Intravenous iron therapy in patients with idiopathic pulmonary arterial hypertension and iron deficiency

PVRI Member Authors: Luke Howard, Martin Wilkins, Harm Jan Bogaard, Anton Vonk Noordegraaf


In patients with idiopathic pulmonary arterial hypertension (iPAH), iron deficiency is common and has been associated with reduced exercise capacity and worse survival. Previous studies have shown beneficial effects of intravenous iron administration. In this study, we investigated the use of intravenous iron therapy in iron-deficient iPAH patients in terms of safety and effects on exercise capacity, and we studied whether altered exercise capacity resulted from changes in right ventricular (RV) function and skeletal muscle oxygen handling. Fifteen patients with iPAH and iron deficiency were included. Patients underwent a 6-minute walk test, cardiopulmonary exercise tests, cardiac magnetic resonance imaging, and a quadriceps muscle biopsy and completed a quality-of-life questionnaire before and 12 weeks after receiving a high dose of intravenous iron. The primary end point, 6-minute walk distance, was not significantly changed after 12 weeks (409 ± 110 m before vs. 428 ± 94 m after; P = 0.07). Secondary end points showed that intravenous iron administration was well tolerated and increased body iron stores in all patients. In addition, exercise endurance time (P < 0.001) and aerobic capacity (P < 0.001) increased significantly after iron therapy. This coincided with improved oxygen handling in quadriceps muscle cells, although cardiac function at rest and maximal V͘o2V͘o2 were unchanged. Furthermore, iron treatment was associated with improved quality of life (P < 0.05). In conclusion, intravenous iron therapy in iron-deficient iPAH patients improves exercise endurance capacity. This could not be explained by improved RV function; however, increased quadriceps muscle oxygen handling may play a role. (Trial registration: identifier NCT01288651)

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Functional Ability/ Impairment/ Quality of Life/ Physical Activity/ Exercise
Pulmonary Arterial Hypertension
Right Ventricle: Structure, Function and Dysfunction


Gerrina Ruiter, Emmy Manders, Chris M. Happé, Ingrid Schalij, Herman Groepenhoff, Luke S. Howard, Martin R. Wilkins, Harm J. Bogaard, Nico Westerhof, Willem J. van der Laarse, Frances S. de Man, Anton Vonk-Noordegraaf

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Pulmonary Circulation Vol 5: No 3 cover image

September 2015

Pulmonary Circulation Vol 5: No 3

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