DISCUSSION
Only five adult cases of congenital LMS atresia have been reported previously in the literature, and interestingly, one case was only diagnosed on autopsy1 2. This handful of adult cases have confirmed that strong collateralization of the arterial tree let them skip a turn up at an early age.3, 4The range of symptoms vary from being completely asymptomatic to overt heart failure.5 Important differential diagnoses also include origin of the LMS from the pulmonary artery and endomyocardial fibrosis.3,6
Our case report depicts the chain of investigations which are required for diagnosis of coronary atresia and determination of the culprit lesions jeopardizing heart muscle function. Kate et.al emphasized the limited role of invasive coronary angiography in this important diagnosis. Cardiac MR angiography and multi-slice computed tomography (MSCT) gives a 3d visualization and are potentially more powerful modalities to identify this diagnosis. 7
In the present case report, directed investigations played an essential role in committing to the correct treatment. Whereas gated CT angiography provided the actual coronary anatomy, stress echocardiography determined the true origin of ischemia and guided subsequent therapy. This suggests that, in the case of demonstrable myocardial compromise, prompt revascularization is the optimal therapy for patients with LMS atresia.1