Abstract
We present a case of an ex-30 week premature male infant diagnosed postnatally with Tetralogy of Fallot, hemitruncus, and discontinuous pulmonary arteries (PAs) at 6 days of life. The patient was diagnosed by echocardiography, and the diagnosis was confirmed on subsequent dual-energy chest CT angiogram. In our patient, the left PA arose directly from the aorta, whereas the right PA originated normally from the right ventricular outflow tract. At 9 days of life, he underwent successful surgical palliation with placement of a modified Blalock-Taussig (aortopulmonary) shunt from the base of the left subclavian artery to the anomalously connected left PA along with anastomosis together of the right and left branch PAs to establish continuity with the main PA. Such cases have been described and are rare. The specific aim of this case report is to illustrate the added benefit of dual-energy electrocardiographically-triggered computed tomographic angiography (CTA) along with standard echocardiography. In addition, high quality images useful in preoperative planning were obtained noninvasively using an ultra low radiation dose without the need for sedation. The information obtained proved essential for confirmation of the diagnosis, preoperative planning, and post-surgical monitoring of branch PA development.