A 37-year-old lady presented with complaints of acute onset dyspnea and chest discomfort for one day. The patient also gave a history of nasopharyngeal carcinoma managed with surgery and radiotherapy 20 years back.

At presentation, the patient was dyspneic with mild chest discomfort. On examination, she had sinus tachycardia (110 beats/minute), normal blood pressure, and jugular venous pressure elevated till the angle of the mandible. Precordial examination revealed cardiomegaly, left ventricular third heart sound, and an audible pericardial rub. Chest auscultation identified bilateral basal crackles. The electrocardiogram showed sinus tachycardia, normal QRS axis, T wave inversion noted in leads II, III, aVF, and V2-V6. Chest roentgenogram showed cardiomegaly with a cardiothoracic ratio of 0.65 and grade 1 pulmonary venous hypertension. Echocardiography revealed a left ventricular apical pseudoaneurysm with preserved thickness and contractility of the basal and mid-segments of the left ventricle. There were moderate aortic regurgitation and moderate circumferential pericardial effusion with no features of cardiac tamponade. Serial troponin T levels remained below the 99th centile, but the N-terminal pro-brain natriuretic peptide (NT-proBNP) was elevated (2140 pg/ml). Other blood investigations revealed anemia (hemoglobin—8.7 g/dl), elevated erythrocyte sedimentation rate (82 mm/1st hour), elevated C-reactive protein (88.92 mg/L), normal renal and liver function.

Computed tomography showed a wide-necked aneurysm arising from the left ventricular apex with mural thrombus, mild circumferential wall thickening of aortic arch, descending thoracic aorta and proximal great vessels and irregular fusiform aneurysm of left axillary artery. Cardiac MRI confirmed apical pseudoaneurysm of the left ventricle and moderate circumferential pericardial effusion (Fig. 1a). The wall of the aneurysm was thinned out with transmural late gadolinium enhancement suggestive of fibrosis (Fig. 1b). The pericardial effusion was hypointense on T1 imaging suggestive of non-hemorrhagic effusion. Bilateral axillary fusiform aneurysms, outpouching from infra renal abdominal, bilateral common iliac fusiform aneurysms were noted consistent with aortoarteritis (Fig. 2a). Delayed gadolinium enhancement was noted involving the aortic arch, arch vessels, and descending thoracic aorta suggestive of disease activity (Fig. 2b).

a Axial section of the cardiac MRI showing the left ventricular apical aneurysm and moderate circumferential pericardial effusion. Transmural late gadolinium enhancement of the aneurysmal segment is seen in (b)

a Magnetic resonance angiogram showing stenosis of the proximal segments of bilateral subclavian arteries and infrarenal abdominal aorta. Fusiform dilatations of the distal left subclavian artery and bilateral common iliac arteries are also seen. b Late gadolinium enhancement of the aortic walls consistent with active aortoarteritis

Immunology workup reported negative for antinuclear antibody immunoblot profile and antinuclear cytoplasmic antibodies (pANCA and cANCA). Viral serological markers for HIV, HBsAg, HCV were negative. Epstein–Barr virus (EBV) serology was done. The reports were positive for the immunoglobulins IgG and IgM to both viral capsid antigen (anti-VCA) and anti-Epstein–Barr nuclear antigen (anti-EBNA).

A diagnosis of active Takayasu arteritis was made, and patient was started on immunomodulators after an immunology consultation. After control of disease activity, a coronary angiogram was done which revealed normal coronaries. The left ventricular angiogram showed left ventricular dysfunction and an apical aneurysm (Video 1). The patient was taken up for aneurysmectomy and surgical reconstruction (Dor’s procedure). However, the procedure was abandoned after identifying widespread inflammation intraoperatively with friable tissue. Patient was planned for staged re-operation and repair after a course of steroids and azathioprine. Unfortunately, the patient suffered a sudden cardiac arrest on the 10th postoperative day and could not be revived.