Arterial Methemoglobin Level Predicts Therapeutic Effectiveness of Phosphodiesterase Type 5 Inhibitors in Patients with Idiopathic Pulmonary Arterial Hypertension

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in patients with systemic sclerosis that can be misclassified as pulmonary arterial hypertension. Differentiation between pulmonary veno-occlusive disease and pulmonary arterial hypertension is challenging because of the similar clinical picture. Nevertheless, discrimination is important because pulmonary veno-occlusive disease has a worse prognosis. Vasodilators including phosphodiesterase type 5 inhibitors and endothelin receptor antagonists should be started with caution and often in combination with diuretics to prevent pulmonary edema.

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Practical management of riociguat in patients with pulmonary arterial hypertension

Therapeutic Advances in Respiratory Disease ◽

10.1177/1753466619868938 ◽

2019 ◽

Vol 13 ◽

pp. 175346661986893 ◽

Cited By ~ 2

Author(s):

Michael Halank ◽

Kristin Tausche ◽

Ekkehard Grünig ◽

Ralf Ewert ◽

Ioana R. Preston

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Randomized Study ◽

Signs And Symptoms ◽

The Elderly ◽

Open Label ◽

Phosphodiesterase Type 5 Inhibitors ◽

Practical Management ◽

Pulmonary Arterial ◽

Phosphodiesterase Type

Riociguat is one of several approved therapies available for patients with pulmonary arterial hypertension (PAH). Treatment should be initiated and monitored at an expert center by a physician experienced in treating PAH, and the dose adjusted in the absence of signs and symptoms of hypotension. In certain populations, including patients with hepatic or renal impairment, the elderly, and smokers, riociguat exposure may differ, and dose adjustments should therefore be made with caution according to the established scheme. Common adverse events are often easily managed, particularly if they are discussed before starting therapy. Combination therapy with riociguat and other PAH-targeted agents is feasible and generally well tolerated, although the coadministration of phosphodiesterase type 5 inhibitors (PDE5i) and riociguat is contraindicated. An open-label, randomized study is currently ongoing to assess whether patients who do not achieve treatment goals while receiving PDE5i may benefit from switching to riociguat. In this review, we provide a clinical view on the practical management of patients with PAH receiving riociguat, with a focus on the opinions and personal experience of the authors. The reviews of this paper are available via the supplemental material section.

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Medication adherence, hospitalization, and healthcare resource utilization and costs in patients with pulmonary arterial hypertension treated with endothelin receptor antagonists or phosphodiesterase type-5 inhibitors

Pulmonary Circulation ◽

10.1177/2045894019880086 ◽

2020 ◽

Vol 10 (1) ◽

pp. 204589401988008 ◽

Cited By ~ 1

Author(s):

Robert P. Frantz ◽

Jerrold W. Hill ◽

Cassandra A. Lickert ◽

Rolin L. Wade ◽

Michele R. Cole ◽

...

Keyword(s):

Medication Adherence ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Endothelin Receptor Antagonists ◽

Endothelin Receptor ◽

Receptor Antagonists ◽

Phosphodiesterase Type 5 Inhibitors ◽

Proportion Of Days Covered ◽

Pulmonary Arterial ◽

Phosphodiesterase Type

Adherence to therapy for pulmonary arterial hypertension is essential to optimize patient outcomes, but data on real-world adherence to different pulmonary arterial hypertension drug classes are limited. This retrospective database analysis evaluated relationships between adherence, hospitalization, and healthcare costs in pulmonary arterial hypertension patients treated with endothelin receptor antagonists or phosphodiesterase type-5 inhibitors. From the IQVIA Adjudicated Health Plan Database, patients with pulmonary arterial hypertension were identified based on diagnostic codes and prescriptions for endothelin receptor antagonists (ambrisentan, bosentan, macitentan) or phosphodiesterase type-5 inhibitors (sildenafil, tadalafil) approved for pulmonary arterial hypertension. Patients were assigned to the class of their most recently initiated (index) pulmonary arterial hypertension therapy between 1 January 2009 and 30 June 2015. Medication adherence was measured by proportion of days covered; patients with proportion of days covered ≥80% were considered adherent. The proportion of adherent patients was higher for endothelin receptor antagonists (571/755; 75.6%) than for phosphodiesterase type-5 inhibitors (970/1578; 61.5%; P < 0.0001). In both groups, hospitalizations declined as proportion of days covered increased. Among adherent patients, those on endothelin receptor antagonists had a significantly lower hospitalization rate than those on phosphodiesterase type-5 inhibitors (23.1% versus 28.5%, P = 0. 0218), fewer hospitalizations (mean (standard deviation) 0.4 (0.8) versus 0.5 (0.9); P = 0.02), and mean hospitalization costs during the six-month post-index ($9510 versus $15,726, P = 0.0318). Increasing adherence reduced hospitalization risk more for endothelin receptor antagonists than for phosphodiesterase type-5 inhibitors (hazard ratio 0.176 versus 0.549, P = 0.001). Rates and numbers of rehospitalizations within 30 days post-discharge were similar between groups. Mean total costs were higher with endothelin receptor antagonists than phosphodiesterase type-5 inhibitors in all patients ($91,328 versus $72,401, P = 0.0003) and in adherent patients ($88,867 versus $56,300, P < 0.0001), driven by higher drug costs.

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