Idiopathic Pulmonary Arterial Hypertension: Evolving Therapeutic Strategies

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Abstract

The field of pulmonary arterial hypertension (PAH) has seen substantial changes in the epidemiology, associated comorbidities, treatment algorithms, and prognosis in the past decade. Specifically for idiopathic PAH (IPAH), there has been a noted increase in the age of initial diagnosis. Increasing focus is being placed on appropriate categorization of patients with PAH, both in terms of diagnosis and individualized risk stratification. This is especially relevant given that patients being diagnosed with incident IPAH are now older and with multiple comorbidities, putting them at a higher risk of being mislabeled as PAH. The biggest impact has been seen in the number of pharmaceutical options available for these patients, resulting in improvement in both short- and long-term outcomes. In addition, there has been a shift in the treatment approach to the early use of combination therapies in PAH, since it has been demonstrated to improve clinical outcomes. Novel therapies are in the pipeline for patients with IPAH to impact both the morbidity and mortality of this deadly disease.

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