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487 A 17-YEAR-OLD FEMALE WITH PULMONARY CAPILLARY HEMANGIOMATOSIS AND PULMONARY VENO-OCCLUSIVE DISEASE.
  1. M. White,
  2. D. Hsing,
  3. J. Brill,
  4. D. Epstein
  1. Mattel Children's Hospital at UCLA, David Geffen School of Medicine; Los Angeles, CA

Abstract

A 17-year-old female with pulmonary hypertension, presenting with headache, cough, and worsening hypoxemia, was admitted to the pediatric intensive care unit (PICU) for observation. She was diagnosed with pulmonary hypertension 9 months prior to admission after several months of worsening shortness of breath and chest pain. Six months prior to admission, she was started on bosentan and sildenafil, after a cardiac catheterization revealed significantly elevated pulmonary artery pressures. She had also been on home oxygen therapy and was a NYHA Class IV heart-lung transplant candidate. Upon admission to the PICU, pulse oximetry revealed 75% saturation on high flow face-mask oxygen. Her physical exam was suggestive of right heart failure, for which a milrinone infusion was started. A chest CT angiogram performed on the day of admission was negative for pulmonary thrombosis. Cardiac catheterization performed on the second hospital day revealed suprasystemic pulmonary artery pressures. Unfortunately, her cardiac output declined and she progressed to acute renal failure. Cardiopulmonary arrest occurred shortly thereafter. Although she was resuscitated, she suffered severe hypoxic-ischemic brain injury. The family subsequently decided to withdraw support. The postmortem examination of the lungs revealed markedly increased numbers of capillary blood vessels and numerous hemosiderin-laden macrophages consistent with the diagnosis of pulmonary capillary hemangiomatosis. Microscopic evidence of venous fibrosis suggested a concomitant diagnosis of pulmonary veno-occlusive disease. While more common in the adult population, the diagnoses of pulmonary capillary hemangiomatosis and veno-occlusive disease are extremely rare in children.

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