LAUSR

Critical Care Medicine • Volume 46 • Number 1 (Supplement) www.ccmjournal.org Learning Objectives: Isolated unilateral absence of pulmonary artery is a rare congenital anomaly. Many of these patients survive into adulthood with only minor symptoms. It is more common for this anomaly to be associated with other congenital heart anomalies. Massive hemoptysis is rare in pediatrics Methods: We describe a 13-month-old female with no significant past medical history who presented with hemoptysis. Parents reported that she had 4 episodes of coughing blood in the last 5 months. Upon arrival to the emergency room (ER), her body temperature was 36 °C, pulse rate 156/minute, blood pressure 104/64 mmHg and SpO2 of 95% on supplemental oxygen. While in the ER, she began coughing blood, and then she was urgently intubated for acute respiratory failure and hypoxia. Patient was resuscitated and stabilized; she received blood transfusion and transferred to the Pediatric Intensive Care Unit (PICU). Initial work up including bronchoscopy and upper endoscopy did not reveal the source of the bleeding. CT of the Chest revealed the absent right pulmonary artery (RPA). She stayed intubated for 2 days during which there was no more hemoptysis reported with stable hematocrit (Hct) at 22%. She then was successfully extubated. Echocardiogram showed normally structured heart, normal biventricular function, and no pulmonary hypertension. Cardiac catheterization showed small remnant of the right-sided Patent Ductus Arteriosus (PDA), and a distal very small right RPA, with small collaterals. The decision was made not to do any surgical intervention and to continue to monitor closely for any further episodes of hemoptysis or the development of pulmonary hypertension. Patient was sent home. She presented 2 months later with a mild episode of hemoptysis that resolved spontaneously, with Hct of 32%. She was observed in PICU overnight before going home Results: Physicians should be aware of this anomaly especially in patients with recurrent episodes of lower respiratory tract infection and hemoptysis. Patients with this anomaly tend to have good prognosis, but close follow up is necessary to monitor for complications