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Oral Treprostinil in 2 Pediatric patients with IPAH

Anne Davis


Delphine Yung

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Conference: 2017 PH Professional Network Symposium

Release Date: 10.06.2017

Presentation Type: Abstracts

File Download: 2017 PHPN Abstract 1037

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Abstract presented at the 2017 PH Professional Network Symposium held in Bethesda, MD on October 5-7, 2017

Purpose

Describe the experience of oral treprostinil in 2 children with IPAH.

Background

IV, SQ and inhaled treprostinil use had been described in children and can improve PH symptoms and quality of life. Oral treprostinil is simpler and less risky to deliver. In adults, side effects can limit dosing. No reports on oral treprostinil have been published.

Methods

Patient 1
10-year-old girl with severe idiopathic pulmonary hypertension on triple therapy (IV treprostinil, tadalafil, and bosentan) underwent Potts shunt. Eleven months later; she was transitioned from IV to oral. 

Patient 2
10 year old boy with IPAH on tadalafil and ambrisentan


Results

Both patients experienced improvement in 6 min walk distance and functional class. Both patients experienced severe headaches, which improved by improving hydration and dividing tadalafil. In addition, one patient’s headaches improved by changing to QID dosing and the other needed to slow up titration. Both families report good quality of life.

Conclusion

Children with IPAH can tolerate and experience improvement in PH on oral treprostinil. Side effects of headaches can be due to many reasons and requires dedication to treat effectively