Short- and Long-Term Effects of Inhaled Iloprost Therapy in Children With Pulmonary Arterial Hypertension
D. Dunbar Ivy, MD*,1,*,
Aimee K. Doran, CPNP*,3,
Kelly J. Smith, MD ,
George B. Mallory, Jr, MD,
Maurice Beghetti, MD ,
Robyn J. Barst, MD ,4,
Daniela Brady, RN,
Yuk Law, MD||,
Donna Parker, RRT*,
Lori Claussen, RN* and
Steven H. Abman, MD*,2
* The Pulmonary Hypertension Program and Pediatric Heart Lung Center, Department of Pediatrics, The University of Colorado School of Medicine and The Children’s Hospital, Denver, Colorado
Pediatric Pulmonology, Texas Children’s Hospital, and Baylor University School of Medicine, Houston Texas
Pediatric Cardiology, Hospital of the University of Geneva and Children’s Hospital of Geneva, Geneva, Switzerland
Columbia University College of Physicians and Surgeons and New York Presbyterian Hospital, New York, New York
|| Children’s Hospital & Regional Medical Center, Department of Pediatrics, University of Washington, Seattle, Washington.
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Figure 1 Acute Inhalation of Iloprost Lowered Mean PAP Equivalent to the Response to 40 ppm Inhaled NO
Inhaled nitric oxide (NO) (40 ppm) reduced mean pulmonary artery pressure (PAP) from 66 ± 13 mm Hg at baseline to 58 ± 18 mm Hg (p < 0.05 vs. baseline); n = 8. The reduction in mean PAP after acute inhalation of iloprost was similar to the level achieved with inhaled NO therapy (57 ± 19 mm Hg; p < 0.05 vs. baseline).
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Figure 2 The Acute Effects of Inhaled Iloprost Were Assessed by Pulmonary Function Tests in 13 Patients
Baseline forced expiratory volume in 1 s (FEV1) (expressed as % predicted) was 84% (range 56% to 119%) and decreased after a single inhalation of iloprost to 79% (range –18% to +3%; p = 0.02).
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Figure 3 Acute Effects of Inhaled Iloprost Were Assessed by Pulmonary Function Tests in 13 Patients
At baseline, mean mid-volume forced expiratory flow (FEF25%–75%) was 82% of predicted (range 32% to 119%). After iloprost inhalation, mean FEF25%–75% decreased to 72% of predicted (p = 0.03). In 5 of 14 (38%) patients, FEF25%–75% decreased by more than 15% (range –53% to –17%).
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Figure 4 6MW Tests Were Obtained for 13 of 22 Patients at Baseline and After 6 Months
Overall, there was no change in 6-min walk distance (6MWD) from baseline (397 m) to 6 months (428 m); however, 6MWD did increase by >10% in 5, was unchanged in 7, and decreased by >10% in 1 child.
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Figure 5 Of 22 Patients, 20 Remained on Therapy at 6 Months
World Health Organization (WHO) class improved in 7 patients, remained unchanged in 10 patients, and worsened in 3 patients. BL = baseline.
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Figure 6 Of the 22 Patients Treated With Iloprost, 9 Were Transitioned From IV Prostanoids With 8 Tolerating the Transition
In 13 children, iloprost was used as add-on or de novo therapy. Of the 13 patients, 6 remain on therapy and 4 required transition to intravenous (IV) prostanoids. Two patients discontinued iloprost, owing to airways reactivity, and 1 patient died. SQ = subcutaneous.
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