The Indian Journal of Pediatrics

, Volume 78, Issue 11, pp 1417–1419

Evaluation of Pulmonary Hypertension in a Child: Role of Computed Tomography

  • Saurabh Kumar Gupta
  • Anita Saxena
  • Gurpreet Singh Gulati
Clinical Brief


Unexplained pulmonary hypertension in pediatric patient is a diagnostic challenge. The natural history as well as management depends upon etiology of pulmonary hypertension. Despite newer insights in pathophysiology and management strategies, the outcome of pulmonary veno-occlusive disease remains dismal. The author’s report a case of 12-year-old girl who presented with severe pulmonary hypertension. Stepwise evaluation and CT angiographic assessment in the index case led to definitive diagnosis of pulmonary veno-occlusive disease. This case highlights the role of cardiac imaging in localising site of pulmonary hypertension. It is important for health care professionals involved in care of patients with pulmonary hypertension to make correct diagnosis of pulmonary veno-occlusive disease for better prognostication as well as to avoid therapeutic mishaps.


Pulmonary hypertension Idiopathic pulmonary arterial hypertension Pulmonary veno-occlusive disease 


  1. 1.
    Frazier AA, Franks TJ, Mohammed TL, Ozbudak IH, Galvin JR. From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 2007;27:867–82.PubMedCrossRefGoogle Scholar
  2. 2.
    Galiè N, Hoeper M, Humbert M, Torbicki A, Vachiery J-L, Barberá JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2009;30:2493–537.Google Scholar
  3. 3.
    Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.PubMedCrossRefGoogle Scholar
  4. 4.
    Runo JR, Vnencak-Jones CL, Prince M, et al. Pulmonary venoocclusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003;167:889–94.PubMedCrossRefGoogle Scholar
  5. 5.
    Holcomb Jr BW, Loyd JE, Ely EW, et al. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118:1671–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Cohn RC, Wong R, Spohn WA, Komer M. Death due to diffuse alveolar hemorrhage in a child with pulmonary veno-occlusive disease. Chest. 1991;100:1456–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Kuroda T, Hirota H, Masaki M, et al. Sildenafil as adjunct therapy to high-dose epoprostenol in a patient with pulmonary venoocclusive disease. Heart Lung Circ. 2006;15:139–42.PubMedCrossRefGoogle Scholar
  8. 8.
    Hoeper MM, Eschenbruch C, Zink-Wohlfart C, et al. Effects of inhaled nitric oxide and aerosolized ioprost in pulmonary venoocclusive disease. Respir Med. 1999;93:62–4.PubMedCrossRefGoogle Scholar
  9. 9.
    Palmer SM, Robinson LJ, Wang A, et al. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest. 1998;113:237–40.PubMedCrossRefGoogle Scholar
  10. 10.
    Overbeek MJ, van Nieuw Amerongen GP, Boonstra A, Smit EF, Vonk-Noordegraaf A. Possible role of imatinib in clinical pulmonary veno-occlusive disease. Eur Respir J. 2008;32:232–5.PubMedCrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2011

Authors and Affiliations

  • Saurabh Kumar Gupta
    • 1
  • Anita Saxena
    • 1
  • Gurpreet Singh Gulati
    • 2
  1. 1.Department of CardiologyAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of Cardiovascular and Interventional RadiologyAll India Institute of Medical SciencesNew DelhiIndia

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