PMC4578064_01
Chest PA films. Note the markedly enlarged heart size with prominently dilated both pulmonary arteries and centralization of pulmonary hilum, suggestive of pulmonary arterial hypertension.
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The chest PA showed markedly enlarged cardiac size with prominently dilated main pulmonary artery and centralization of pulmonary hilum, suggesting pulmonary arterial hypertension (Figure 1).
Chest CT scan with contrast. Markedly dilated pulmonary trunk and pulmonary arteries (Figure 2A). There are a concave disfigurement of the left side of the ascending aorta (closed arrow) and just beside, the focally occluded left main coronary artery (open arrow) that was retrogradly contrasted by collateral circulation from the right coronary artery (Figure 2B)
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Chest CT scan with contrast revealed markedly dilated pulmonary trunk and both pulmonary arteries (Figure 2A), a concave disfigurement of the left side of the ascending aorta and nearly total occlusion the ostium of the left main coronary artery that was retrogradly filled with collateral circulation from the right coronary artery (Figure 2B).
Simultaneous intracardiac pressure monitoring of right ventricle (RV) and aorta revealed severe increase of RV systolic pressure which was much higher than systolic aortic pressure.
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Cardiac catheterization revealed PASP of 105 mmHg, Qp/Qs of 1 and Rp/Rs of 1.4 (Figure 3), which mean no remaining shunt but much aggravated pulmonary hypertension compatible with Eisenmenger syndrome compared with preoperative data.
The coronary angiography for the right coronary artery (RCA) showed normal appearance with grade III collaterals to the left descending artery (LAD) and the left circumflex artery (LCX) up to the proximal left main coronary artery.
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The coronary angiography showed normal right coronary artery and the collaterals that came out from the conus branch to mid-left anterior descending artery (LAD) and that from the distal right coronary artery to the left circumflex artery (LCX) and to the distal LAD, respectively (Figure 4, 5).
The selective angiography of the conus branch of the right coronary artery showed collaterals to the left anterior descending coronary artery (LAD) of grade III that looked like a bird-beak during systole due to pulsating dilated pulmonary artery (arrow).
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The coronary angiography showed normal right coronary artery and the collaterals that came out from the conus branch to mid-left anterior descending artery (LAD) and that from the distal right coronary artery to the left circumflex artery (LCX) and to the distal LAD, respectively (Figure 4, 5).
Aortography on the left anterior oblique view revealed only the right coronary artery (RCA) without stump of the left coronary artery, suggestive of the total occlusion of the ostium of the left main coronary artery.
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On aortography, the left main coronary artery was not visualized with no stump, suggesting total occlusion of the ostium of the left main coronary artery (Figure 6).