Int J Cardiovasc Imaging (2015) 31:427–428 DOI 10.1007/s10554-014-0549-y
Tetralogy of Fallot with double aortic arch and discontinuous left pulmonary artery Ananth Ravi • Shilpa Hegde • Bruce Greenberg
Received: 1 October 2014 / Accepted: 6 October 2014 / Published online: 16 October 2014 Springer Science+Business Media Dordrecht 2014
Abstract We report a case of Tetralogy of Fallot with double aortic arch and discontinuous left pulmonary artery, supplied by the ductus arteriosus, a rare entity.
Case presentation A newborn was evaluated for murmur. On physical examination, cyanosis with oxygen saturation of 70 %, grade 2-3/4 murmur, bounding symmetric pulses in both extremities and hepatomegaly were noted. Echocardiography demonstrated tetralogy of Fallot and double aortic arch with dominant left arch. In addition, left pulmonary artery was discontinuous to the main pulmonary artery and is supplied by a PDA. Chest CTA (Figs. 1, 2) was performed to further evaluate the double aortic arch and the pulmonary arteries.
Discussion/teaching points •
Vascular rings comprise up to 1 % of congenital heart disease and TOF accounts for 3.5 % of congenital heart disease. Though right aortic arch is commonly associated with tetralogy of Fallot, a double aortic arch is a rare association . There are 2 reported cases of discontinuous left pulmonary artery, arising from the ductus in association with tetralogy of Fallot . 2 cases of absent left pulmonary artery and double aortic arch with Tetralogy of Fallot have been described in the English literature . An association of left pulmonary artery originating from the ductus with a double aortic arch in a case of Tetralogy of Fallot was not found in the literature.
A. Ravi (&) Indiana University School of Medicine, Indianapolis, IN, USA e-mail: [email protected]
S. Hegde B. Greenberg Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Int J Cardiovasc Imaging (2015) 31:427–428
Fig. 2 Posterior view of 3D reformatted image of CT chest demonstrates the double aortic arch Fig. 1 Multiplanar reformatted CT of chest with IV contrast shows the overriding aorta with a ventricular septal defect (arrow) and the ductus supplying the discontinuous left pulmonary artery (arrow head)
Conflict of interest
1. Li ZQ, Liu AJ, Li G, Liu Y, Zhu YB, Liu YL (2013) Two cases of a rare association: double aortic arch with tetralogy of Fallot. J Cardiothorac Surg 8:39 2. Ak K, Arsan S, Akalın F, Gursu O, Isbir S (2011) Autologous right pulmonary artery tissue for repair of left pulmonary artery originating from left patent ductus arteriosus in a patient with tetralogy of Fallot and absent pulmonary valve. Pediatr Cardiol 32(7):986–989 3. Talwar S, Gupta A, Choudhary SK, Airan B (2009) Absent left pulmonary artery and double aortic arch in tetralogy of Fallot: reconstruction using homograft saphenous vein or iliac artery. Interact CardioVasc Thorac Surg 8(2):277–279
Double aortic arch represents persistence of the embryologic left and right fourth aortic arches. This is a common cause for vascular rings and can lead to stridor or dysphagia if unrecognized. In patients with structural cardiac anomalies, echocardiographic evaluation with suprasternal fossa short axis and long axis views to evaluate the aorta is warranted to rule out arch anomalies. Suspicious findings on echocardiography need CT or MRI evaluation for confirmation of arch abnormalities.