Excerpt

31-year old male, with no significant family history presented with shortness of breath on exertion for last 2 years. On examination patient had decreased pulse and blood pressure in left radial artery (right upper limb 190/40, left upper limb 110/40). He had early diastolic decrescendo murmur in neoaortic area occupying two-third of diastole. ECG revealed sinus tachycardia with LAE, LVH and LV strain. Chest X-ray showed cardiomegaly, atretic right pulmonary artery and calcified aorta. Echocardiographic examination revealed anomalous LCx crossing aorta (crossed aorta sign), single left pulmonary artery, severe aortic regurgitation with dilated left ventricle and regional wall motion abnormality in basal and mid inferior and inferoseptal segments. He was taken for coronary angiography for evaluation of coronary anatomy which revealed anomalous LCx arising from right coronary cusp and was dilated with fistulous drainage into right lung. His CT and MR angiography revealed porcelain aorta with active large vessel vasculitis which involved entire thoracic aorta, arch vessels, abdominal aorta, left renal artery and complete thrombosis of right pulmonary artery with multiple MAPCA and collaterals formation including one large collateral from left circumflex coronary artery (Fig. 1). He was diagnosed as case of Takayasu arteritis and managed with steroids and methotrexate.

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