The Journal of Pediatrics
Volume 155, Issue 2 , Pages 176-182.e1 , August 2009

Clinical Characterization of Pediatric Pulmonary Hypertension: Complex Presentation and Diagnosis

  • Rosa Laura E. van Loon, MD

      Affiliations

    • Department of Pediatric Cardiology, University Medical Center, University of Groningen, Groningen, The Netherlands
    • ,
  • Marcus T.R. Roofthooft, MD

      Affiliations

    • Department of Pediatric Cardiology, University Medical Center, University of Groningen, Groningen, The Netherlands
    • ,
  • Magdalena van Osch-Gevers, MD, PhD

      Affiliations

    • Erasmus Medical Center, Rotterdam, The Netherlands
    • ,
  • Tammo Delhaas, MD, PhD

      Affiliations

    • University Hospital, Maastricht, The Netherlands
    • ,
  • Jan L.M. Strengers, MD, PhD

      Affiliations

    • University Medical Center, Utrecht, The Netherlands
    • ,
  • Nico A. Blom, MD, PhD

      Affiliations

    • Center for Congenital Anomalies of the Heart, Amsterdam/Leiden, The Netherlands
    • ,
  • Ad Backx, MD

      Affiliations

    • University Medical Center, Nijmegen, The Netherlands
    • ,
  • Rolf M.F. Berger, MD, PhD

      Affiliations

    • Department of Pediatric Cardiology, University Medical Center, University of Groningen, Groningen, The Netherlands
    • Corresponding Author InformationReprint requests: Rolf M.F. Berger, MD, PhD, Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, PO Box 30 001, 9700 RB Groningen, The Netherlands.

Received 19 November 2008 ,Revised 14 January 2009 ,Accepted 13 February 2009.

References 

  1. Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):5–12S
  2. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004;25:2243–2278
  3. Barst RJ, Ivy D, Dingemanse J, Widlitz A, Schmitt K, Doran A, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003;73:372–382
  4. Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart. 2009;95:312–317
  5. Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46:697–704
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  9. Schulze-Neick I, Beghetti M. Classifying pulmonary hypertension in the setting of the congenitally malformed heart—cleaning up a dog's dinner. Cardiol Young. 2008;18:22–25
  10. van Albada ME, Berger RM. Pulmonary arterial hypertension in congenital cardiac disease—the need for refinement of the Evian-Venice classification. Cardiol Young. 2008;18:10–17
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  12. Andrews R, Tulloh R, Magee A, Anderson D. Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease?. Pediatr Cardiol. 2002;23:528–530
  13. Tulloh RM. Congenital heart disease in relation to pulmonary hypertension in paediatric practice. Paediatr Respir Rev. 2005;6:174–180
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  18. Harrison RE, Berger R, Haworth SG, Tulloh R, Mache CJ, Morrell NW, et al. Transforming growth factor-beta receptor mutations and pulmonary arterial hypertension in childhood. Circulation. 2005;111:435–441
  19. Cua CL, Blankenship A, North AL, Hayes J, Nelin LD. Increased incidence of idiopathic persistent pulmonary hypertension in Down syndrome neonates. Pediatr Cardiol. 2007;28:250–254
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 R.B. has served on the advisory boards of Actelion Pharmaceuticals and GlaxoSmithKline, Gilead.

PII: S0022-3476(09)00157-7

doi: 10.1016/j.jpeds.2009.02.036

The Journal of Pediatrics
Volume 155, Issue 2 , Pages 176-182.e1 , August 2009

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