How to detect disease progression in pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a rapidly progressive disease, ultimately leading to right heart failure and death. Accumulating evidence indicates that intervention early in disease progression results in better outcomes than delaying treatment. In this review we will discuss the assessments and strategies that can be used to monitor disease progression and guide clinical management. Many tools, such as symptoms, functional classification, exercise capacity, haemodynamic measures, findings on cardiac imaging and levels of biomarkers, have shown to be prognostic for survival both at diagnosis and during treatment. However, attempts to define goal thresholds have produced a variety of results. Several groups have developed risk calculators to estimate individual patients' mortality risk, but the accuracy of these tools across different patient populations remains unknown. What is clear is the importance of regularly assessing a range of parameters and then tailoring treatment goals to each patient. In addition, the use of a multidisciplinary team approach is crucial in order to support patients through all aspects of managing their condition. There is still an urgent need for prospective collaborative initiatives to assess novel goals and improve treatment strategies that would allow physicians to personalise and optimise clinical management for their patients with PAH.

Figure 1.

Baseline New York Heart Association functional class (NYHA FC) predicts survival in patients with pulmonary hypertension using infused epoprostenol therapy. a) 162 patients followed for a median of 36 months. Reproduced from [5] with permission from the publisher. b) 178 patients followed for a median of 26 months. Reproduced from [6] with permission from the publisher.

Figure 2.

Target 6-min walk distance (6MWD) after 3 months of epoprostenol therapy (a) but not change in 6MWD between baseline and 3 months of epoprostenol therapy (b) predicted survival in 178 patients with pulmonary hypertension followed for a median of 26 months. Reproduced from [6] with permission from the publisher.

Figure 3.

Goal-oriented strategy developed at the pulmonary hypertension clinic at Erasme University (Brussels, Belgium). FC: functional class; 6MWD: 6-min walk distance; RHC: right heart catheterisation; CPET: cardiopulmonary exercise testing.