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Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

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Published on: May 11, 2015

Vasodilator therapy for primary pulmonary hypertension in children

R J Barst1, G Maislin, A P Fishman

  • 1Department of Pediatrics, Columbia University, College of Physicians and Surgeons, New York, NY, USA.

Circulation
|March 9, 1999
PubMed

Insights

Long-term vasodilator therapy significantly improves survival for children with primary pulmonary hypertension (PPH). Calcium channel blockers are effective for acute responders, while prostacyclin (PGI2) benefits nonresponders and those unresponsive to other treatments.

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Hypertension Research
  • Vascular Pharmacology

Background:

  • Presents 13 years of vasodilator therapy experience for pediatric primary pulmonary hypertension (PPH).
  • Compares two treatment eras: oral calcium channel blockers (1982-1987) and intravenous prostacyclin (PGI2) post-1987.

Purpose of the Study:

  • To evaluate the long-term efficacy of vasodilator therapies in children with PPH.
  • To determine survival benefits of different vasodilator strategies based on patient response.

Main Methods:

  • Conducted short-term vasodilator testing with intravenous PGI2 on 74 children.
  • Classified patients as acute responders or nonresponders to PGI2.
  • Treated responders with oral calcium channel blockers and nonresponders with long-term PGI2 or conventional therapy.

Main Results:

  • Calcium channel blockers improved survival in 31 responders compared to 43 nonresponders (P=0.0002).
  • Long-term PGI2 treatment significantly improved survival in nonresponders compared to those without PGI2 access (P=0.0005).
  • Overall, 31 children failing conventional therapy showed improved survival with vasodilator treatment (P=0.002).

Conclusions:

  • Long-term vasodilator therapy is crucial for improving survival in pediatric PPH.
  • Oral calcium channel blockers are generally sufficient for acute responders.
  • Continuous intravenous PGI2 infusion enhances survival in nonresponders and those refractory to calcium channel blockers.
Abstract

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