• Kristin B Highland, Kathleen E Hughes, Kenneth J Williams, Brigit Kyei-Baffour, and Samantha Ferguson.
• Cleveland Clinic, Cleveland, Ohio.
• Am J Manag Care. 2019 Jun 1; 25 (7 Suppl): S119-S127.

AbstractPulmonary arterial hypertension (PAH) is a progressive, complex disease. PAH is a type of pulmonary hypertension (PH) and can be further categorized into 7 subdivisions, representing a variety of causal and phenotypic factors. Patients with PH, including PAH, are typically fragile and experience multiple comorbidities; they therefore require individualized treatment plans based on their risk status and etiology. Based on a review of clinical evidence, a wide variety of treatment options exist for PAH, including general measures (eg, physical activity and oral anticoagulants), nonspecific pharmacologic intervention (eg, calcium channel blockers), and targeted pharmacologic intervention. Guidelines point to a flexible approach, frequently including upfront or sequential combination therapy, to mitigate disease progression. Payer-driven drug exclusion policies, including formulary restrictions and noncoverage policies, can detract from the ability of providers to offer treatments consistent with guidelines, as they limit access to the range of treatment options needed for individualized patients. Providers must be able to work with each patient to develop a tailored strategy through open access to treatments, leveraging all available options, to mitigate against exacerbation of comorbidities and optimize care.

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