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Published on: February 20, 2017
Paradoxical increase of pulmonary vascular resistance during testing of inhaled iloprost
M Emmel1, B Keuth, S Schickendantz
1University of Cologne, Cologne, Germany. mathias.emmel@medizin.uni-koeln.de
Insights
Inhaled iloprost, a treatment for primary pulmonary hypertension, unexpectedly caused a severe increase in pulmonary vascular resistance (PVR) in a young patient. This case highlights the potential for paradoxical reactions to this medication.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Pharmacology
Background:
- Primary pulmonary hypertension (PPH) is a severe condition affecting children.
- Domiciliary oxygen therapy is a common initial treatment.
- Nitric oxide (NO) inhalation demonstrated a positive response, suggesting potential for vasodilator therapy.
Observation:
- A 14-month-old girl with PPH was evaluated.
- Inhaled nitric oxide showed a beneficial effect on pulmonary vascular resistance (PVR).
- Subsequent testing with inhaled iloprost led to a paradoxical, severe increase in PVR.
Findings:
- Pulmonary vascular resistance (PVR) increased from 392 to 1192 dynes x s x cm(-5) with the addition of iloprost.
- Ventilatory and technical issues were ruled out as causes for the PVR increase.
- This represents a rare "paradoxical" reaction to inhaled iloprost.
Implications:
- While iloprost is generally effective for PPH, this case demonstrates a potential for adverse "paradoxical" reactions.
- Clinicians should be aware of the possibility of severe PVR increase with iloprost in select patients.
- Further investigation into the mechanisms of paradoxical reactions to pulmonary vasodilators is warranted.
Abstract:
The case of a 14 month old girl with primary pulmonary hypertension treated with domiciliary oxygen is described. After invasive evaluation and testing of nitric oxide with very good response, the testing was repeated to study the effect of inhaled iloprost on pulmonary vascular resistance (PVR). An unexpected and severe increase of PVR was observed, rising from 392 dynes x s x cm(-5) with oxygen to a maximum of 1192 dynes x s x cm(-5) with oxygen and iloprost. Underlying ventilatory and technical problems were excluded. While inhaled iloprost has been described to be highly effective in the treatment of primary pulmonary hypertension, the possibility of contrary "paradoxical" reactions in isolated patients is emphasised, with a dramatic increase of PVR and a possible adverse outcome.
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