. Monotherapies with the strongest evidence include endothelin receptor antagonists and phosphodiesterase type 5 inhibitors, while the combination therapy with the strongest evidence is ambrisentan (Letairis, Gilead Sciences) plus tadalafil.

If the patient does not respond, a double or triple sequential combination should be prescribed, the task force wrote, noting that among the combinations with the best evidence are macitentan (Opsumit, Actelion Pharmaceuticals) plus sildenafil, riociguat (Adempas, Bayer) plus bosentan and selexipag (Uptravi, Actelion/Nippon Shinyaku) added to an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. Selexipag is not yet approved for use in the United States.

If those combinations are not effective, doctors should consider referring the patient for lung transplantation, according to the guideline.

Other recommendations

Therapies approved for PAH are not recommended in patients with pulmonary hypertension due to left heart disease or a lung disease, according to the guideline.

In addition, women with PAH should not become pregnant.

PAH referral centers should have a multidisciplinary team caring for patients, including cardiologists, respiratory medicine physicians, clinical nurse specialists, radiologists and personnel who can provide psychological and social work support, they wrote.

The guideline was published in the European Heart Journal and the European Respiratory Journal and also presented at the European Society of Cardiology Congress. – by Erik Swain


Galiè N, et al. ESC/ERS Guidelines – Pulmonary Hypertension. Presented at: European Society of Cardiology Congress; Aug. 29-Sept. 2; London.

Galiè N, et al. Eur Heart J. 2015;doi:10.1093/eurheartj/ehv317.

Disclosures: Galié reports financial ties with Actelion, Bayer-Schering, Eli Lilly, GlaxoSmithKline, Novartis and Pfizer. See the full guideline for a list of the relevant financial disclosures of the other authors and reviewers.