Abstract
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.
References
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.
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.
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.
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.
Holcomb Jr BW, Loyd JE, Ely EW, et al. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118:1671–9.
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.
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.
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.
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.
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.
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Gupta, S.K., Saxena, A. & Gulati, G.S. Evaluation of Pulmonary Hypertension in a Child: Role of Computed Tomography. Indian J Pediatr 78, 1417–1419 (2011). https://doi.org/10.1007/s12098-011-0471-4
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DOI: https://doi.org/10.1007/s12098-011-0471-4