We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.
Computerized tomography-coronary angiography (CT-CAG) is gaining popularity as an alternative to conventional CAG to evaluate grafts in a post-coronary artery bypass graft (post-CABG) patient, since it is a noninvasive procedure and is less influenced by cardiac motion. The primary challenge is to image a rapidly beating heart. With introduction of 64-slice scanner, the coronary imaging became a possibility with acceptable accuracy. In recent years, with the development of 128,256 and 320 multislice CT scanners, further enhancement in the temporal and spatial resolution is achieved due to lesser influence of the respiratory and cardiac motion, enhancing the accuracy of the lesion assessment in the grafts and the native vessels. Achieving low heart rate and artifact-free image acquisition, proper reconstruction, and image interpretation are challenges to the radiologists and the technicians involved in coronary imaging. Women pose special subset because of smaller sized coronary vessels, interference due to breast shadows, low-referrals, and gender-specific reluctance to accept the procedure itself. Cardiologists and radiologists caring for these patients must be familiar with the pros and cons of CT-CAG and gender-specific challenges.
Objectives: The aim of the study was to analyze the utility of CT-coronary angiogram (CT-CAG) in assessment of long-term graft patency compared to conventional invasive coronary angiography (ICA) and highlight any gender-specific issues in female post-coronary artery bypass surgery (CABG) patients. Materials and Methods: The medical records and images of 30 patients including four female patients who had CABG in the past and underwent both conventional and CT-CAGs were analyzed retrospectively. Results: We studied 30 patients who had CABG in whom both CT-CAG and conventional coronary angiograms were performed. CT-CAG was able to evaluate the grafts better than ICA and was useful even in those which could not be assessed due to technical failure by ICA. There were only four female patients in this series. In first patient, the arterial graft (left internal mammary artery [LIMA]) was patent but distal run off was absent in LAD in CT-CAG images and was reported as poor in conventional coronary angiogram. The second patient had CABG 10 years back. The arterial graft (LIMA) to LAD was patent and there was total block in SVG graft to RCA at proximal anastomotic site. The third patient with hypertension and diabetes also had CABG 10 years back. Both LIMA to LAD and SVG to PDA were patent in her. In last case who had CABG 7 years, two of the three SVG grafts were blocked which were well-demonstrated on CT CAG, including one SVG missed on conventional CAG. Conclusion: CT-CAG is a non-invasive and less cumbersome alternative to conventional CAG for the assessment of grafts long after CABG even in women. The data generated by CT-CAG in post-CABG are as good as the invasive CAG and it has distinct advantage of greater acceptability.
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