Pulmonary arterial hypertension (PAH) is a rare and complex vascular disease with poor prognosis and high mortality rate, characterized by progressive increase of pulmonary vascular resistance and loss of pulmonary vascular compliance. Ultimately, these hemodynamic changes lead to right ventricular (RV) failure and hence rapid progression to death. The symptoms are often unspecific, which can lead to a delay in diagnosis. In addition, the gold standard for diagnosis of PAH, right heart catheterization (RHC), however with low risk of morbidity and mortality, is an invasive method. Early non-invasive risk assessment can be of aid in diagnosis, selecting patients for RHC as well as in treatment management and prognosis. Currently, echocardiography is the most used non-invasive method for evaluating cardiac function in patients with PAH, but the RV function and size is difficult to assess accurately with echocardiography. Cardiac magnetic resonance imaging (CMR) can be used to accurately assess the RV, why CMR is the gold standard for measuring volumes and global ventricular function, such as ejection fraction. However, even with preserved ejection fraction subtle changes in cardiac function has been shown in patients with pulmonary hypertension. New methods of measuring cardiac function with CMR, such as strain, atrioventricular displacement and kinetic energy in patients with PAH has not yet been fully explored.
Therefore, the aim for this project is to a) evaluate novel methods using CMR for early risk assessment of PAH b) if these CMR measures are related to invasive measurements, c) if CMR can assist improved prognostication and treatment efficacy.
The outcome may be of great importance for potential identification at a lower risk status, rendering earlier treatment initiation, better treatment evaluation and improved prognosis.
The project plan is designed to establish a progression of challenges, such that each step represents a clear increment in challenges and independence of the PhD student. In the intended fifth study ethics application was performed and the patient inclusion will be performed by the PhD student.
The project is a collaboration among the Department of Clinical Sciences Lund, Clinical physiology, Cardiology and Rheumatology Lund University, and Skåne University Hospital, Lund, Sweden.
Supervisors from clinical physiology will be Ellen Ostenfeld and Håkan Arheden (co-supervisor), from cardiology Göran Rådegran (co-supervisor) and from rheumatology Roger Hesselstrand (co-supervisor)