Tuberculous Pseudoaneurysms of the Thoracic Aorta
Comprehensive Evaluation by Cardiovascular Magnetic Resonance
A 16-year-old girl from an area in Brazil where tuberculosis was endemic was admitted because of recurrent chest pain, progressive dyspnea with exertion, and inability to climb more than 1 flight of stairs. She described fatigue, weight loss, night sweats, and adenopathy of her right axilla and neck during the previous 3 months. On admission she looked distressed, with a heart rate of 76 bpm and blood pressure of 110/55 mm Hg. The ECG demonstrated sinus rhythm with first-degree heart block (Figure 1).
Her admission chest radiograph demonstrated cardiomegaly with pulmonary edema (Figure 2). Her cardiac enzymes were negative. The patient underwent cardiac evaluation with 2-dimensional echocardiography (not shown), which revealed an interatrial septal mass, mild tricuspid regurgitation, preserved systolic function, and moderate pericardial effusion.
For further characterization of the mass, cardiovascular magnetic resonance imaging (MRI) was performed. Coronal black-blood T2-Turbo Spin Echo and T2-Short T1/Tau Inversion Recovery images of the chest showed adenopathy of the right axilla and a multicystic mass located on the posterior mediastinum (Figure 3). Four-chamber view cine-MRI demonstrated mitral and tricuspid regurgitation, pericardial effusion, and an interatrial septum mass (Figure 4A and 4B). A large retrocardiac pulsatile lesion involving the distal thoracic aorta was also noted (Figure 4 and Movie I of the online-only Data Supplement). Delayed-enhancement MRI confirmed the rounded interatrial septal mass with no evidence of enhancement, consistent with a hematoma of the interatrial septum (Figure 4C), possibly secondary to a leaking aortic root pseudoaneurysm (Figure 5B and Movie II of the online-only Data Supplement).
Time-resolved contrast-enhanced 3-dimensional magnetic resonance angiography demonstrated multiple pseudoaneurysms extensively distributed throughout the ascending and descending thoracic aorta. Longitudinal aorta and 4-chamber views further depicted partially thrombosed pseudoaneurysms, extensive thickening, and late enhancement of the thoracic aortic wall (Figures 4C and 5⇑), indicating a likely inflammatory cause.
Excisional biopsy of her palpable cervical node was performed after her purified protein derivative test proved to be highly reactive (18 mm). The biopsy results demonstrated caseous and noncaseous necrotizing granulomas with positive culture for acid-fast bacilli (Figure 6).
The patient was given an antitubercular 4-medication regimen. Planned surgical intervention decision was precluded when the patient experienced sudden hypovolemic shock and was unresponsive to the standard resuscitatory maneuvers.
Tuberculous pseudoaneurysms of the aorta are exceedingly rare, but the lesion is uniformly fatal if not diagnosed promptly. Typical clinical scenarios include evidence of tuberculous lymph nodes in 70% of cases, with 1 or more of 3 presentations: (1) fever and persistent pain related to the location of the aneurysm, (2) hypovolemic shock or other evidence of massive bleeding, or (3) pulsatile, rapidly expanding para-aortic mass.1 The perforation of the aortic wall is generally surrounded by thrombotic debris and inflammatory tissue.2 Cardiovascular magnetic resonance provides a comprehensive and noninvasive imaging tool for integrated evaluation of the morphology, physiology, and tissue characterization of the heart and great vessels. Combined antitubercular chemotherapy and surgical intervention seem to offer the best chance for a cure and constitute the only way to salvage patients with this disease.1
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/120/24/e285/DC1.