Adherence to disease-specific drug treatment among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

Abstract

BackgroundPulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong treatment. The aim of the present study was to investigate adherence to disease specific treatment in patients with PAH or CTEPH.MethodsThe study comprised an adult population diagnosed with PAH (n=384) or CTEPH (n=187) alive in 2016–2017. The study utilised three registries; the Swedish PAH registry, the National Board of Health and Welfare and, Statistics Sweden. Withdrawals from pharmacies of disease-specific oral treatments were studied. Adherence was assessed as; 1) Number of days covered defined as the difference between the total number of daily dosages dispensed and the total number of days covered and 2) Manual assessment by two persons that independently reviewed each patient's prescription fill history to detect anomalies or patterns of deteriorating or improving adherence over time.ResultsThe mean age was 61±16 years, 61% were female and mean time since diagnosis was 4.6 years. Adherence was 62% using the Number of days covered method and 66% by the Manual assessment method. Drug specific adherence varied from 91% for riociguat to 60% for sildenafil. Good adherence was associated to shorter time since diagnosis in patients with PAH and to lower number of concomitant other chronic treatments in patients with CTEPH. Age, sex, socioeconomic status, or number of PH-treatments were not associated to adherence.ConclusionAdherence to oral disease specific treatment was 60–65% and associated to time since diagnosis and number of concomitant chronic treatments. Sex, age or socioeconomic factors did not affect adherence.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…Recent studies have indicated that adherence to drug therapy in patients with PAH may be suboptimal [348,349]. Given the complexity of PAH treatment, potential side effects, and risks associated with treatment interruptions, adherence should be periodically monitored by a member of the multidisciplinary team, to identify non-adherence and any changes to the treatment regimen spontaneously triggered by patients or non-expert physicians.…”

Prevalence of potential drug–drug interactions with disease‐specific treatments in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A registry study

“…Anticoagulant treatment with the vitamin K antagonist warfarin is recommended for patients with CTEPH, 1 and though no longer recommended for patients with PAH, 1 it is still commonly used in this population. 10 In the present study, a vast majority of patients with CTEPH and almost half of the patients with PAH were treated with warfarin. The combination with bosentan may induce hepatic metabolism (cytochrome P2C9) and reduce warfarin plasma concentration.…”

“…[2][3][4][5] An improved survival and an older population being diagnosed have increased the presence of comorbidities and thus, polypharmacy is common in this population. [6][7][8][9][10] Further, side effects from pulmonary hypertension (PH)-specific drugs such as headache, nausea, diarrhea, or constipation often require additional medical treatment.…”

Clinical Outcome of Closure of a Small Atrial Septal Defect in a Patient with Pulmonary Arterial Hypertension

“…Therefore, it was somewhat difficult to predict potential worsening of his PAH in a timely manner, as shown in the RHC and cMRI data actually obtained three years after ASD closure. Adherence to oral PAH drugs was said to be around 60% ( 14 ), and patient education concerning medical treatment is critically important, especially for patients with progressive diseases like IPAH. We strongly suggest that even after successful ASD closure, periodic examinations and confirmation of the use of previously prescribed PAH drugs should be performed, especially for patients with IPAH.…”