Monitoring & follow-up in PAH

Pulmonary arterial hypertension is a progressive disease and the it has been shown in the National Institute of Health (NIH) registry in 1981 that the median survival was 2.8 years if not treated early.   Even though survival over 7 years has improved in the later era with advances in therapy as shown in the Registry to Evaluate Early And Long-term PAH disease (REVEAL registry) which enrolled PAH patients from 2006 to 2009 in the US  who had PAH targeted therapy within 6 months of diagnosis, it is still suboptimal with a survival rate of only 58.9 ± 2.7% .

There are many reasons for this but strategic monitoring and objective assessment of these patients on follow up following initiation of PAH targeted therapy is crucial.

Some important principles are:

1. Early review with 1-3 months following initiation of therapy and 3-6 months subsequently. This allows for monitoring of adverse events, compliance and response to therapy. Treatment can be revised and optimized early.
    
2. Objective multiparametric assessment of the response to therapy using risk assessment at follow up. ,  (e.g. ESC risk assessment table, Reveal risk score , French registry  etc , .).
   Clinical assessment as well as appropriate investigations to assess the functional capacity using 6min walk test/ cardiopulmonary exercise testing, monitoring of the right ventricular function with biomarkers, echocardiogram and Cardiac magnetic resonance as well as cardiac catherization when indicated is currently the standard of care for PAH patients
    
3. Accelerating PAH targeted therapy based on the risk of the patient either upfront combination therapy, early sequential therapy or even triple combination therapy in high risk patients is recommended by the guidelines. The aim is to get the patients into the low risk status and maintain them in the low risk to optimize survival.iii,iv,
   
    
4. It is also important to understand the PAH’s patients’ experience and challenges they face living with PAH. Hence patient-reported outcome measures (PROM) must be also assessed during follow up. A number of PH-specific HR-QoL instruments have been developed and validated (e.g. Cambridge Pulmonary Hypertension Outcome Review [CAMPHOR] , emPHasis-10  ,living with Pulmonary Hypertension  ,and Pulmonary Arterial Hypertension-Symptoms and Impact [PAH-SYMPACT]).