Abstract
IntroductionDisconnected pulmonary arteries are unusual and may result in pulmonary hypertension with acute right heart failure.Case presentationWe report a case of a three-month-old Asian girl who presented with heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery. An epoprostenol (prostacyclin) infusion was instrumental in lowering pulmonary artery pressures and stabilizing the child prior to surgery.ConclusionsThis is, to the best of our knowledge, the first report of successful prostacyclin usage in such a situation.
Highlights
Disconnected pulmonary arteries are unusual and may result in pulmonary hypertension with acute right heart failure.Case presentation: We report a case of a three-month-old Asian girl who presented with heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery
We report a three-month-old infant who presented in heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery in the absence of conotruncal anomalies
An epoprostenol infusion was instrumental in lowering pulmonary artery pressures and stabilizing the child prior to surgery
Summary
Disconnected pulmonary arteries are unusual, and are almost invariably associated with conotruncal abnormalities [1]. We report a three-month-old infant who presented in heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery in the absence of conotruncal anomalies. Case presentation Our patient was a three-month-old Asian girl of English nationality, born through normal vaginal delivery at full term to healthy and unrelated parents after an uneventful pregnancy. Examination showed irritability of the child, with respiratory distress, subcostal retraction and saturations of 80% to 85%, which improved marginally with nasal prong oxygen.
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