INTRODUCTION:Bronchial artery ectasia (BAE), also known as bronchial artery malformation, is a rare anomaly of the bronchial artery. We report a case of BAE complicated by a fistulous shunt between the bronchial artery and the pulmonary venous system.
CASE PRESENTATION:A 21-year-old white female with a strong family history of Marfan’s syndrome was referred to our institution for evaluation of abnormal chest radiographs. She was seen by a neurologist for the evaluation of heachache, which was ultimately felt to be migraine in nature. These headaches were occasionally associated with chest pain, and a chest radiograph was obtained. The chest radiograph showed a well circumscribed 2.4 cm tortuous nodule in the right lower lung adjacent to the right main pulmonary artery. This abnormality seen on the chest radiograph led to CT scan and subsequently angiography of both the pulmonary and bronchial arterial systems. The patient was diagnosed with bronchial artery ectasia. Moreover, a fistulous connection between the right bronchial artery and pulmonary vein was surprisingly discovered.
DISCUSSIONS:BAE is a rare anomaly of the bronchial artery. We chose to use this term given its large size and tortuosity noted on chest imaging, as previous reports of vessel caliber of this magnitude have not been described. Further, the presence of a fistulous connection between the bronchial artery and the pulmonary venous system itself is extremely rare, which in most cases requires interruption to minimize complications.The pathophysiology of a left to left systemic shunt results in a relatively high flow state through the systemic circulation. This increase in flow over time may result in continued vessel enlargement, increased left to left shunting, and stagnant blood flow with resultant thrombus formation. This, in turn, may result in potential vessel rupture, high output cardiac failure, and thromboemboli formation. Chest radiography is of limited value. A contrast enhanced CT scan of the thorax will help delineate anatomy. CT enhanced, 3D reconstruction images may further enhance detail. The gold standard for diagnosis, however, remains arteriography of the bronchial circulation. Treatment is focused on prevention of complications, and it is therefore recommended that management be focused on shunt interruption via embolization or surgical correction.
CONCLUSION:Bronchial artery ectasia alone or in combination with fistulous shunt formation is extremely rare. Physicians should be aware of its life threatening complications related to an arterial shunt formation. If suspected, a bronchial arteriogram should be performed. If a vascular shunt is detected, treatment should be considered early to minimize potential risks associated with progressive vessel enlargement.
DISCLOSURE:Maulik Patel, No Financial Disclosure Information; No Product/Research Disclosure Information