Summary: A 4-year-old boy with Tetralogy of Fallot (TOF, Figure 1), pulmonary atresia (PA) and major aorto-pulmonary collateral arteries (MAPCAs) was referred to the radiology department of Sakakibara Heart Institute for a follow-up examination using a SOMATOM Definition Flash, Dual Source CT in Flash Spiral mode following treatment of his pulmonary artery stenosis.
The patient was diagnosed shortly after birth with TOF, PA, MAPCA. When he was 10 months old, a stent was inserted in the largest MAPCA and a central shunt was placed when he was 16 months old. When he was 2 years old, he underwent right and left modified Blalock-Taussig shunt surgeries (therefore, the subclavian artery was connected with the pulmonary artery) within 9 months. Then, at the age of 3, an artificial vessel was constructed from the right ventricle (RV) to the pulmonary artery by palliative Rastelli procedure.
The patient underwent a percutanous transluminal angioplasty (PTA) of the pulmonary artery. A low-dose Dual Source CT scan in the Flash Spiral mode was ordered to confirm his postoperative condition, in particular, concerning the pulmonary circulation. The patient’s weight was 15.6 kg (34.39 lb). He was not sedated and no breath-hold was needed during the scan. His mean heart rate was 95 bpm.
The right coronary artery (RCA) was found to originate from the aorta at the upper level of left coronary artery.
The Dual Source CT images showed that the RV-pulmonary artery conduit was patent and that the anastomosis site had no stenosis. Neither the right nor the left pulmonary arteries (about 4 mm in diameter) presented any significant stenosis (Figure 2).
A stent was confirmed in the biggest MAPCA, which bifurcated from the descending aorta at the level of the left atrium. It went to the right superior and inferior lung lobes, and connected one artery originating from the right central pulmonary artery. Although the stent itself was patent, a stenotic part was seen distal of the stent (Figure 3).
The Dual Source CT images revealed a tortuous artery originating from a right subclavian artery that supplied the right and left inferior lung lobes. The left lung was perfused mainly by the left central pulmonary artery. The right middle lung lobe was perfused by the large right inferior diaphragmatic artery (its distal end was connected to an artery originating from the central pulmonary artery).
Incidentally, the right coronary artery (RCA) was found to originate from the aorta at the upper level of left coronary artery, the left coronary cusp (Figure 4), which could neither be seen in the previously performed catheter angiography nor in a 16-MSCT examination. Based on these findings, a catheter PTA of the pulmonary artery stenosis at the distal part of the stent was planned.
Dual Source CT angiography has emerged as an essential diagnostic tool for the assessment of complex congenital heart disease. Nevertheless, dose has remained a concern, in particular when referring pediatric patients for cardiac CT. With the Flash Spiral mode of the second generation Dual Source CT, pediatric patients can be scanned at ultra low dose, as in this case at 1.63 mGy (effective dose 0.644 mSv). Apart from dose concerns, additional challenges have been associated with imaging pediatric congenital heart disease patients: the patients have high heart rates, the cardiac vessels are tiny, sedation often presents a risk and most patients cannot hold their breath.
This Dual Source CT Flash scan of 211-mm in length was taken in only 0.51 sec without sedation or breath-hold. Vessels were clearly visualized without artifacts. Even coronary anomaly could be seen despite the patient’s high heart rate of 95 bpm. Pulmonary artery in-stent stenosis could also be evaluated. The Dual Source CT Flash images were extremely helpful for further treatment planning.