Key result
Administration of low-dose epoprostenol in a patient with CREST syndrome and pulmonary capillary hemangiomatosis resulted in flash pulmonary edema and fatal cardiac arrest.
Case Report (n=1)
Administration of epoprostenol can trigger fatal flash pulmonary edema in patients with underlying pulmonary capillary hemangiomatosis.
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Caution advised with epoprostenol in CREST with possible PCH; case report leaves open need for prospective safety data.
This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease.
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McGuire et al. (2010) conducted a case report in Pulmonary Capillary Hemangiomatosis Associated with CREST Syndrome (n=1). Epoprostenol was evaluated on Fatality / flash pulmonary edema and arrest. Administration of low-dose epoprostenol in a patient with CREST syndrome and pulmonary capillary hemangiomatosis resulted in flash pulmonary edema and fatal cardiac arrest.
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