Pediatric Cardiology

, Volume 39, Issue 4, pp 757–762 | Cite as

Acute Effect of Inhaled Iloprost in Children with Pulmonary Arterial Hypertension Associated with Simple Congenital Heart Defects

  • Qiangqiang Li
  • Konstantinos Dimopoulos
  • Chen Zhang
  • Yan Zhu
  • Qian Liu
  • Hong Gu
Original Article


Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH–CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included. Iloprost was administered by atomization inhalation and changes in hemodynamic parameters were recorded. In total, 100 children with PAH–CHD were enrolled. Median age was 13 [1.5–18.0] years and 34% were male. A ventricular septal defect was present in 84%, a patent duct in 12%, a complete atrioventricular septal defect in 2%, and an isolated atrial septal defect in 2%. Pulmonary vascular resistance indexed (PVRI) was above 8 WU m2 in 96% and was above a third systemic (Rp/Rs > 0.33) in 97%. Iloprost was well tolerated in all patients. Following iloprost inhalation, mean pulmonary arterial pressure decreased from 78.4 ± 9.2 to 72.8 ± 10.8 mmHg (p < 0.01) and pulmonary-to-systemic blood flow ratio (Qp/Qs) increased from 1.12 ± 0.48 to 1.37 ± 0.63 (p < 0.01), with no change in cardiac index (Qs). PVRI decreased from 21.0 ± 9.0 to 16.9 ± 8.0 WU m2 (p < 0.01) following inhalation but 92% patients still had a PVRI > 8 WU m2 and 93% an Rp/Rs > 0.33. Acute inhalation of iloprost in children with PAH associated with CHD resulted in a significant improvement in hemodynamic parameters. Despite this, few patients achieve strict criteria of operability, underscoring the importance of early screening and timely repair of CHD.


Catheterization Congenital heart defect Pulmonary arterial hypertension 



This study was funded by National Natural Science Foundation of China (Grant Number 81570442).

Compliance with Ethical Standards

Conflict of interest

Hong Gu, Qiangqiang Li, Chen Zhang, Yan Zhu, and Qian Liu have no conflict of interest. Konstantinos Dimopoulos has received unrestricted educational and research funds and has acted as a Consultant for Actelion, GSK, MSD, and Pfizer.

Ethical Approval

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Qiangqiang Li
    • 1
  • Konstantinos Dimopoulos
    • 2
  • Chen Zhang
    • 1
  • Yan Zhu
    • 1
  • Qian Liu
    • 1
  • Hong Gu
    • 1
  1. 1.Department of Pediatric Cardiology, Beijing Anzhen HospitalCapital Medical UniversityBeijingChina
  2. 2.Adult Congenital Heart Centre and Centre for Pulmonary HypertensionRoyal Brompton Hospital and Imperial CollegeLondonUK

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