Pulmonary hypertension without associated congenital heart defect is uncommon in children. When it occurs, pulmonary hypertension is usually caused by pulmonary arterial involvement and only rarely will primary pulmonary veno-occlusion be found. The symptom in pulmonary veno-occlusive disease is progressive "breathlessness," dyspnea on exertion.1-3
We studied a case of pulmonary veno-occlusive disease whose main symptom was severe, recurrent abdominal pain. As abdominal pain is a common childhood complaint, the associated increasing dyspnea was over-looked and the diagnosis of pulmonary veno-occlusive disease not immediately made.
Report of a Case.—A 6-year-old girl was referred for evaluation of recurrent abdominal pain. She had always been healthy and active. For several months she had had episodes of severe abdominal pain after an upper respiratory tract infection. This pain was sudden, severe, and she would turn pale. She complained that her heart pounded and her breathing was rapid. Sometimes she vomited and was
WEINBERG HD, KASSEL S. Pulmonary Veno-Occlusive Disease Appearing as Acute, Recurrent Abdominal Pain. Am J Dis Child. 1980;134(1):86–87. doi:10.1001/archpedi.1980.02130130068020
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