Large Pulmonary Artery Aneurysm Rupture in Hughes-Stovin Syndrome
Multidetector Computed Tomography Pattern and Endovascular Treatment
A 42-year-old man with a history of recurrent pulmonary embolism was admitted to the respiratory intensive care unit of our hospital for massive hemoptysis (600 mL in one time). The multidetector computed tomography angiography (MDCTA) showed a large aneurysm originating from the middle lobe pulmonary artery with an air bubble in the aneurysm sac (Figure, A) and enlargement of systemic bronchial and nonbronchial arteries (Figure, B). The MDCTA showed 5 aneurysms of the pulmonary arteries at the right lower lobe (n=2), left upper lobe (n=1), and left lower lobe (n=2). Percutaneous vaso-occlusion of the aneurysm in the pulmonary artery of the middle lobe was performed (Figure, C). Cardiac MDCT indicated a thrombus in the right atrium (Figure, D) and an anomalous origin of the right coronary artery. Hughes-Stovin syndrome (HSS) was diagnosed on the basis of the association of multiple pulmonary arteries aneurysms, pulmonary embolism, and thrombus into the right atrium. Immunosuppressive therapy was started. The clinical condition of the patient improved at the 4-month follow-up.
The typical presentation of HSS involves in 3 phases: a first stage involving symptoms of thrombophlebitis, a second stage consisting of formation and enlargement of pulmonary aneurysms, and a third stage of aneurysmal rupture that triggers massive hemoptysis, leading to death. This case illustrates the endovascular treatment option for large pulmonary artery aneurysm rupture in HSS.