Updated European guidelines for the diagnosis and treatment of pulmonary hypertension include a new treatment algorithm, and for the first time recommend early consideration of lung transplantation.
The guidelines published by the European Respiratory Society and the European Society of Cardiology reflect evidence accumulated since the previous joint guidelines were published in 2009.
Professor Nazzareno Galiè, ESC Chairperson of the guidelines Task Force, said: “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.”
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 PAH.
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 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 centres are given a pivotal role in the management of pulmonary hypertension, starting with the diagnostic process said Professor Humbert.
“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.”