The European Society of Cardiology (ESC) together with the European Respiratory Society (ERS) recently launched a new treatment algorithm for pulmonary arterial hypertension in its updated pulmonary hypertension guidelines. The amended protocol aims to provide patients with the best chance of a positive clinical outcome despite being diagnosed with an untreatable, highly debilitating, and limiting disease.
The new guidelines are now published online in the European Heart Journal, European Respiratory Journal as well as on the ESC Website. The two societies co-wrote the document, which also draws from evidence collated by the previous set of guidelines from 2009.
The document introduces the new treatment algorithm for pulmonary arterial hypertension (PAH) together with treatment tables summarizing when to administer some of today’s newer medications. Outlined treatment strategies take patients’ risk profiles into account, and sequential and initial combination therapies are recommended based on new findings, and for the first time, early consideration for lung transplantation is recommended. A new treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) has also been made available, and includes surgical, medical and interventional strategies.
Professor Nazzareno Galiè, ESC Chairperson of the guidelines Task Force, said: “Since the 2009 guidelines a huge amount of evidence has accumulated on the pharmacological treatment of patients with PAH. We have reconciled all the available data in a comprehensive treatment algorithm and in multiple tables that give recommendations for different patient risk profiles. The information is inclusive enough to be applicable in different healthcare systems with heterogeneous drug availability. The algorithm for CTEPH is more detailed and recommends specific imaging tests to better define the treatment strategy.”
Both clinical and hemodynamic classifications have been updated with newer evidence on genetic and drug causes. Disorders among children have been classified, and pulmonary vascular resistance (PVR) has been included in the diagnosis of PAH. The document also introduces a novel diagnostic algorithm that 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 CTEPH.
“Pulmonary hypertension can involve multiple clinical conditions,” said Professor Marc Humbert, European Respiratory Society (ERS) Co-Chairperson of the guidelines Task Force. “The updated classifications and new diagnostic algorithm will help clinicians to better define an individual patient’s disease so that the most appropriate treatment can be given.”
Expert centers are given a pivotal role in the management of pulmonary hypertension, starting with the diagnostic process. Professor Humbert said: “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.”
The new guidelines marks the first time satisfactory/unsatisfactory clinical responses to treatment are defined based on the change or maintenance of an individual patient’s risk profile. There are 9 clinical, functional, exercise, biochemical, imaging and haemodynamic parameters to be assessed in order to arrive at an estimated one year mortality of (<5%), intermediate (5-10%), or high (>10%).
Professor Galiè concluded: “The new data and the new recommendations included in this updated version of the pulmonary hypertension guidelines are intended to provide a practical approach to the management of these complex patients. In addition, the typical multidisciplinary approach will enhance the level of collaboration between various specialists and centres with different levels of expertise and experience.”