Diagnosis of the double aortic arch and its differentiation from the conotruncal malformations

Abstract

Purpose: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases.

Materials and methods: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed.

Results: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnostic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology.

Conclusion: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Fig. 1

Plain chest radiographs showed a bulged contour (arrow) at the right upper cardiac border of a 4.5-month-old male (A), and deviations (arrow) of the trachea in a 16-day-old male (B). Barium esophagograms taken from a 4.5-month-old male showed an indentation (arrow) at the upper third portion of the esophagus, which is located on the left aspect of the frontal projection (C), and the posterior aspect of the lateral projection (D), respectively.

Fig. 2

Subcostal view of the left ventricular outflow tract of echocardiography showed a characteristic morphology of the aortic bifurcations in cases with DAA (A), which has a longer bifurcation distance (from the semilunar valve to the site of bifurcation) than the bifurcation distance measured in cases with d-TGA (B), HTA (C), type I TA (D), and APW (E). Parasternal short-axis view showed an anomalous origin of the right pulmonary artery from the ascending aorta in a case of isolated HTA (F), and when complexed with APW (arrows) in the Berry syndrome (G), as well as an abnormal take-off of the main pulmonary artery from the left-posterior aspect of the aorta in cases with type I TA (H). Over the parasternal long-axis views, we may visualize bifurcation either from the ascending aorta, as in the case of HTA (I), or from the truncus in TA cases (J), which can be similar to d-TGA cases (K). AAo, ascending aorta; APW, aortopulmonary window; DAA, double aortic arch; d-TGA, dextro-transposition of the great arteries; HTA: hemitruncus arteriosus; LA, left atrium; LAA, left aortic arch; LPA, left pulmonary artery; LV, left ventricle; MPA, main pulmonary artery; RA, right atrium; RAA, right aortic arch; RPA, right pulmonary artery; TA, truncus arteriosus.

Fig. 3

Ascending aortography (A) and digital subtraction angiography (B) showed a Y-shaped and bifurcated DAA. The right and the left brachiocephalic arteries were visualized as arising separately from the ipsilateral right aortic arch (RAA) and left aortic arch (LAA). The R1, R2, L1, and L2, in Fig. 3A and 3B, denote the right common carotid artery (1^st branch of the right aortic arch), the right subclavian artery (2^nd branch of the right aortic arch), the left common carotid artery (1^st branch of the left aortic arch), and the left subclavian artery (2^nd branch of the left aortic arch), respectively. (C) In the patient with d-TGA, the left ventriculography showed a discordant ventriculoarterial connection. (D) In the patient with HTA, the ascending aortography showed an anomalous origin of the right pulmonary artery from the ascending aorta. (E and F) In the patient with type I TA, the ascending aortography showed a bifurcation of the main pulmonary artery leftward and posterior to the aorta above the truncal valve. (G) In the patient with APW, type B aortic interruption, VSD, PDA, and DiGeorge syndrome, the countercurrent ascending aortography (with digital subtraction) showed simultaneous opacification of the pulmonary arteries through the aortopulmonary defect (arrows) and interruption of the aortic arch between the left common carotid artery and the left subclavian artery. The Arabic numerals 1, 2, 3, and 4, in Fig. 3G, denote the right subclavian artery, right common carotid artery, left common carotid artery, and left subclavian artery, respectively. AAo, ascending aorta; APW, aortopulmonary window; DAA, double aortic arch; DAo, descending aorta; d-TGA, dextro-transposition of the great arteries; HTA: hemitruncus arteriosus; LAA, left aortic arch; LV, left ventricle; MPA, main pulmonary artery; PDA, patent ductus arteriosus; RAA, right aortic arch; RPA, right pulmonary artery; TA, truncus arteriosus; VSD, ventricular septal defect.

Fig. 4

Magnetic resonance imaging in a 6.5-year-old boy visualized that the right and the left brachiocephalic arteries arose separately from the ipsilateral right aortic arch (RAA) and left aortic arch (LAA). The R1, R2, L1, and L2 denote the right common carotid artery (1^st branch of the right aortic arch), right subclavian artery (2^nd branch of the right aortic arch), left common carotid artery (1^st branch of the left aortic arch), and left subclavian artery (2^nd branch of the left aortic arch), respectively.