World Journal of Pediatrics

, Volume 6, Issue 1, pp 13–31

Advances in diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease

  • Monnipa Suesaowalak
  • John P. Cleary
  • Anthony C. Chang
Review Article

DOI: 10.1007/s12519-010-0002-9

Cite this article as:
Suesaowalak, M., Cleary, J.P. & Chang, A.C. World J Pediatr (2010) 6: 13. doi:10.1007/s12519-010-0002-9



This article aims to review recent advances in the diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease.

Data sources

Articles on pulmonary arterial hypertension in congenital heart disease were retrieved from PubMed and MEDLINE published after 1958.


A diagnosis of primary (or idiopathic) pulmonary arterial hypertension is made when no known risk factor is identified. Pulmonary arterial hypertension associated with congenital heart disease constitutes a heterogenous group of conditions and has been characterized by congenital systemic-to-pulmonary shunts. Despite the similarities in histologic appearance of pulmonary vascular disease, there are differences between pulmonary arterial hypertension secondary to congenital systemic-to-pulmonary shunts and those with other conditions with respect to pathophysiology, therapeutic strategies, and prognosis. Revision and subclassification within the category of secondary pulmonary arterial hypertension based on pathophysiology were conducted to improve specific treatments. The timing of surgical repair is crucial to prevent and minimize risk of postoperative pulmonary arterial hypertension. Drug therapies including prostacyclin, endothelin-receptor antagonist, phosphodiesterase inhibitor, and nitric oxide have been evolved with promising results in neonates and children.


Among the different forms of congenital heart diseases, an early correction generally prevents subsequent development of pulmonary arterial hypertension. Emerging therapies for treatment of patients with idiopathic pulmonary arterial hypertension also improve quality of life and survival in neonates and children with congenital heart disease associated with pulmonary arterial hypertension. Heart and lung transplantation or lung transplantation in combination with repair of the underlying cardiac defect is a therapeutic option in a minority of patients. Partial repair options are also beneficial in some selected cases. Randomized controlled trials are needed to evaluate the safety and efficacy of these therapies including survival and long-term outcome.

Key words

children congenital heart disease endothelin-receptor antagonist neonate nitric oxide phosphodiesterase inhibitor prostacyclin pulmonary arterial hypertension 

Copyright information

© Children's Hospital, Zhejiang University School of Medicine and Springer Berlin Heidelberg 2010

Authors and Affiliations

  • Monnipa Suesaowalak
    • 1
  • John P. Cleary
    • 2
  • Anthony C. Chang
    • 3
    • 4
  1. 1.Pediatric Cardiac Surgery FoundationBangkokThailand
  2. 2.CHOC Pediatric Subspecialty FacultyChildren’s Hospital of Orange CountyOrangeUSA
  3. 3.CHOC Heart InstituteChildren’s Hospital of Orange CountyOrangeUSA
  4. 4.CHOC Heart InstituteChildren’s Hospital of Orange CountyOrangeUSA

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