Case Report

Our case was a 9 years old girl and common-conseille of Cardiovascular Surgery and Pediatric Cardiology diagnosed her having incomplete double aortic arch+VSD(pm-restrictive).We accepted her to our clinic for VSD repairment. MRI showed that arcus aorta is at right and image was consistent with distal left arch aplazia which was associated with incomplete double aortic arch(Figures 1 and 2).

Figure 1

Figure 1

Figure 2

Figure 2

Left CCA arised from aorta first and then right CCA and SCA followed it.Left CCa and vertebral artery seperately arised from aortic diverticula(Figure 3).

Figure 3

Figure 3

Catheterization and angiocardiography defined the aortic arch anomaly similarly.Although there wasn't dysphagia or gastroesophageal reflux symptoms,esophageal MRI which was performed at the same time,showed an indentation at postdan T3 esophageal level which was attached to vasculary ring(Figure 4).

Figure 4

Figure 4

VSD was repaired with a 1x1cm PTFE patch and PFO was closed primarly.She was discharged on 5th postop day and controls were normal.

Comments

Cardiac imaging is critical for the initial assessment of congenital heart disease as well as for the treatment planning(₁). Because that noninvasive MRI can be used to evaluate congenital heart diseases and gives quantitative information about anatomy,cardiac functions and flow dynamics,it takes place in clinical routine. MRI is a promising method for further assessment of the cardiovascular pathologies diagnosed by echocardiography(₁).

Aortic malformations may be associated with other congenital heart abnormalities or may present independently, as incidental findings in asymptomatic patients. For more than 30 years, conventional imaging techniques for detection and assessment of congenital anomalies of the aorta have been chest X-ray, echocardiography and angiography. In recent times, considerable interest in congenital aortic diseases has been shown, due to technical progresses of noninvasive imaging modalities. Among them, magnetic resonance imaging (MRI) almost certainly offers the greatest advantages, especially in young patients in which a radiation exposure must be avoided as much as possible(₂). Cine angiography and echocardiography have been utilized to diagnose congenital aortic arch anomalies. However, the visualization of great vessels by echocardiography is limited, while cine angiography requires cardiac catheterization with ionizing radiation(₃).MRI, with its inherent multiplanar imaging capability, is well suited for evaluation of the thoracic and abdominal aorta and can be used with or without contrast enhancement(₄).

In the study of Dillman et al., interrupted aortic arch (IAA) has been evaluated traditionally with echocardiography and angiography, MRI can accurately diagnose and characterize the various forms of IAA and associated congenital heart defects. MRI can also be used to evaluate for postoperative complications after repair(₅).

In the study of Ming et al., the population consisted of 416 patients. Congenital obstructive aortic arch anomalies were diagnosed in 213 patients. Among the 213 patients with anomalies, coarctation of aorta was diagnosed in 174, interruption of aortic arch was diagnosed in 35, and persistent fifth aortic arch with fourth aortic arch interruption was diagnosed in 4 patients. The diagnostic sensitivity, specificity and accuracy of MR imaging techniques were 89% (189/213), 84% (170/203) and 86% (359/416), respectively(6).

In the study of Cantinotti et al., three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right-sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, magnetic resonance imaging was used to plan the surgical management. They noticed a strong correlation with well-defined subgroups of congenital heart disease. Right-sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%).In conclusion they suggested that magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies(₇).

Contrast-enhanced MRI is a safe, accurate, and fast imaging technique for the evaluation of aortic arch and may obviate the need for conventional cine angiography(₃). MRI provides an excellent visualization of vascular structures with a wide field of view, well suited for evaluation of the thoracic aorta malformations(₂). Cardiac catheterization may be reserved for some types of complicated congenital heart disease and for obtaining hemodynamic information(₃).

Correspondence to

Doç. Dr. Ufuk YETKIN, 1379 Sok. No: 9,Burç Apt. D: 13 - 35220, Alsancak – IZMIR / TURKEY Tel: +90 505 3124906 , Fax: +90 232 2434848 e-mail: ufuk_yetkin@yahoo.fr