SURGICAL TREATMENT OF PULMONARY ARTERY AGENESIS

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Unilateral pulmonary artery agenesis is an extremely rare disorder, presenting in adulthood with life-threatening hemoptysis, exertional dyspnea, and recurrent respiratory infections. The optimal treatment strategy has not been well defined. For significant hemoptysis, embolization of aorto-pulmonary collaterals is commonly performed, although the effect is often temporary. Pneumonectomy is usually avoided due to risk of intraoperative bleeding associated with interruption of systemic collaterals; we hypothesized that preoperative embolization followed by pneumonectomy might afford a safe treatment strategy. We present a single institution’s experience with three cases of pulmonary artery agenesis treated successfully with a standardized program of systemic collateral embolization followed by pneumonectomy. CASE PRESENTATION: This series describes three consecutive patients with symptomatic pulmonary artery agenesis treated in a uniform fashion over 11 years. Average age was 48 years, with 2:1 male predominance. Two patients presented with massive hemoptysis, while the other presented with recurrent, progressive infections of the involved lung due to Mycobacterium abscessus. In all cases, optimal conservative therapy failed resulting in recurrent symptoms. CT imaging with vascular contrast was performed to confirm the diagnosis. In the case of infection, sputum analysis and appropriate antibiotic treatment was initiated. All patients underwent arterial contrast studies and selective preoperative embolization of aorto-pulmonary collaterals. Pneumonectomy was then performed via thoracotomy. There was no operative mortality or blood transfusion, and the average length of stay was 6 days. In follow-up, all patients remain symptom free. DISCUSSION: Due to the rare nature of pulmonary artery agenesis, no consensus exists regarding optimal management. In patients with hemoptysis, embolization of the hypertrophied systemic collaterals is an appropriate first treatment strategy, although associated with high rates of recurrence. Further, repeated attempts at embolization subject the patient to the rare but devastating complications of stroke and spinal cord ischemia. Hypertrophied aorto-pulmonary collaterals present a significant surgical challenge with uncontrolled blood loss following pneumonectomy. This series presents three patients successfully treated with preoperative embolization followed by planned pneumonectomy, none of which had long-term complications and all reported significant symptomatic improvement. This treatment paradigm may provide a safe, definitive option for patients with recurrent symptoms after embolization. CONCLUSIONS: Pulmonary artery agenesis is an extremely rare condition causing life-threatening symptoms in the majority of affected adult patients. We describe a standardized approach, including indications for operation, for three patients with successful outcomes. Reference #1: Ten Harkel, A. D. J., Blom, N. A., & Ottenkamp, J. (2002). Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest, 122(4), 1471-1477. Reference #2: Kruzliak, P., Syamasundar, R. P., Novak, M., Pechanova, O., & Kovacova, G. (2013). Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Archives of cardiovascular diseases, 106(8-9), 448-454. DISCLOSURES: No relevant relationships by Bryn Launer, source=Web Response No relevant relationships by Robert Meguid, source=Web Response Scientific Medical Advisor relationship with Medtronic Please note: $1001 - $5000 Added 03/18/2019 by John Mitchell, source=Web Response, value=Honoraria No relevant relationships by Christopher Scott, source=Web Response No relevant relationships by Stephanie Serva, source=Web Response Consultant relationship with medtronic Please note: $1001 - $5000 Added 03/22/2019 by Michael Weyant, source=Web Response, value=Consulting fee