Hemothorax secondary to pulmonary AVM: A near-fatal presentation with difficult management.
American Journal of Respiratory and Critical Care Medicine
Introduction: Non-heritable pulmonary arteriovenous malformation (AVM) is rare, but can lead to life-threatening presentations, including hemothorax. Our case demonstrates a near-fatal presentation of hemothorax from ruptured pulmonary AVM managed with surgical approach secondary to diagnostic uncertainly and critical patient status. Case Report: A 37-year-old male without significant medical history presented following syncopal episode. On initial evaluation he was hypoxic and in shock, with decreased left sided breath sounds. Workup revealed a large left sided pulmonary pleural effusion with rightward shift on chest radiograph. Labs demonstrated anemia and acute kidney injury. He was resuscitated with intravenous fluids and vasopressors, then transferred to ICU. A non-contrast CT Chest showed left sided hemothorax and a 3.8 x 2.9 x 3.6 cm left upper lobe density, adjacent to the pericardium. Diagnostic and therapeutic thoracentesis were performed, yielding 1700cc of bloody output. CT angiogram narrowed the differential to pulmonary AVM versus bronchial artery aneurysm. Interventional radiology (IR) pulmonary angiogram (Figure) suggested pulmonary artery aneurysm versus pseudoaneurysm versus AVM. Technical difficulties made definitive IR management impossible. The patient was taken for urgent surgical control. A 4-cm, pulsatile, lingular nodule was discovered and successful lingular segmentectomy performed. The patient made a full recovery. Pathology returned as consistent with pulmonary AVM. Discussion: Pulmonary AVM represent an abnormal vascular connection between pulmonary vessels. They are uncommon, acquired or congenital, with majority associated with Hereditary Hemorrhagic Telangiectasia. Our patient had lack of evidence for heritable disease. Life-threatening complications of AVM include hemorrhagic sequalae related to rupture. Rupture of pulmonary AVM, as a cause of hemothorax, is extremely rare. While transcatheter embolization remains the current treatment of choice, clinical circumstances as in the presented case may necessitate a surgical approach. Currently, there are no clear indications for transcatheter embolization over surgical resection. Literature review reveals that surgical management of hemothorax secondary to AVM is rare. Only one case, aside from the currently presented, was managed with segmentectomy. This case demonstrates the benefits of multidisciplinary critical care. Conclusion: Pulmonary AVM can present with life-threatening complications, including rupture and hemothorax. Although interventional radiologic management of AVM rupture is current standard of care, the case complexity does not always allow for this management. Diagnostic uncertainly and severity of presentation are instrumental to management decision. The presented case exemplifies a multidisciplinary approach to a rare and near-fatal presentation of spontaneously ruptured non-heritable pulmonary AVM, successfully managed with segmentectomy.
Krater R, Figueroa F, Abbot E, Bozyk P. Hemothorax secondary to pulmonary AVM: A near-fatal presentation with difficult management. American Journal of Respiratory and Critical Care Medicine 2019;199:A3620